“I would never look at things in the same way ever again. I had been made aware and I had met the one who was responsible for that taking place. She was beautiful and I would soon mourn along with all of those who followed her. This, as she passed into eternity just three months later. Why Batten Disease and these families? Why do I continue to hold them in such high regard, and why do I pay so much attention to this one childhood disease? My perspective in life had changed once I was made aware, and these precious Batten warriors have become much of my focus.”
Taken from the blog titled “Lasting Impressions”
It’s hard to believe that I have been at this for two years now. It has, in fact, been a little longer than that. I started writing back in 2016 in order to share the experience of diving an underwater memorial and also to share about meeting a very special Batten princess. I had also written a little bit about being Benjamin’s Daddy. There was a time when I was asking myself something like “where do I go from here?” I had found that I loved expressing myself and discovered that I was okay at putting words together. I didn't want to quit but I felt that anything that I could write about myself would be boring to most people. Maybe my career, yawn! Sure, rockets and helicopters are cool but I didn’t exactly invent the things. I never have been the best at any one thing that I have done for a living so telling people about all I have achieved would not be possible. I really do not have much in the way of advice that I can offer so blogging about myself wouldn’t be of much help. I do love my life for the most part but telling you about all the things I have achieved, well, It is just not happening.
I was at one point made aware about Batten Disease through a set of circumstances that I consider to be part of my story. Everybody that has been made aware has their own story as to how it happened. For many, it came about simply as a result of searching for things of interest on Facebook. In the end, I suppose the means by which you were made aware doesn’t matter, as long as you were made aware. People need to be made aware because that effects change. Just knowing about these families is important because these children are so very special. What about all of the other kids that suffer from cancer or other rare diseases? Are they not important also? Of course they are! It doesn’t matter what the affliction is. We are talking about children and sometimes young adults. Devotion to those who battle a disease exists no matter what the source of the battle is. Kids are to be adored, no matter what.
So why have a blog that just talks about one disease? You know, it might not be that way if I had more time on my hands. I do think that more attention needs to be drawn to rare diseases in general. At one point I had started to reach out to the pediatric cancer community but then I realized something. There was no way that I could do a good job of being an advocate in that community and at the same time, continue to work on the behalf of Batten families. I just work too many hours. Anything worth doing is worth doing well and I am passionate about this. Children with Batten Disease have a big piece of my heart and there was no way that I was going to stretch myself too thin. I do also feel that you as families in the Batten community represent well all of those who battle other rare diseases. Your stories have much in common with theirs!
Working with the Batten community and being allowed to write these stories is very special to me. It is a privilege to have been given people’s trust and to be involved. The Batten community is a very special group of people. I have used a phrase a few times in my blog now that I like to repeat from time to time. I say that Batten families are made up of ordinary people that have been asked to do something that is extraordinary. To be given that news concerning your child’s diagnosis and the effect it would have on a parent. It is difficult for someone on the outside of the community to understand. You had hopes and dreams for a life that you helped create and then, all a sudden, you are in the midst of a search for answers. Hope for a diagnosis that can be dealt with eventually leads to something that you never would have expected. Something that most often, a parent didn’t know existed. The experience of going through the whole thing is as rare as the disease itself.
This leaves the Batten community a somewhat closed one, and rightfully so. This meaning that only those affected by the disease can truly understand what it is like to experience the things that are dealt with. Once welcomed in, each family gains access to a group of people that are able to understand what they are going through. This, because of going through it, or having gone through it themselves. There are some fantastic people out there that bring comfort and concern as they follow these family’s journeys and that work is so important as well. So why am I bringing all of this up? I am again, just expressing my gratitude towards all of you in the Batten community that have allowed me to write about you. I am also so appreciative of the families that have expressed their gratitude for what I am doing through their very kind words. I will never forget a comment on Facebook that was made by a Batten mom earlier on and it was simply this, “He is writing about us.” My thought when I saw that short comment was something like, “Well, yes I am!” You bet I am! There is a reason why.
I have never tried to claim that we have experienced what a Batten family will go through. We are, however, parents of a special needs adult. We were told by a geneticist that Benjamin’s condition was most likely degenerative when he was a young child (He is now 28 years old). She could never pin down a exact diagnosis and we have never continued to look for one. We knew because of the background in my wife’s biological family that we were predisposed to have a child like our Benjamin. We just never thought about the fact that it might happen. The end result was that we wound up with this most wonderful human being in our house, as well as, all of the responsibility of having him with us. What is my point? We know what it is like to have a child that does not function normally but we do not know what it is like to be told that our child has Batten Disease. I do know what it is like to have an extra strong bond with a child but I do not know the heartache that comes with having received that news. The news that your child has Battens. My sensitivity to my own son lends itself to a sensitivity to kids who battle Batten Disease. Does that make sense to you? There are other people out there that have a sensitivity towards these kids for their own reasons. I am friends with some of the best of them!
In the end of it all, I have this opportunity that I have been presented and I will continue for as long as I can and also, for as long as I am allowed to be involved. I have been rewarded abundantly for what I have done so far. Words really matter and they can be a great way of rewarding someone for something that is appreciated. Just knowing that I can make a difference in the lives of those who battle Battens has been very rewarding to me. Several people have commented about the fact that I have put a lot of time into writing about Batten families. A comment like this would be a true statement as this is where my time away from work is now spent. Some things have been set to the side but when you are involved in a “labor of love”, you gladly set those things aside. I have said in the past that it is because of these dear children that I discovered that I do love to write. I did not find this out until my mid 50s. Would it not be a great reward if I was able to write in my retirement? All because I fell in love with some of the most amazing little people on the planet? All because I took the time to write about them? I have been rewarded almost non-stop since the time that I first started doing this. Even if the only thing that I have gained from this is the kind words of people within the Batten community and those who follow them!
I created a Facebook group page and named it Benjamin’s Daddy - The Batten Blogger. I did this because I wanted to have a special place to connect with Batten families and also to keep people informed about the blog. The group page has a significant amount of members, although it is not as big as some of the pages that were created by Batten families. That is just fine with me. These families deserve all of the attention that their pages get because they are sharing their journey with all of us. I do appreciate the fact that my posts are seen by families within the Batten community as they often leave comments. These are SO appreciated because I feel like I am working on their behalf. Sometimes, I do wonder if my motives for doing what I do are questioned. I really am an honest person. I do not do this for monetary gain. If I was to do so, I would make sure that a very significant amount of the proceeds would go back into the Batten community or to research. I have not seriously considered doing this for profit because what I get from doing these stories is something that money just cannot buy.
Am I trying to gain notoriety and or acceptance by doing this? First of all, this is not anything that I ever planned to do. I have always worked in a production environment in Aerospace and Defense or in private industry. I now work in the aviation field as a mechanic. I never, in a million years, could have planned this. Let me just say this. I am not trying to gain notoriety but it is nice to be known. I am so touched by these stories and I am rewarded by knowing that people see the effort that goes into doing them. The parents need to be acknowledged also because of the effort it takes in providing the material to me. They write the stories through living out the details. There are so many people that are on social media whose time is spent honoring families that battle Batten Disease. I am sure that people that make up Batten families will acknowledge how important these folks are to them. Words are so powerful and I know how much kind words mean to the families. So am I trying to receive an additional level of acceptance by doing what I am doing as a writer? While it is nice to be accepted, that is not the reason that I write. I simply fell in love with these kids and their families as I continued to do these stories.
I sometimes comment out of ignorance or a lack of caution. I would ask that you would forgive me when I do. I know that I need to be responsible when I write and that I need to have my facts straight. This subject is very sensitive in nature and I know that I need to be careful and also very respectful. I hope that I have done this the majority of the time as it is always on my mind to do so. These kids just melt your heart if you allow yourself to be vulnerable to that. I have used words like “sacrifice” and phrases like “undying love” to describe the action of Batten parents and other family members. I wonder if Batten parents ever read these stories with skepticism as they to say to themselves, “we are just regular people. Quit with the drama!” Remember this. These stories are written from an outsiders point of view. That, with the help of those who have, or are, battling Batten Disease. There are different ways to handle the journey. Some just want to live a private life while others want people to know what it is like for them. Some are anxious to have their stories told by me or someone else. Others are like, “No thank you!” For yet others, it is just too difficult to talk about. They also have a piece of my heart!
The number of stories that I have been able to do has decreased as they have become more detailed. The amount is not as important as the quality of the story. You deserve the very best. Hopefully, you can see where my heart is at even though my level of experience is not as great as others. I was trying to do two stories a month in order to keep interest in the blog going. The way that I use this blog is out of the ordinary. Most blogs are shorter with entries made frequently. This one has stories that are equal to, or greater than full length magazine articles. I wouldn’t know how to do things differently at this point. I have thought about using the help of a Batten parent while serving as the editor. This way, I could have entries in between the larger stories. We will see what the future holds. Any idea?
Some people would criticize me for paying attention to the number of people that view the blog. If my only purpose for doing this was to honor the families that battle Batten Disease, that would be enough for me. That, in fact, is my primary reason behind what I do. Spreading awareness was never my first thought but the fact is that having this blog does help to do so. I view the numbers because they are made available to me by Weebly, the host of my blog site. Now there are some stories that see a huge spike in viewership all at once. This is because the family that the story is about is so well connected to the community in which they live. Sometimes, they have a huge presence on social media. Still, there are others whose stories get looked at gradually over a period of time. Either way, all of these families are part of the whole. We are not talking about a New York Times best seller here folks. People do look though and I am so pleased at times by what I see. You are all so special to me and I am honored to be involved in the small way that I am! What I really love to see is when I look at the total number of stories that get viewed in a month. It makes me happy when I see that a story that was written over a year ago is still being looked at. Even if it is only a small amount of views in a month. Page views for the last thirty days has been a little over 2500. Shortly before that it was closer to 3000. It fluctuates and a lot of those views are by people who frequent the blog. I am sure that many of those views are people that stumble upon the blog or who were told about it. The big point is that the blog increases awareness in some part and looking at the numbers provides me with some confirmation of that.
Batten families, I have poured myself into this and you are all always on my mind. I have served in the military and worked at jobs that support our nations defense. The helicopters that I support by working in my company’s overhaul department fight fires. I am a husband and a father of three. I am Benjamin’s Daddy and he is one of the most special guys you are ever going to meet (I am a little biased I suppose). Regardless of the number of people who know who I am, I do feel that I have never done anything as important as telling these stories. I still cannot wrap my head around it. What is it like to be a young adult whose big desire is to raise a family. All of a sudden, you are asked to travel down a road that you never knew existed. You all amaze me! Your level of devotion is truly inspirational.
My big intent for the blog is to just keep going with it for as long as it is practical for everyone involved. If I were never to write about another Batten child, I would consider this to have been the best thing that I have ever done. The fact is that there will be more on the way. More people have shown interest and the desire for me to continue is still there as well. This blog is my baby and my passion. Anybody that knows me is aware of that. These stories belong to the families they are written about as much as they belong to me. These stories are about your babies and I guard them in such a manner!
Some what of a funny story is how the content of the blog was developed. I chose Weebly’s free version as my host, originally to get started. You could find the stories if I first shared the link and that was good. Then, I found that I could have my own website domain and it really wasn’t that much in cost. That was the birth of gregster60.com. I was off and running so to speak. I was doing shorter stories at that time and incorporating beautiful pictures of these kids and their families. This, of course, helped to tell the story in a bigger way. I then remember that I was in the middle of posting a story and adding photos as I went. All of a sudden, I wasn’t able to add anymore pictures. I was outraged and then realized that I was going to have to “Upgrade” my package with Weebly. I was like, “You’ve got to be kidding me!” Well, the amount of money increased but they had me at that point. The yearly cost is certainly affordable. This in order to be able to do something that is my passion. Trust me, I spend a lot more on other things (I would spend more in a single weekend when I was still diving). What an amazing experience this has been for me. Maybe I shouldn’t be telling you that part but I am leading up to something and here it is.
Even if this were to come to an end for some reason, I would pay the small amount required to keep these stories in tact by keeping the domain on the worldwide web. I would do a story about Batten Disease itself and then invite people to look into the table of contents and to read about these kids and young adults. They are just the most beautiful of all human beings! Batten Disease really isn’t that rare. Is it? The definition of what makes a disease rare varies from one country to the next. In Europe a disease is defined as being rare when it involves fewer than 1 in every 2,000 people. Here in the USA a disease is considered to be rare when it involves fewer than 200,000 people. Really? As I continue to write, the number of places and countries where families reside continues to increase. Turkey, Greece, Scotland, the UK, South Africa, and so many different places here in America. Every story is unique even though some of the circumstances are shared across the globe. I have grown in my ability as I have continued to write. I can’t claim to be the best advocate that there ever was because I never will be that. That means that there is endless room for improvement.
The stories have gotten longer but many of the details have always been sweet. No matter what. At least they have been to me! Thank you so very much for putting up with me. Hopefully, I have done some good by what I am doing. I hope that 2019 is the most amazing year yet. Not just because of this blog, but more so because of progress towards a cure for all of the variants of Batten Disease. There is more work to be done and I will stay on it!
Dear families, I want to thank you for the opportuniy that I have been given by you to grow as a person. I have recieved a lot of compliments on my work by many of you. I was even called “The Famous Batten Blogger” and “Our Batten Blogger” by a couple of awesome Batten moms. I have been told that I am amazing several times but really, you are all the amazing ones. You write these stories as you care for these very special little warriors who are so deserving of that care. I only take what I am given by you and I put into print using as much care as I possibly can. This is always a team effort and I am humbled by the fact that I am allowed to take part. I am looking forward to being involved as long as I am allowed to be and for as long as I am able to be. There is more on the way and I am looking forward to it. Thank you once again and by the way, Happy New Year!!
Blogger and Advocate
“I truly love these beautiful children with every beat of my heart. I am an advocate for those with special needs and I am passionate about these children and their families. I will continue to follow them and I will advocate for them and their families until a cure is found!”
My good friend and
What is it about these kids? Not everyone that is told about children with Batten Disease will show an interest in their plight. Perhaps most will have a casual interest. I suppose many would be sympathetic. I know that I have seen a number of different responses when I tell people about the disease. I also get a mixture of responses when I tell people that I have been writing about the families that battle it. Some will think that what I do is interesting. Others will have a look on their face as if they are questioning why? What could I possibly gain from doing this? To sum it all up, it is about these kids. They do something to you. That is if you are the type to care enough to pay attention.
What does a person mean when they say that they have been changed by following the journey of a Batten child? It is often difficult to put into words the things that are felt by a person. I don’t know if I have done an adequate job of explaining the changes that have taken place in me since the time that I started to write about kids that battle Battens. I have some things to say about it but I thought that it would be nice to get some help with this. I asked my friend Christen Brawley to help me as I felt a little stuck at the time that I started to write this story. Like myself, Christen is the parent to a child with special needs. She, in fact, has two young sons who are autistic. Her life has been changed by caring for her own children but that is not all that has influenced her thinking. Christen would spend much of her childhood with a friend that had a disease named Lissencephaly. I will let you Google the condition on your own but I will say that Lissencephaly is most often fatal at a young age. Her friend, Jacinta, was only supposed to live until age two, however she would go on to live to the age of twenty-seven. Christen spent much of her young life helping in her friend’s care. Their time together taught Christen much about compassion and how to listen with your heart as her friend was mostly non-verbal. Words are not always needed to communicate. My point in sharing these things with you is that it was natural for Christen to fall in love with Batten children as she discovered her first Batten princess.
This would happen as she was seeking support for herself in raising her children. Her search for help included groups and pages on Facebook and that is when she would become aware. Christen would find and start to follow this little princess of a girl and it is amazing to read how her life was impacted by finding her. Here is what she had to say, “I was instantly drawn to this beautiful child. I could not get enough of her. Her beauty and her inner strength literally brought tears to my eyes the first time I saw her. I followed her everyday after that, cheering her on as she so courageously fought against the Batten monster. Relishing in the joys that she found in every single day. Her smile was bright and beautiful and it is forever etched into my consciousness. My first Batten girl. Oh, how special she was!” Christen would continue to speak about this princess and she would share her reaction when this much loved child was taken by the disease. Christen said, “I remember when she was making her transition into heaven. I cried reading every update. I was surprised at how emotional I became over a child I had never met. The sadness that engulfed me when she gained her wings was almost unbearable. All I could think about was how this beautiful girl had changed me. I had to know more. I had to see more of these gorgeous children.”
And so, Christen would continue to look for other kids to follow. She absolutely adores these children. The means by which she first became aware would be different than with myself but the result would be the same. Her first Batten princess would also be the one who made me aware and I have never been the same since that time. The way that I look at life and my position on this planet has changed. For example, I no longer draw my identity from what I do for a living. Following sport teams is just a source of entertainment when I have the time. I no longer sit and yell at the television while watching the news. I don’t do politically angry posts on Facebook any longer. I have better things to do with my time now. It would eventually be through these children that I would learn that I love to write and writing is what I will do until I am no longer able to do so. It has become my way to honor these children and also a means by which I can be their advocate.
Just like anyone else, I suppose that I have always noticed children when they are around but I now look at them more intently. I study the quality of their innocence and the contribution that they make. The joy that is expressed by them is without equal. Their lives are most often uninhibited by the stresses that are part of our everyday existence. They just enjoy their little lives so much as they absorb new information. Children sure have their ways of showing us that they are part of the human race but they also show us a quality of innocence that is unseen in the rest of us. With all this in mind, it is so disheartening when we see a child in distress. Whether it be a disadvantaged child in a third world country or one in a pediatric ward of a hospital that treats cancer, we do not like seeing them suffer.
So again, I ask the question. What is it about these kids? Speaking specifically now about children with Batten Disease. I think that it is their plight in combination with the qualities that I have mentioned above. Batten Disease is a highly degenerative disorder that is fatal. While there is some room for optimism, we know what the eventual outcome will be. The qualities of a child in combination with what is faced as a result of a disease like Battens just melts us inside. That is if we are the type that cares. Parents of Batten children are realists. They cling to the hope that a cure or treatment will be found as they also fight for their kids in a most unselfish manner. Their display of love and strong character is often the subject of what I write. They are strong for their children because they have to be. My level of respect and admiration for these parents is just off the chart. Christen sees the same things I do and that is why I have featured her in my introduction to my next story.
Kayla Neveri is a mom to a most beautiful little Batten princess. We were friends on Facebook previous to the Batten conference in Nashville but we were able to meet in person at that time. She expressed an interest in me writing about her daughter who is named Breanna and you just know that I would not pass up the opportunity to do so. Christen Brawley had not yet seen Breanna’s picture on Facebook until I posted it just prior to starting this next story. Upon seeing Breanna’s picture for the first time, she referred to Bree as a “REAL LIFE DOLL” (upper case added for emphasis). I couldn’t agree with her more on this because that is what Breanna is. She is Kayla’s Perfect Princess and this is her story.
It was in the midst of the years 2008 and 2009 that Scott Neveri and Kayla Green met while while working as security guards at a factory. As is sometimes the case, love stories begin at work. That is where Scott and Kayla’s story would start. They met after Kayla had been on the job for about six months. First they were just co-workers and then they would become friends. The friendship would lead to an attraction towards one another and they began to date. Scott and Kayla would really hit it off and found that they had like ideas and desires for the future. They would date for about a year and a half and then, Scott would ask Kayla to marry him. That would be in the beginning of 2010. Kayla would, of course, say yes and plans would be made. They had already been discussing a Birthday trip to Las Vegas when the subject of marriage came up and this would lead to an idea. Why not get married there? Is was cost effective for them to do it that way, and so, they did things Las Vegas style. Kayla would tell me that they would marry on May the 8th of 2010. With them was a maid of honor and a best man. Close friends and family attended too. Everything was perfect and they became man and wife!
Scott and Kayla were happy and enjoying their new life together. They discussed what they wanted for their future and like most couples, that included children. The couple would enjoy their time together for about six months and then they decided that it was time to start their family. Their plans would become a reality as they welcomed their first little bundle into the world. That bundle was a boy and they would name him Garrison. The little guy made his appearance on October the 4th of 2011 and he would go from adorable to handsome in no time at all. Scott and Kayla were oh, so happy and life was good! It wouldn’t be long after little Garrison came along that they decided he would need a sibling to grow with. Tried as they might, Kayla was not getting pregnant as easily the second time around. There were a lot of negative pregnancy tests and they would eventually decide to take a break from trying. In the meantime, they had their little man to enjoy along with enjoying one another.
It goes without saying that difficult times can come into everyone’s life. Kayla would experience a time like this, even as she enjoyed the experience of being a new mom. Her own mother, Brenda Lee Ruhland, would pass away at the young age of forty-four. How devastating this must have been. This happened in April of 2013. Brenda had oral cancer that quickly became bone cancer and it spread throughout. Not many things can be more difficult but Kayla and her family would persevere. They still desired to have a little brother or sister for Garrison and this would soon happen. It was a month after her mother’s passing that she did another pregnancy test. Kayla felt certain that she was pregnant and her suspicions were confirmed. She was going to have another baby! The sadness over losing her mother must have been eased a tiny bit by the joy of knowing that another baby was on the way. Once it was determined that they were going to have a girl, Scott and Kayla decided on the name Breanna Lee. The name was chosen in memory of Kayla’s mother. How very fitting!
Scott and Kayla were excited. They were going to have both a boy and a girl. How perfect is that! The pregnancy would go smoothly up until near the end of term but then a problem would develop. Little Breanna was due to make her entrance on the 27th of January in 2014 but she would not be able to wait until then. Kayla had gone to the doctor for a routine appointment and once there, it was determined that her blood pressure was very high. She had developed Preeclampsia. As was the case with Kayla, this condition develops in the later part of a woman’s pregnancy. It can be very serious if it goes undetected. And so, it was on January the 15th that the doctors would take action. Little Breanna would be delivered just as her big brother had been, by cesarean section. Breanna Lee was, oh, so perfect in appearance! She had all her fingers and toes and she was covered in brand new baby skin. Baby Breanna weighed seven pounds and two ounces at birth. She was just beautiful and she was so healthy.
Everything would go well with Breanna as she made her appearance but it didn’t go as well for Kayla. As the medical staff opened her up to deliver Breanna, they noted that some of Kayla’s organs had been fused together. Kayla said that it was a scary moment for everyone. This had to be fixed and so she would be put under in order to correct the issue. Her opportunity to hold her perfect princess was delayed until the time in which she would awake. Either way, I am sure that her reaction would have been the same. Intense love at first sight! Here is how she put it to me, “After I was fixed up I finally was able to hold my baby girl. I had no clue that the love of a parent grew bigger when it came to more kids. I was worried about how I was going to stretch the love I had for Garrison to Breanna, but quickly I realized my love would keep growing. Our family was finally complete.” Things at that point, must have been wonderful for this young family. There was a lot of love in the air as they welcomed Breanna into the world. A mom and a dad that loved each other and now, a boy plus a girl. How sweet is that! The future looked to be incredibly bright and who would argue that it was not?
Baby Breanna was not only beautiful. She was also very strong and quick to pick up new skills. Kayla said that Bree took her first steps when she was a little over nine months old. She was walking, running, and climbing by ten months of age. It was at ten months that Breanna would give her mom a little scare while also providing one of the cutest memories that you will ever hear of. Here is what Kayla told me about it, “When she was 10 months old, Breanna went ‘missing’ in the house. I was running around like a madman because I couldn’t find her. That’s when I heard a giggle come out of the bathroom. I found her standing on the tank of the toilet hiding behind the hand towel which was hanging above. That’s when I realized I had a Breannasaurus on hand.” Someone that is as big as a hand towel was already showing off her little personality and that little person inside was as sweet as could be. Brother Garrison, in the meantime, was growing into a great big bother. He loved his little sister and as Breanna grew, so did their love for one another. I am certain that the house was filled with laughter and innocent fun. That is what children do. They fill the house with fun while making priceless memories for their parents.
Breanna’s development was right on track. In fact, she was always ahead of other children her age. There was, however, something that Breanna was doing that got Kayla’s attention. It would happen when little Bree would fall. Kayla would notice a weird kind of leg jerk or twitch whenever Breanna would fall. It started to happen as soon as she learned to walk. It only happened a handful of times the first year of walking and it would only last for a second. Moms are amazing at picking things up that go unseen by others and such was the case with Kayla. She was the only one that would notice this as it happened. Kayla said that as Breanna turned two she was noticing that it was happening more. She tried to point it out to friends, family, and the pediatrician. Everyone thought that it was the startle of falling that was causing it. Whatever the reason, Kayla wasn’t worried because Breanna was developing as a normal toddler would. She was even ahead of schedule. Life would go on and Breanna was getting cuter by the day. Kayla continued to observe her princess as her development continued. As Breanna was approaching three years old, Kayla would notice something else. Breanna’s vocabulary was not as large as some of the kids. Then again, it was at the same level as some of the others.
Still, there were the leg twitches and the number was increasing with Breanna’s age. This was starting to cause Kayla to really become concerned. She was worried at this point. Finally, a cousin had seen the twitching as it happen when Breanna fell. That first sighting, according to Kayla, seemed to have a “snowball effect”. More people started to see it and as Kayla would put it, she no longer felt crazy! Kayla finally felt like she had something that she could take to the doctor in the way of a concern to be talked about. Before that though, a development would take place that would indicate that Breanna was beginning a journey. This would be a journey that no one would expect her to go on. That, because it was one that nobody knew existed. The journey that is Batten Disease. Concerning her and her family, Kayla would put it to me like this, “I had no clue what was going to hit us.”
Kayla remembers the date. It was the 22nd of March in 2017. An event would take place that would indicate what the future held for little Breanna and her family. Garrison had stayed home that day. He was sick with the stomach flu which included a fever. Breanna, who at that point had turned three years old in January, had caught the bug that evening. Kayla said that Breanna had a slight temperature of 100.8 degrees and she had been throwing up. Kayla, being an wonderful mom, was comforting Breanna by snuggling with her as she held her daughter on her chest. Little Bree was watching a movie on Kayla’s phone and had started to whine. She would do this when the movie was interrupted by another app that was opening on Kayla’s phone. I’ll quote Kayla directly here as she explains what would happen next, “So I looked at the phone to fix it and nothing was wrong with it. I started talking to her to calm her down because she had whined again. That’s when I looked at her. Her eyes were rolled completely backwards. She was unresponsive.” How scary this must have been for Kayla. She called out to Scott who came flying into the room to see what was happening. They thought that little Bree was maybe choking or experiencing cardiac arrest. This, as she stopped breathing and her face started to turn a bluish color. Scott then began CPR as Kayla called for help. The couple realized that Breanna was experiencing a seizure as she started to twitch after receiving CPR. She would seize for about twenty minutes and had stopped by the time that the ambulance arrived at the emergency room. As is the norm, the family would be at the emergency room for a couple of hours and as Kayla would put it little Bree would return to her normal “Breannasaurus” self. The medical staff called the event a febrile seizure, attributing it to the low grade fever that Breanna was experiencing with the flu.
Speaking of a “snowball effect”, this would just be the beginning of what this family would experience before an answer was found. The next occurrence would take place a week later as Breanna and Garrison were watching a movie in Kayla’s bedroom. Kayla heard a loud bang and went to see what was taking place. It would be better if I let her speak for herself. Here is what Kayla had to say, “That’s when I heard a loud bang and went into the room. Garrison said Breanna was climbing on a box and fell off. As I picked her up she went limp again and became unresponsive like the week prior. I called 911. By the time rescue arrived Breanna was 100 percent fine. She was telling them to leave her alone. She was running around the house like a nut. I felt nuts.”
You can tell from Kayla’s comments that this all had to weigh on her and Scott as things continued to develop. Still, they were just at the beginning of things. Kayla skipped taking Breanna to the ER when this took place but she took her to the pediatrician a couple of days later. While there, she mentioned the seizures and also the leg spasms. Upon hearing this, the doctor decided that little Bree needed to see a neurologist. However, another incident would take place before they could get to that appointment and it would happen two weeks after the first incident. Breanna had another long seizure and she was taken to the ER by ambulance. This all had to be part of what was becoming traumatic experience. What was taking place with little Bree, the princess of the family? Whatever it was that was going on was very serious. The neurologist would come to see Breanna while the family was at the hospital. She ordered an EEG, an MRI, as well as, other tests. Kayla said that when all was said and done, it was determined that Breanna had epilepsy and she was started on a seizure medication. This all seems too familiar to me after doing so many of these stories. I genuinely feel for each family that travels down this road. We are after all, talking about their babies.
It was after the third seizure that the leg twitching would become more frequent than before. Soon after that, Breanna was falling backwards and her body would twitch as she did. This would be a difficult thing for any mom to observe. Sometimes it was only a limb that would twitch and other times it was her entire body. Kayla said that the twitching would last anywhere from one to three seconds. The number of times that this would begin to happen would be most alarming. Breanna would do this at least one hundred times a day. Some days, it was more than that. Oh, my gosh!
It was between April and May of 2017 that more would transpire. The constant seizing would eventually lead to Breanna regressing in her level of function. A little girl that was at one time so quick to learn new things, was now regressing. One important example concerned the rock wall that the kid’s grandfather had built in the backyard next to the swing set. Breanna had, in fact, been the first child to learn how to use the wall. The weather had improved and the yard had dried out, so Kayla allowed the kids to go out and play. According to Kayla, Breanna tried to climb the wall but couldn’t. Here is how she put it to me, “I let the kids go play. Breanna tried her best to climb that wall and she couldn’t do it. It was as if she forgot what to do at first, then figured out the concept of it but it was like her body couldn’t do what her mind wanted. That’s when I knew something terrible was wrong. After that, I called her neurologist and she also agreed.”
As one might expect, the initial diagnosis of epilepsy would eventually prove to be too general for what was going on with Breanna. There had to be more to this and much to the family’s despair, they would one day find that to be the truth. Breanna would be referred to Milwaukee Children’s Hospital. Scott and Kayla’s princess would be there for quite a few days as several tests were performed, one of which was a 48 hour MRI. Kayla would tell me of a conversation that she overheard out in the hallway as this was all going on. A group of doctors were discussing which conditions they should be testing for and apparently, one of those was Batten Disease. Even so, the family would leave the hospital with no concrete answers as to what was going on with little Bree. Here is what Kayla had to say about it, “After that hospital stay, the doctors were at a loss. She still was diagnosed with epilepsy but the reason was unknown. I was told by one doctor ‘If it were serious it would have shown up by now’. Those words haunt me to this day. This was just the start of Breanna’s Batten journey.” And in fact, much more would happen. Breanna would continue to regress in her development skills and this had to be most concerning to those that love her.
Kayla said that eventually, Breanna’s speech was no longer at the level that was held by other kids her age. What was also very alarming was the fact that she had no fear of danger. She would go as far as to do things that could kill her. This included no fear of heights, water, or road traffic. The hospital visits would continue in abundance at this time. This would include tests and blood work. Near the end of 2017, genetic testing was recommended. Kayla said that four to five different doctors had requested that the testing be done for Breanna but this was declined by the family’s insurance company. She would go on to say that it was finally the genetics lab that did the hard work in getting the approval. On Thanksgiving of 2017 Breanna would have another large seizure while Kayla was home alone with the children. Regardless of what was causing the seizures, Kayla had become experience at handling them. Mr. Garrison also knew how to lend a hand. This family, however, really needed an answer as to what was going on and eventually, the answer would come. It was also that November that Breanna had blood drawn to be tested by the genetics lab. It would be just before Christmas of 2017 that the lab would contact the family because they wanted Scott and Kayla’s blood to be tested as well. Batten Disease is confirmed in a child when both parents are found to be carriers of the same defective gene.
They both reported to the genetics office for the blood draw and Kayla recalls what took place, “It was just before Christmas of 2017 that Scott and I were called into the genetics office and they had told us that they needed to test both Scott and I to confirm diagnosis. Scott didn’t want to know what the diagnosis could be until it was confirmed. I needed to know, so they had him leave the room while they told me the name. Late infantile Batten Disease is what they told me could be Breanna’s diagnosis if Scott and I are both carriers. I had never heard of it before so the second she left the room I googled it. I was in instant shock and crying. I didn’t expect to read what was written. But quickly I wiped the fear off my face because my husband was going to enter the room soon.” Kayla would not waste any time after that. She started reading about the disease and she would even find the BDSRA Facebook group while online. There, she started reading the stories of families who had children with the disease. Depression hit her as she already felt that she knew the truth. Kayla knew deep down inside that Breanna had Batten Disease.
Kayla had all of this information bottled up inside of her and it was more than she could bare alone. She decided to reach out to a few close family members for support. It was also just after Christmas that she would finally inform Scott as to what the diagnosis could possibly be. You see, Breanna is Kayla’s perfect princess but to her big strong daddy, Bree is his little peanut. As the New Year came, so did the knowledge that would bring the answer that was expected. It was January the 3rd of 2018 (12 days before Breanna’s Birthday) and Kayla was out grocery shopping for the family. She received a call from the genetics lab that she knew would be important. The lady at the lab asked Kayla if she was somewhere safe and free to talk. She could wait no longer, so Kayla told her that she was. I will let Kayla take it from here, “That’s when she informed me that our results came back. Scotty and I were both carriers and Breanna had Late Infantile Batten Disease (CLN2). In a state of shock, I finished our conversation which I no longer remember much of. I finished my grocery shopping and went to my car and cried. The details are kind of blurry as to how things happened but once I was done crying, I went home to talk to my husband and tell him the things I had been informed of. Since I had time to process and learn about this disease it wasn’t a shock in the way it was the first time I looked it up. But, it was the fact it was confirmed. My baby girl had this monster in her and it would take away everything about her before taking her away from this world. This, before her teenage years and I couldn’t save her.”
It seems like the initial space in time after receiving the diagnosis is always the worst part of the the beginning for Batten parents. That is what it was like for Kayla. There was, however, help on the way and it would come quickly. Here is more of what she had to say, “I felt lost. I had a gaping hole in my heart and I didn’t know how to fix it. So, a couple days after the diagnosis I finally had the courage to reach out on the BDSRA Facebook page for help. I was overwhelmed with love, support, and help. I now have a new family in the Batten community that I’m grateful was there in one of my most vulnerable moments. They helped pick up some the pieces and fill some of that gaping hole.”
The importance of the help that is available for these families through the Batten Disease Support and Research Association (BDSRA) and through other families via social media cannot be over emphasize. Compared to many, Kayla’s search for answers wasn’t as traumatic as it could have been. The speed at which she received help was critically important (I will explain further in a moment). Kayla said that most of the doctors that she has met have never heard of Batten Disease. Some knew the name but not the details and many used Google to learn more. In the case of the genetics department, they knew the name and had knowledge of the disease but they didn’t know where to direct the family after the diagnosis was given. It does seem that they did a fantastic job of helping this family get the answer they sought in a timely manner. Well done! Kayla would also say that she has found those in the medical community to be most sympathetic and sensitive after being filled in on the details. One look at little Bree is all it takes!
Again, Kayla found the BDSRA Facebook group something to be very grateful for. If not for this group of people the search for help could have been long and traumatic. She said that her plea for help was handled very quickly. Two couples that she was quick to mention were Allen and Vanessa Severance and Tracy and Jennifer VanHoutan. Both families are fantastic ambassadors within the Batten community. They are largely responsible for the speed at which the Neveri family would learn about a life changing treatment and a physician named Dr. Berry-Kravis at Rush University Medical Center in Chicago. Most people that follow Batten families know of the enzyme replacement therapy that is available for kids that have the CLN2 variant. It uses a drug that takes the place of a missing enzyme in the child’s brain and the trial has been very successful. This gives the child the ability to live a much more ‘normal’ life as it slows the progression of the disease in a most dramatic fashion. These little people are so amazing to me. Breanna had her 23rd treatment on December the 5th of this year and she is rocking the house with them. Little Bree’s port to her brain was put into place on January the 3rd of 2018 and her first treatment took place shortly thereafter. The entire process would actually be quick and go smoothly. This, although the days must have crept by for Scott and Kayla. There have of course, been complications along the way as Batten Disease can be so unpredictable in nature. Breanna became ill with a cold in September of this year and as a result, she refused to eat or drink. Her refusal or inability to eat and drink would lead to her getting a feeding tube put in place. This was necessary for her to get the nutrients that she needs even though she still enjoys snack foods like popcorn or chips.
There is no way that Kayla could have predicted what was going to take place in her life but both her and Scott are meeting the challenges. Kayla said that the experience has changed her 100 percent. It has been just shy of two years since she witnessed her first seizure and Kayla realizes that she now knows how to respond to any type that Breanna might experience. She is able to change little Bree’s feeding tube like a professional and she can handle medical equipment and different situations as they occur. Scott and Kayla are parents to a special needs child of the most beautiful kind. Kayla shares what she has learned about a world that involves those who are dependent on us for assistance, “I’ve also learned SO many things about the disability world that I had no clue about. I didn’t understand just how hard it could be get life saving treatments. Or, how expensive medical equipment, treatments and medication truly were. It's a huge eye opener, and I hope one day to help with making changes. I want to help make things easier for people with disabilities. Even if it’s just providing medical equipment faster or educating people about Batten Disease.”
Kayla would share what is felt by all parents with children that battle Battens. The disease is a monster that first takes away a child’s ability to function at the same level as their peers. It is painful to watch your child regress in areas of development right before your eyes. Kayla would share that Breanna went from being an average three year old to one who was unable to keep up with other kids her age. This happened in less than a year. I know that the Neveri’s are grateful that enzyme replacement is available for their child. This gives them time as they wait and hope for additional treatments and even a cure. As a family, they have been through changes as a result of facing the monster that is Battens. Kayla shared the following to describe the changes that have taken place in them as a family, “It (Batten Disease) tore us down, but with the tearing down we found a strength we never knew was in us and it brought us closer together.” And that is exactly what should happen. Struggles like this disease should draw people closer together. Again, you never know how strong you can be until being strong is the only choice that you have.
Kayla shared about what life is like at the Neveri house, “Our life includes having lots of unknowns. It’s full of doctor appointments, medications, and medical equipment. But, it’s also full of laughter, dancing, and love.” I shared earlier that little Breanna is having difficulties in eating regular food but she loves to eat snack foods like chips and especially, popcorn. Kayla has gotten around to creating a group Facebook page for Breanna and she gave it an interesting name. Most of the pages that parents create will include the child’s name and perhaps a word such as “battle” or “journey”. Breanna’s page is different and I was a little curious as to why Kayla had given it the name that she did. I found the answer as she shared with me a bit about the things that they now consider to be important. When she did, it became one of those “Oh My Gosh” moments for me. This is what she said, “The things we used to take for granted are now things we cherish. Like those trails of popcorn. I used to clean them up right away, now I’m finding myself taking pictures of them.” The page that Kayla created to share Breanna’s journey with the public is named “Trails Of Popcorn”. Does that get to you like it did me? My prayer would be that those trails continue to show up on the floor for a long time to come.
They would and should be just your average family. That would be the case if it wasn’t for Batten Disease. Scott appears to be a big bear of a man but I bet he is the type you could call a gentle giant. A sensitive guy who cares deeply about his wife and his children. That’s a good thing! I loved having a few minutes to get to know Kayla at the Batten conference. She is another of a growing number of people on my awesome mom list. I bet she would give anything to be just ordinary again. I asked Kayla for a little bit of information about big brother Garrison who is now seven years old. She started by telling me that he is smart, funny, and handsome. She also told me that Garrison is a softy even though he is big and tall for his age. He loves art and storytelling and in fact, he is a talent when it comes to both. Garrison is a great big brother and he is best buddies with little Bree. Garrison has noticed the changes that have taken place with Breanna. He has grown in his understanding of Breanna’s situation but he still has questions as to why things are not the same as they use to be. I know that he loves his little sister an I am glad that Breanna has a big brother like Garrison!
Every family has difficulties but this family is facing something that is very challenging on an emotional level. I said at the beginning that there is something special about these kids. Can you see that too? They really tug at your heartstrings. There will be more challenges ahead for the Neveri family but they will do their best to meet everyone of them. This, because of their love for such an angelic little person as is Breanna. Hope and love carries them through each day and Kayla talked a little about that as she shared all of the following, “Our love for Breanna is strong and never ending. Some times Batten Disease gets the best of all of us, but the love we have trumps it all. Because each Batten kiddo is different, it’s hard to know exactly how the future will play out. So, we live in the now. We make as many good memories for Breanna and the rest of us as we can. We do many fun things as a family. Our hope is that a cure is to come soon. Or at least more treatments that let Breanna be her truest self. Where she can enjoy life without pain or regression. Life with Battens has introduced us to many, many challenges. Growing up my mom always said “Fake it ‘till you make it”. It’s a motto that I often find myself saying to myself. Most of the time we aren’t handling the challenges, but we sure know how to keep that smile on our face and keep going. Changes happen so often that once you have a handle on things. It changes on you. As parents you need to learn to adapt to the challenges.”
My friend Christen referred to Breanna as a “Real Life Doll” and she is correct in saying that she is. Breanna is her daddy’s little peanut and her momma’s perfect princess. Her eyes are beautiful. They are so deeply brown that they sometimes appear to be black. That is what stands out the most to me but it doesn’t end there. Kids are meant to be adored and they most often are. That is why it hurts so much to see them battle something like Batten Disease. Research and a cure needs to be a priority but telling people about these little warriors needs to be one also. It is a priority with me and it will be for as long as I am able to make it one. Just like Christen, I am passionate about these kids. I wish the Neveri family all the best and I feel privileged to be allowed to tell their story.
Blogger and Advocate
There is more that needs to be said. I had written this story over the span of an entire month as it was a challenge between work and the pending holiday. I had written it after viewing just a few of Breanna’s most recent pics. Upon being close to finishing, I requested pictures from Kayla and she sent what you see in the post. The story may have been written a little differently had I seen these pictures before starting to write. It may have been written with a little more passion and emotion, although I always try to capture my own feelings as I write. I really am moved by these stories but I was moved to tears when looking at all of these pics at the same time as they paint a picture all by themselves. What do you see? I see a family that is a perfect little story in progress. The dad is ruggedly handsome and mom is of course, young and attractive. Together, they have two children to make up what a lot of us would consider to be the perfect size family. Tragically, something happens that interrupts and changes the story. It involves a monster named Batten Disease. This is so typical with Batten families.
What I see from a distance is what I often talk about. Batten parents are stuck in the middle of the muck and the mire as they watch the disease progress. They are in a battle named Batten Disease. What I see from a distance are the details of undying love and sacrificial care. The depth of love that is often modeled by these families is, for the most part, without equal. I HATE Batten Disease because of what it does to children and for the way it affects families. I do though, truly love these children and their families whether that is always welcomed or not. I am so happy that some very wonderful things have taken place in the area of research and clinical trials. Finally, some real hope is in the air but everyone who follows these kids will tell you that it has taken too long. I am glad that Breanna is involved in one of these trials. She takes your breath away. Does she not?
Nobody will impact this family like Breanna will. This, both with close family and extended. Her life is priceless. People will be changed because of her. You can count on that! Breanna, you keep fighting like the little warrior princess that you are! I could go on but I better stop now. Thanks for taking the time...
“It always stuns me that the Batten children are ALWAYS beautiful and glowing with such light and love reflected in their faces!! I seriously have a soft spot in my heart for every single Batten child. Rex, our very special angel, taught me a new kind of pure love.”
~Tina Halyama Emery~
Home Healthcare Nurse
The question was “why us” and it came from Christopher Velona. Most people know my long distance friend Christopher from Project Sebastian. People who know him, know that his son Sebastian battles the CLN8 variant of Batten Disease. You also know that Christopher has done everything in his power to help his son in the battle that is Battens (He, of course, has not been alone in this). One of the things that Christopher has done as of late is to start doing podcasts in conjunction with his other activities for Project Sebastian. I don’t know why he chose me to be his inaugural guest, but he did, and I was honored that I was chosen by him. We started the taping by talking about me and my wife Rosie’s life as parents to our special needs son Benjamin. Then we went on to talk about my involvement in the Batten community and why it is that I do what I do. I can’t remember exactly how he worded the question but it was something along the lines of, why? Why us, meaning the Batten community. Why am I doing what I am doing as an advocate? Why do I take the time to interact with Batten families, and why do I devote so much time to writing these stories in my blog? Why have I chosen the Batten community in particular?
The answer to that last question is that I didn’t choose to do this. It was just that through a set of very important circumstances that I first became aware of these beautiful kids that battle Batten Disease. Through this, a passion for them and their family’s stories was ignited. To me, it is so unlikely that I would learn about Batten Disease by following the legacy of a Navy SEAL, but I did. He wasn’t just any SEAL, although they are all amazing! Facebook and, social media in general, has taken a bad wrap at times. Sometimes, deservedly so. However, in many cases social media has been a force for good! A series of Facebook pages that led me to an underwater memorial dedicated to the legacy of this Navy SEAL would also lead me to becoming aware. My sensitivity towards people with special needs lended itself to a sensitivity to children with Batten Disease. I first began to write by writing about following this SEAL’s legacy all the way to his hometown. It was there that I also met a very special Batten princess. After that, one thing led to another.
I am a person that is driven by passion and once I started to write, the entire thing just blossomed into something special. This gave me a way to be involved in a community that I have grown to love and respect greatly. Greater awareness concerning Batten Disease was not my first thought but I think that the blog has helped with that. What happened was that I was so moved by the details of what I was writing that it continued to feed this new found passion that I have. Not only this, but the passion was fed by the appreciation that I have been shown by the families. I have repeated myself over and over again when it comes to the things that I see in the Batten community as a whole. That is so easy to do because it takes a certain type of character in a person in order to devote one’s self to the care of these children. Undying love in the face of adversity. I Understand that I am on the outside looking in and my perspective often reflects that. How difficult is it to move forward each day, knowing the eventual outcome? Deciding that you are going to do your very best in the worst situation, heartbroken and yet, resolute. What is it like to look into the face of your child each day? This, as you battle the disease together. These children seem more special than any other because of the circumstances that surround them. The whole thing just makes my heart melt inside of me.
These things weigh in heavily into this next story. There is more before we get started though, and it has to do with the Batten community as a whole. I have talked about this on several occasions. I was able to see from my activity online that the people that make up the community were very special to one another. Sure, there will always be some who will not get along but I think that this is in the minority. Being at this year’s Batten conference and seeing this group of people in person was an amazing experience for me. What is it like to experience the feelings of loneliness that comes with the diagnoses? You return home and eventually find out that you are part of a community that exist worldwide. There are others that know exactly what you have just experienced. They also know what you will go through in your journey. My next story is about a mom who went through great lengths, literally, to be at this year’s conference. This, only a short time after learning that her daughter had Batten Disease. Like many others, the answer came after a long search and several wrong diagnoses. She felt that it was so important to be at the conference and I can certainly understand why.
Laura Johnson had experienced the trauma of receiving the news that her daughter Hannah has Batten Disease and she wanted to be with others that could understand what she was facing. I had met Laura online through Facebook, of course. I knew that she was a Batten mom but I didn’t know much about her as we had just gotten acquainted. I saw that she had set up a gofundme account and that she was seeking help for getting to the conference. As it was approaching quickly, she still was not sure at that time as to whether or not she would make it to Nashville. As for me, I had made my way there and found myself at the Airport Marriott hotel. There were so many people at the conference and it would be impossible to meet everyone. I was able to spend time with most of the people that I already knew and it was especially nice to spend meal time together. As I was leaving after dinner on one of the first nights there, I took a random path through the dining room in order to exit. This particular path led me to a table that included a mom with her daughter and the mom greeted me with a warm smile. It was Laura and Hannah! It is very possible that had we not crossed paths at that point that we may have never met, but we did. I loved meeting them, along with many of the others, and we would go on to spend a few moments together on a few occasions after that.
One of the reasons why I do what I do is because I think so highly of people like Laura. She received what she needed to attend the conference just in time to get moving. Laura rented a car and her and Hannah would drive thirty hours straight to get there. To me, that is amazing as I don’t think that I would be capable of doing that myself. In the blog that I did about attending the conference, I had given the title of Tiger Momma to Laura. I meant to use her as an example of the many that represent moms in the Batten community. Moms that persevere and do whatever it takes. Always putting the interest of their child before themselves. Laura would tell you that she is just in survival mode and that she was just doing what was instinctive. I am telling you that what I see from the outside looking in is that she is amazing. Me and Laura talked about the blog and the possibility of doing her’s and Hannah’s story. To this, she said yes and so here we go!
Laura hadn’t been opposed to the idea of having children but she didn’t expect it to happen so soon. She and her future husband, who was serving in the military, had eloped. They had plans for saving and having a real wedding, with a reception, a year later. However, the news that she was pregnant with their first child changed all of that. Laura had never babysat or changed a diaper in her life and this left her worried. How was she going to care for a baby? After all, she wanted to get things right and so Laura read every baby book in the library in order to prepare herself. Talk about training through correspondence!
Having children and raising them is a life changer for sure. Laura had no idea at the time as to how this first baby would impact her life, but that was a fact in the making. She would deliver her baby at Balboa Naval medical center in San Diego, California as her husband was in the Coast Guard. They would have a baby girl and named her Hannah. The baby was born close to her due date and the day that she entered this world was September the 16th of 2003. It was after midnight that she would make her entrance and she was oh, so beautiful. She had all her fingers and toes and she was covered in brand new baby skin. Her first cry was loud and strong as she was so healthy! Neighbors were nervous as to how cool Laura was under pressure. Labor would take twenty hours and during that time, she could be seen painting her nails. This, because she didn’t want them to look bad when everyone came to see her newborn baby.
A small indication of what Hannah’s personality would be like came at the very beginning as they placed her on Laura’s chest. Hannah started to nurse right away. She would go on to be so strong and good at nursing that she actually would injure her mom in that way. Laura would have to endure this for a good year. This, because she wanted to continue nursing Hannah for as long as she could for the best start possible. The way that Hannah took to nursing would not be the only way in which she would show mom how quick she was. Hannah had a slight fever at birth and so the hospital staff kept her for three days. Seeing that they were new at this, Hannah’s parents liked this as they felt it would be good to have some back up early on. The nurses had wheeled one of those clear plastic boxes, used for a crib, into the room but Laura refused to allow Hannah to leave her arms. They would scold her for this but Laura stood her ground. Hannah was eight pounds and twelve ounces at birth and she was gaining weight already by the time they left the hospital. The nurses said that this never happens but it could be attributed to Hannah’s ability to nurse so well right after birth. Hannah would go on to be in the 99th percentile in height and weight her whole life. That though, was not the only way in which you could consider her to be exceptional.
Laura said that her baby girl was so striking although she was slightly annoyed by her daughter’s appearance. What? You see, she had carried Hannah all that time and Laura felt that she was supposed to look like her. However, Hannah was a mini-me to her dad. She had bright blue eyes and pitch black hair. Nevertheless, Laura loved her newborn baby and she was going to give motherhood everything that she had to give. As it is with most young military families, there is not a lot of extra money to spare. The young couple would leave the hospital with their newborn baby girl in a small Nissan truck. The three of them barely fit into it as little Hannah’s car seat took up a lot of space. They would take Hannah home to her first apartment which would be in Ocean Beach. This was in the greater San Diego area where Hannah would begin her life as a beach baby. Hannah would continue her development and she would have no fear as she grew. The couple would frequently take her to Dog Beach with them. Hannah was completely comfortable with the water and the huge dogs that were there. Again, Hannah was a big girl for her age. Laura said that the pediatrician would match up the rolls on her legs to make sure that they were developing equally. She joked that Hannah looked so much like a very small Stay Puft Marshmallow man!
Laura stated that Hannah would go on to meet most of her early milestones of development. The only exception was walking. In this area, she would not take flight until age fourteen months. Laura is quick to admit that, as a new mom, she didn’t know all the do’s and don'ts of parenting. This is how she would put it, “I was an overachiever mom, catching her when she fell, and giving her what she wanted when she grunted. I didn't know any better when I was a new mom.” Perhaps the only other thing that was noticed to be a little different in Hannah was in the area of her speech. Some of her words would come out sounding funny but that often happens with children as they are developing.
Laura said that Hannah would not tolerate a hearing test. This, to check to see if her speech problems were related to her hearing. As Hannah grew, it was apparent that she was extremely intelligent. Laura said that for example, she knew how to change the disc in the DVD player when she was still in diapers. It was noted by teachers that she was in the top of her class in preschool. Laura said that she had potty trained Hannah in two days. She was enrolled on Friday and started school on Monday in regular underclothes. No diaper! She would remain at the top of her class all throughout early education. You could only imagine the level of pride that was felt by all of those who loved Hannah. Not only was Hannah smart but she was determined and also beautiful. Here is more of what Laura had to say, “She was so smart and so beautiful, and also, very independent. She would never hold my hand crossing the street. I felt like a failed mom, not being able to get her to hold my hand.”
There was no reason to believe anything other than that Hannah’s future was packed full of potential. This, even with the slow start in the couple of areas that she would overcome. The future looked nothing but bright. Hannah was so sweet and so much fun from a very early age. She amazed her mother and all others who took notice of her. Even with Hannah’s independent attitude she was, overall, very well behaved. However, like the rest of us, she wasn’t perfect. Laura recalls one situation in which Hannah felt that she would take care of business when a situation in preschool arose. It happened after being picked on all throughout the day by a little boy named Tupac. It must have been at the final recess of the day. Hannah had thought about how she might handle Tupac and as a result she laid in wait at the tunnel that was next to the slide. She decided that she would clean Tupac’s clock and by doing so in the tunnel her crime would go unnoticed. She was successful in taking care of Tupac but somehow, the staff would find out and Hannah would wind up in the principal's office. This is where Laura would pick her up at the end of the day. I am sure that Tupac learned his lesson. Don’t mess with Hannah. How cute!
Hannah would also do the cutest things. Part of the families existence early on included having to move frequently. This was due to Hannah’s daddy’s military service. Hannah would observe her mom as she packed bags and dragged them to the front door. Hannah, being as smart as she was, would copy her mommy. It would be moving time and there was Hannah in her diaper, dragging her little bags to the door. There were many ways in which Hannah stood out as a child. One area was her ability to predict when women are pregnant. Laura stated that Hannah, in fact, had a sixth sense when it comes to this. According to Laura, Hannah, who was three years old, had informed her that Laura was expecting. Laura didn’t even know at the time but she was pregnant with Hannah’s little brother. In another case, Hannah had broken the news to Laura’s sister that she was expecting twins and she provided details with the news. She new that one twin would be a girl and one would be a boy. She also predicted that the girl would be born first, and she in fact was correct.That, is down right spooky!
Laura was not the type to just sit around. She has always been active in furthering her education by attending college, and various other classes. She would actually bring Hannah with her to classes and that shows real commitment. Laura attended so many classes that she started to stack up credits and would eventually apply most of them to an AA degree in Liberal Arts. It didn’t matter where they had moved to. She would be in school and gained all of her credits by attending as many as four different colleges. Moving didn’t stop her. Laura would later become very involved in physical fitness and she would, as an example, one day even become a Zumba instructor. She also would receive her certification as a volunteer emergency medical technician. The skills that she learned would actually help her save her own daughter’s life one time when Hannah was choking. Laura would admit, though, that it wasn’t a good career choice. This, because she isn’t good around blood. Yes, that is important in that line of work.
As Hannah continued to grow, it was easy to see her charm and beauty. She was so full of promise and personality. Her independence and strong will would lead one to believe that she would be successful in whatever she chose to do with her life. She was full of life and was so much fun to be with. Why would all of these great character traits not continue to develop in her as she continued to grow. I have stated the following so many times in the past. There is no love like that which exists between a mother and her child. I believe that to be especially true when it comes to a mother and her daughter. As one would expect, the bond between Hannah and her mom was especially close. Hannah would, however, have to share the love that was felt for her with a new sibling. Her new little brother James came into this world when Hannah was three and a half years old. Their little family now seemed to be well rounded and complete. A girl and a boy! Every family has its challenges along the way. Financial challenges, for example, are always a big concern. Health issues can be also. For children, there are always the usual bumps and bruises along the way. Operations to fix defects or maybe a broken bone may have to be set. Those things are normal and we expect them to happen. Although, we do not look forward to those types of events.
We normally think of marriage as being a lifelong commitment. I don’t think that anyone steps into marriage thinking anything else, however, things don’t always go as planned. People change and we are sometimes changed by circumstances that we don’t expect to happen. Kids are affected by separation and divorce but sometimes, it can’t be avoided. When it comes to these stories, the kids that battle Battens are always the focus but life doesn’t stop for the disease. By that, I mean that other things happen around us and often, they take place before a diagnosis is found. Sometimes things happen after the diagnosis, or even, as a result of the added stress. It is never my intent to assess blame or to decide who has the greater responsibility. The circumstances related to a divorce do impact a story greatly though when that story is about a child. Laura’s marriage was a troubled one and it would not last. The worst part was that she would find herself in a situation where she would have shared custody of the children. To further complicate things, custody was shared across state lines and from a great distance. The situation was a complicated one and it left Laura with only having her children in the summer and on holidays. Why would I even bring this up? It is because this story is about the love that exists between a mother and her daughter. That being Laura and Hannah. Much of the time, they were separated by several hundred miles of interstate highway. Hannah was less than five years old when this began. Her little brother was only a year and a half year in age. Oh, my gosh! How stressful this must have been for all that were involved.
Laura said that she would often return home to California with tears streaming down her face. A young mom without her children. I cannot imagine! I suppose that there is much more that could be written about Hannah’s life during the dark years of partial separation but much is lost because of the time away. I am sure that there are many sweet details that could be shared from the time that her and Laura had together. The focus here though, will shift to the challenges. It had to be at about the age of seven to eight years old that Hannah started to change. She started to exhibit signs that something was developing in her. Something that took her outside the normal range health for kids her age. Hannah was healthy when it came to illness. She didn’t catch colds easily, for example, as others might. There was, however, behavioral issues involved with her. Laura said that Hannah had a family practitioner that she was seeing while at her dad's house and that he had misdiagnosed her four times. ADHD was the first diagnosis. That one is usually the first to be thrown at kids who exhibit problems or behavioral issues. Then it was Autism and later, Asperger’s Syndrome. This happened over the seven years that Hannah was separated from Laura. During that time, Hannah was prescribed one medication after another. None of these solved the problem. Eventually, Laura would take Hannah to the Autism Assessment Center for Excellence in San Bernardino. While there, it was determined that Hannah did not have Autism.To be fair, doctors don’t always know what to do when the puzzle is a difficult one to put together. I think that the best doctors are those that are good at solving problems. They take a real interest in a child and they also know when the child needs to be referred to a specialist.
Hannah would experience her first seizure when she was eleven years old. This happened after being up late while having a slumber party. Laura said that the seizures were not very frequent but also, that she couldn’t be sure. This because Hannah was not with her full-time. It was about the same time, at eleven years old, that Hannah came home from her dad’s with glasses on. Why now? She had never had vision problems before. One of the things that caused Laura to become concerned early on was when she would ask Hannah to get something out of her room. She would return repeatedly empty handed and Laura chalked it up to a behavioural issue. It was, however, related to a loss of vision. Laura would find out later that Hannah couldn’t see. According to her, Hannah was hiding this from them because Hannah thought that they would love her less if she wasn’t perfect. Laura said that she, personally, was devastated when she found this out. I am sure that the situation was complicated by the fact that Hannah was living in two different places during each year after her parents separated. So it was over a seven year period that Hannah was either misdiagnosed or undiagnosed but things would soon change. Laura did not know what was taking place in her daughter. Whatever it was, it was delaying Hannah’s development. Laura kept investigating and would consult different specialists as she looked for answers. Something wasn’t right and her daughter was being taken from her by something. Something that she did not yet know existed. Meanwhile, Hannah’s father would become angry with Laura over the fact that she was not accepting the diagnosis from the out of state family doctor. Hannah was just being given medication and Laura knew that there was more going on than what was being discussed.
And, it was at this time that Hannah was becoming more difficult while at her father’s house. Laura said that as she started losing her vision she would get stuck on the way home from school. This, as she walked alone after getting off of the bus. She would even get stuck on the wrong side of snow berms. Hannah would call out for her mom frantically. You could only imagine that Laura was frantic as well. Knowing that her daughter may not be getting what she needed. Hannah knew what she needed, and that was HER MOTHER! Hannah would tell anyone that would listen. Teachers, doctors, nurses, anyone! She was always asking for her mother, as well as, trying to call her. As a result, she was just becoming hard to handle. My heart aches as I write this part. It was just the best thing for everyone involved that she would be with her mom full-time. And so, she was reunited with Laura! There is no love like a mother’s love. Especially when we are talking about a mother and her daughter. For whatever Hannah had going on, she needed special attention.
As has already been stated, Laura would not stop until she had the answer as to what was happening with Hannah. She continued to seek the help of specialists and that would eventually lead her and Hannah to a geneticist. Tests would be performed and the answer was found. Hannah had CLN3, Juvenile Batten Disease. So as it most often is with Juvenile Battens, there is a long search and a tendency to think that the conclusion is obvious, i.e., several false diagnoses. In saying this, I don’t mean to indict any one parent or health professional. Moms have a special intuition that cannot be ignored or denied. Laura had the answer that she sought but it was not one that any parent would want to find out about. The devastation that followed came from learning about something that Laura never knew existed. Batten Disease. This is how she put it to me, “It was truly traumatic. Nobody had ever heard of this.” She continued to explain in another place concerning her reaction and the effect that that the news had on her, “I couldn't believe it. I cried for two months. I felt just like I did when my mom died. I am not really here. I am just Hannah’s caregiver. I can’t hear what she is saying. I am grieving so hard already.”
Already grieving as if she had lost Hannah and yet functional in Hannah’s care. These comments come from a mom that would do anything and everything in order to help her daughter. I know from following Laura and Hannah’s story that she would regroup as best as she could and she now provides daily, the best quality of life possible for Hannah. Yes, emotions run high at times but it is so easy to see the love connection that exists between this special mom and daughter. Laura had a huge smile on her face when we met at the conference. There must have been a big mixture of emotions behind that smile. Her long drive to get to there shows a deep level of commitment to Hannah. It also shows the need to be connected to the others that make up the Batten community. This, because only they can truly understand what she is going through. Laura stated, in so many words, that finding a physician that truly cares is difficult to come by. They are out there though! She would meet one at the conference that she would love to have for Hannah. The fact that Laura found this doctor there at the conference speaks volumes.
The continuous driving that Laura would do in order to be with her children had conditioned her for the drive to the conference. It would be only a short time after Hannah’s diagnoses that they would find their way to Nashville and I am so glad that they did. I know that Hannah has her bad days and I am sure that she is often frustrated as she goes through changes because of the disease. She is so sweet. It was special to be able to meet these two. Hannah was quiet and soft spoken. I could tell that she was happy to be there and I know that she was made to feel welcome by those who cared for her. These, after all, are people that understand. Hannah was so happy to show off the things that she was making in craft time and she was such a pleasure to talk to.
These kids should be fussed over because they are truly special. What a dear she is! Hannah and her mom, as well as, the entire Batten community are the reason why I am writing at this time. It started with the love I felt for one little girl and her mom. This soon spread to an entire community and I feel privileged by every story that I am allowed to write.
Laura is a very bright and intelligent person. That is easy to see by what she has already accomplished in life. This leads to a question. What would Laura be able to accomplish if she was not struggling with Hannah’s disease? You see, this is the type of thing that I have talked about when I have mentioned the sacrifices that Batten parents are often asked to make for their children. This is especially true when we are talking about single moms that cannot work full-time, and sometimes not at all. For Laura, it is a struggle to pay bills and to keep a roof over her’s and Hannah’s head. She has been advised by some to put Hannah in a full-time care facility. Laura stated the fact that this is too difficult and too expensive. She would go on to say that which I already knew to be the true of her, “ I cannot give up. I will serve her selflessly until I die.”
Laura has gotten some really amazing moral support from her boyfriend who regularly spends time with her and Hannah. I love it when I here about men that step up to the plate (a baseball term), so to speak. There are so many men that would see a situation like Laura’s and say, no thanks! You already know how I feel about things and it is so nice to see another guy that feels the same way. What’s his name? You’ll never know because he chooses to remain anonymous. An unsung hero. Laura says that his life has been greatly affected by Hannah’s life. Just like I thought. Being around these kids can change a person. They make yours heart go pitter patter. That is if you are the right type of person. Life can be stressful in such a situation as Laura’s and her boyfriend chooses to stay in her life in spite of the stress that exists. Laura would actually tell him that he should leave for his own sake but he refuses to do so. She puts it like this, “I have asked him to leave, to save himself, for his own sanity. But he stays with me for mine.” He has even stayed although it may have kept him from maintaining certain career objectives. Together, they enjoy taking Hannah on outings to places like Lake Havasu for some fun in the water.
I have said it before but I will repeat. I believe that these kids come into people's lives for a reason. Then again, it would be so easy for me to say something like that. I am a proud parent to an adult special needs person but I have never experienced what a Batten parent has. The journey brings with it many special memories but it also brings a ton of heartache as changes take place. Laura had the following to say about her daughter, “Hannah is so bright and so determined. She would be a force worth reckoning with if not for this fatal detrimental disease. She was at the top of her class until the disease started taking effect. It is awful and nobody knows our pain, not even close family.”
In another place, she talks about Hannah’s resilience, “She has an active imagination and loves to play with toys. She does her own research on YouTube on batten disease and toys she would like to own.”
Laura enjoys working at a ranch that uses horses as therapy for kids with special needs. It is called T.H.E. center ranch (stands for therapeutic handicapable equestrian). Hannah received a scholarship after the nine months that it took to complete the process. She now gets to ride for an hour a week and Laura is working on becoming an instructor there. Her desire would be to become an instructor full-time. This, if Hannah’s situation would allow her to someday. What a rewarding career it would be to assist those who have special needs. All because she is the mom of a very special warrior princess! One might think that there aren’t any silver linings when it comes to Batten Disease. One of the few positives may be the people that you meet. In the course of the journey, you become part of a great extended family. One that understands what you are experiencing and feeling. Many of those friendships will last a lifetime. Laura had this to say about her experience and meeting people in the Batten community, “I had never heard of Battens until her official diagnosis. Not even a guess. I was shocked. I am never to be the same. Some of the people I have met from the Batten community have been life changing, they are so strong and so positive. I am glad to have been able to be blessed to have met them.” I have read comments like this from many now. In fact, I have witnessed it first hand. This truth, concerning the Batten community, is another reason why. Strength in the face of adversity.
Life is a struggle for Laura right now as she cares for Hannah. Yes, there are some good things happening as well but the challenges remain. Financial hardship is a big one for her. There is no way that she would do things differently but she wishes that things were not so difficult. I am glad that Laura has discovered the support that is available to her through the Batten community. I wish that there were more people outside of those who battle Battens that could step up. Someone like Laura is so deserving, and what about Hannah? Meeting her was a pleasure and she is so special! More than anything, these kids are the reason why I write. I love my special needs son so much and I have no regrets about having him in our home. What I see in the circumstances surrounding kids with Batten Disease is so much more challenging than anything that we face. Batten kids tug at your heart like none other. They battle so hard and show us so much about resilience and the indomitable human spirit. They often love and are loved in ways that others are not capable of. I know that it has taken me a bit of time but does this explain the reason why? I hope so. Prayers and best wishes to Laura and Hannah. I hope to see them again next year in Denver, if not sooner.
Blogger and Advocate
I don’t normally use this blog as a tool for fundraising. That as it is not my purpose in doing what I do with this blog. I am posting the link to Hannah’s gofundme account because this particular family is in great need. Laura is so deserving of any help that she gets and it’s Christmas!
She is a great mom and, as the saying goes, she is stuck between a rock and a hard place! This last statement from her says a lot about her as a person, “I feel numb. I am not sure how to feel right now. I am weak but strong for my child. I am humbled beyond words but willing to help others and have been volunteering to help with horses.”
Please help if you are able. Click on the link below:
“I knew Oscar’s time was limited and I wanted to share with everybody how amazing he is. I want his life to be filled with happiness, joy and love. It really gives you a new outlook on life when something like this happens. You realize how short life can be and how important it is to make the most of everyday.”
Oscar’s mum sharing
her purpose and
why she started “Oscars Story.”
I am not sure how it is that we met online but I am glad that we did. Like myself, Holly Linville had attended this years Batten conference in Nashville and she had been deeply affected by what she had experienced. Holly is a nurse by profession and she resides in the area around greater Nashville. She was asked if she would like to work at the conference and agreed to do so. She had never heard of Batten Disease up to that point. In addition to being a nurse, Holly is the mother to two boys, ages three and six. She loves what she does as a nurse and also as a mom. After making introductions and beginning our first chat, she described herself as being a true Empath, or someone who is able to show true empathy for someone. One page that I visited stated that Empaths are able to absorb other people’s emotions and or their physical symptoms. These people are highly sensitive. You can only imagine how the combination of being a true empath and a mom to young children affected Holly as she learned about Batten Disease. This, while working at the conference.
Of course, a light bulb went on in my thinking as I thought about using her experience at the conference in a introduction to a blog story. I asked and she agreed to help. I ended up sending her four or five simple questions to answer and then went on with other things. Time went on and I wasn’t hearing back from Holly. I have a no pestering policy when it comes to this blog. I leave people alone when I don’t hear back from them, and I move on to the next thing. This is such a sensitive area and I am truly sympathetic to that. Anyhow, it would be weeks later that Holly would get back in contact with me and she told me that she would not be able to help. Among other things, she said the following, “I truly admire your work. Truly. But I just don't think I can contribute anything positive to your writing.”
Holly had been affected by what she had experienced at the conference above that which an average person would have been. This, as she cared for these dear children. The following is more of what she had to say, “I have nothing nice to say concerning Batten's Disease. I think it is a cruel and devastating disease and I cannot wrap my head around what the children and parents have to go through. I really have nothing nice to say about the Disease.” I can only agree with her comments. There is nothing good that could be said about Batten disease. It truly is hideous. Especially because it impacts children. These kids are so precious! Being with these children and seeing things first hand actually sent Holly into a depression. She said that she would find herself crying at various times. It would happen while sitting at a stoplight (Um, I can relate to this) or as she lay in bed falling asleep. It also happened as she held her own children tight. In fact, she still cries when she sees social media posts about these kids.
She expressed that the whole thing was hard to take in. I think this is because she truly does take things in. She has the ability to internalize things. Holly said that she is a pretty capable writer also (not that I claim to be). I believe her. She shared more about her experience and while doing so, actually started to contribute the type of thing that I was looking for in the first place. Here is what she had to say as she brought a child and her mother, from the conference, into the conversation, “At the conference, I tried to do the job I was paid to do. That is to care for the children and keep them entertained. I was holding it together in the activity rooms. I was playing with the children etc. There was a lot of distractions.....kids playing, parents coming in and out etc. I was distracted and busy. Then I went back to the "nap" room and met Caleb Johnson. His mom said he was tired and asked if I could stay with him, as she wanted to attend a certain seminar. So, it was just me and Caleb. He started whimpering some, so I got him out of his wheelchair and held him like a baby. He was the most beautiful little boy I had ever seen. I couldn't hold it together any longer. It was just me and him. My tears started flowing and I couldn't stop crying. I just don't understand why this has to happen to these beautiful children. I can't imagine what their parents are going through.” Oh, my goodness! I couldn’t believe what I reading as she said this. Holly had already stated that she had nothing to contribute.
As the chat continued, Holly would continue to give me exactly what I needed. This, as what she had to say included her feelings about little Caleb and his dear mom, Ashley Johnson. Holly would also say some things that would relate very well to the mother and son that are the subject of our next story. This amazed me as she originally felt that she would not be able to contribute. In a few sentences, she would share some of the things that we’ve already talked about in this blog. Holly used a quote that she had heard previously in relationship to what she had witnessed about Batten parents. Here is the quote, "You don't know how strong you are until being strong is the only choice you have." Her observation was that Batten moms and dads are so strong! I have said, in the past, that these are ordinary people that have been asked to do something that is extraordinary. And yes, I do believe that in the process, they become stronger than they might ever have imagined they could be. This is, of course, an outsider’s point of view but I don’t think you could argue with it.
Holly had the following to say concerning Batten parents in general and Ashley in specific, “The love she has for him and trying to help him experience fun things while she still can. As with many situations in life, I think, for the parents, it must come down to either giving up all together or making the best of the time they have. Ashley has chosen to move forward and do the very best she can for Caleb. She gives it her all. Driven by pure love for her son, that is what keeps her going. She takes one day at a time and makes the best she can of each day. I admire her courage.” You know what Ashley? I do too! These are the types of things that we’ve talked about in the past. These are the things that amaze me! The bond that is created being one of those things.
There is another story in the UK that takes place and it relates so well to what we have just talked about. It is happening across the Atlantic Ocean, approxiamately 4,000 miles (6437 Kilometers) away from where the Batten conference was held. What Holly stated about a mom in the US is also so true about a young mum in the UK. Her name is Carrie Baugh and she is the mum of a most charming little man named Oscar. This story belongs to them but it also represents all of those, worldwide, who battle Batten Disease. Here we go!
Carrie was young, but that didn’t matter to her. She was just so excited that she was going to be a mum. This was something that she had wanted. She loved children and looked forward to it with great anticipation. Carrie was seventeen and had been with her boyfriend for two years. She described the day that she found out she was pregnant as the happiest day of her life. Her pregnancy would go so well. She had no sickness or weird cravings and everything went smoothly. The only difficult thing was that her baby was very active inside of her. In fact, the baby loved kicking her in the ribs. This, to the point that it made them sore. It became painful to her to have this going on but she felt that it was a good pain. This, because it was an indication that her future bundle was healthy. Carrie was so happy even though she was a little bit sore. She was going to be a mum and Carrie was very excited about this!
The smooth pregnancy would give way to some challenges though. This, as it was time to bring her baby into the world. She would do so two weeks earlier than expected. Carrie remembered that night like it was yesterday and recalled the events that started as she was sitting at her partner’s home. It was just like any other night until she began having cramps of a different kind. The cramps would begin to get longer and more painful. That is when the couple called the hospital. This all would take place on February the 4th of the year 2013. It was around 8pm that they were told to head to the hospital, and so they did. Things were not progressing very quickly and Carrie was dilating slowly. The pain, also, was not getting any worse so they started to settle down into their hospital room in order to get some sleep. This baby had other plans though and so Carrie’s water broke. From that moment on, the pain would become worse and things really started happening. She recalls that at that time, she was taken to the delivery room with her partner Jake. Carrie is loved by her parents and she is especially close to her father. In fact, to her, he is closer than any other. Her father had arrived and she wanted him at her side during this experience. How very special is that!
Carrie would work for a few hours, pushing, but they weren’t getting anywhere. She was losing too much blood and doctors felt that the baby was becoming distressed. That is when it was decided that it would be best to perform an emergency caesarean section. Carrie recalled that all at once, The delivery room was full of people, all with faces that she didn’t recognize. You can only imagine what a whirlwind of events this must have all seemed like and Carrie describes what it would all come down to, “The next part I remember is being wheeled out, back out into the ward, and standing over seeing Jake, my partner, holding our tiny little baby. He was so healthy and so beautiful and they placed him onto my skin and I felt so blessed. I always had wanted kids, I love children! And now I had one. I was aware how young I was, but I always felt like I was born to be a mother.” In fact Carrie was born to be a mother and now she was. Every mom, or mum, remembers the time that she delivered her very first baby and her life is impacted by that event. Carrie could not have known at the time, however, the impact that this particular baby would have on her life. She had a boy and he was the most adorable baby boy. They would give him the most adorable name to match and that name was Oscar. Oscar Luke Somerfield was born on February the 5th of 2013. What a beautiful name!
Life is all about learning lessons and that would especially be true for Carrie as a new mum. The first year with Oscar was a difficult one but Carrie would have her father around to help. As she would put it, “I was lucky enough to have my father around to help. He was brilliant and honestly, I don’t think I could of done it without him.” It was at the time that Oscar was six months old that Carrie and Jake split up and that would make things especially difficult. Carrie said that she was already learning to be a mum and now, she had to learn how to be a single mum as well. Oscar would meet all of his early milestones and this made Carrie happy as she watched her little man develop. He was so much fun to be with. Carrie said that he was the happiest little person around, and in fact, he still is! Can you tell by looking at him? Yes, I think so! The truly beautiful part is that as Oscar was growing up, Carrie was growing with him. She thought that being Oscar’s mum was the best job ever. Who could argue that was not the case? Carrie and Oscar were living with her father when she decided that it would be best for them to become independent. She wanted to set a good example for Oscar and felt that having a place of their own would help with this. Being a mum was important to Carrie and doing the right thing was as well. She wanted to support herself and her son while living on their own.
Carrie had never worked before but the time was now. According to her, she had left college when she had gotten pregnant with Oscar and had never gone back. Motivated by her love for her son she went out and got two jobs in order to afford their first flat. This all happened when little Oscar was two and a half years old. You would think that this would create a lot of stress for this young mum as it had to be a challenge. It was, however, in Carrie’s own words, “so wonderful”. Carrie and Oscar were really enjoying their new life and the time that they spent with one another. I was left with many images in my mind as she described their time together. She said that Oscar would help her cook up their tea and they would sit down together at meals, just the two of them. Then, Oscar would help Carrie pick out a movie to watch on the television as they finished their evening together. There were already many precious memories being formed together. No one can know what the future will hold as life moves forward but Carrie must have believed that her future with Oscar would be bright. What would he grow up to be? Where would all that personality take him in life? The possibilities were without limit.
Life would continue and as Oscar turned three years old it would be time for him to begin going to school. He would attend nursery class at the local primary school and this would be one of his first big life events. What should have been a time of innocent fun and early life lessons would become complicated by some issues that plagued little Oscar early on. Carrie said that it was at this time that Oscar was losing his balance while at school. She was receiving phone calls where she was told that he was falling over for no apparent reason and without cause. It was also noticed that he was not developing at the same rate that his little piers were. One example was that his vocabulary wasn’t growing. This all caused Carrie to become concerned, of course, and it was decided that Oscar should be seen by a health visitor. Once there, Carrie was told that Oscar was just slow and he would eventually catch up to the others. Mothers know their children like no one else can and Carrie knew that something more was going on in her son’s case. There was more to this than just being slow.
Carrie stated that things would escalate quickly and Oscar would have a seizure while at school. I am sure that this was frightening, no matter what. Tests would be run on Oscar and as one might guess, he was initially diagnosed with epilepsy. Carrie said that she was not surprised with the early diagnosis as many of her family members had epilepsy. Was this really all that was taking place in Oscar’s case though? The truth would find its way to the surface as events continued to develop. As Oscar’s mum, Carrie knew that there was more going on with him. As you would expect to be the case, she would seek answers for what was taking place. Carrie would not rest until the question as to what was really going on was answered. Oscar and his mum would return to see his health visitor and this time, Carrie would demand that her son be seen by a pediatrician. In response, she was told that it could take up to twelve months to get seen. What? How could that be? That is unacceptable by any standard. Whatever was happening, things were progressing too quickly and Oscar needed to be seen. Carrie would make the decision to pay in order to have Oscar seen by a private practice physician. This took place at Spire Cardiff Hospital in Cardiff, South Wales.
The search for answers most often brings with it a false diagnosis at the beginning. Many receive several as they seek to put the puzzle pieces in to place. Oscar was given the diagnosis of Cortical Dysplasia while at Spire Cardiff. This condition is a congenital abnormality of the brain’s development. This can be the cause of intractable, or hard to control, seizures. The quest for an answer as to what was happening would start to progress but receiving the answer could not come soon enough. Oscar was then referred back to Glangwili General Hospital so that he could be seen more quickly. Poor Oscar’s condition was getting worse as the days continued on and more appointments were made. Carrie said that the first few visits were mainly for blood work as the medical staff tested for many different things. By this time, Oscar was turning four years old and changes were taking place. Carrie stated that he had completely lost his mobility and he could not even sit up by himself. Oh, how difficult this must have been for Oscar, as well as, for all who love him. I have often talked about what a child’s life should be like. Innocent fun and happiness should be the order of the day. We hate to see it when something like this takes place. How was all of this affecting Carrie?
There were also issues taking place with Oscar’s eyesight. Carrie stated that they would learn that Oscar had very poor vision after attending numerous ophthalmology appointments. In fact, he had very little eyesight remaining at that time and it was due to the damage in the back of his eyes. Whatever was taking place, this was definitely more than simple epilepsy and I think that people understood that at this point. Carrie was in the midst of a journey with Oscar and with every new bit of information the heartache would increase. This is what she said concerning the news about Oscar’s eyesight, “This was unthinkable. We had no idea he would lose his sight. It was unexpected and heartbreaking. I tried my best to learn sign language to communicate with Oscar after he lost his speech. Now, finding out his sight is being taken from him was too scary. I didn’t know how I was going to communicate with my baby anymore.”
A disability team would be assembled for Oscar. This, as a result of his growing needs. The team would include an occupational therapist, a speech and language therapist, a physiotherapist, and a neurologist. In addition to all the services that he was receiving, Oscar also received a walker. Carrie said that this was “magical” because he was able to walk on his own for the first time in months. Unfortunately, this would only last a couple of months because at that point, Oscar was no longer able to stand at all. This must have been so discouraging. His lack of mobility would cause Carrie to ask for a wheelchair but again, she was told that it would take months to be seen. Oscar was of course growing and he could no longer be carried. Carrie would, once again, have to take action on her own. She went out and purchased a disability buggy and it would cost over 300 Euros. As a single mum, Carrie’s income was an issue with all of the added expenses that came with having a child like Oscar. She would apply for a Disability Allowance for him and would have to fight extremely hard to get it. Carrie said that the process took months because Oscar did not yet have a diagnoses. I can understand how difficult this must have been for Carrie. Still not having an answer for herself added to the difficulty of getting what she needed for her son. There was also the need for a specialized type of schooling for Oscar. Carrie would tell me that he was placed in an assessment school until space was available in a program that could more fully address Oscar’s complex situation.
As 2017 rolled around, there would be more challenges and increased concerns. Osar would have to go through much and watching it all happen was so difficult for Carrie. On top of the symptoms of Oscar’s condition, there were all of the blood draws that he would have to endure. Carrie said that drawing blood from little Oscar is awful. It is hard to find a vein and it is a struggle to get the blood out even when one is found. The initial testing was not performed because the samples had been lost. This meant a loss of valuable time. Of course that meant more blood work had to be done. Then again, a few months later, Carrie was told that the amount that was drawn was insufficient for testing. That meant more blood draws and Oscar would cry non-stop during each one. This was so heartbreaking for Carrie to watch and so frustrating as well. She said that all in total, Oscar had five to six lots of blood tests in the span of six to eight months. She also said that the amounts that had to be drawn were not small. I am sure that it was easy to lose track with the number of tests that required blood, however, the experience is difficult to forget. The blood was drawn in order to test for a number of conditions but there was still no answer as the end of 2017 was approaching.
All of the tests had come back clear but there was one test result for which the medical staff was still waiting on. That was for a condition that was named Batten Disease and this was something that Carrie had never heard of. As with everyone else that learns about Batten Disease, Carrie was made aware of the fact that the disease is rare and it is fatal. The fact that it is very rare often leaves some within the medical community unaware of its existence. Carrie said that Oscar’s consultant seemed to speak with uncertainty as he informed her about the disease. She went on to say that he only knew of two cases within the UK. Carrie explained what her thinking was at the time she was informed about the possibility, “They told us that this disease was extremely rare, and then I thought to myself, there is no way Oscar has something this bad. I was adamant that it couldn’t be this, so I was worried about the results. Something like this doesn’t happen to normal people I thought to myself. Oscar was just extra special and he would be okay in time.”
In fact, Batten Disease does happen to normal, everyday people. It happens to families from all walks of life and levels of income. It doesn’t discriminate for any reason. While it may be classified as a rare disease, it really affects many more children than one would expect with it being considered rare.
There would of course be other types of testing that would take place. An important one is an MRI and Oscar would undergo that test. As one might guess, he would would have to be anesthetized in order to have the test performed. All would go well and Carrie would return home with Oscar. It would be the end of last year that the doctors wanted Oscar to have another MRI performed and this one wouldn't go as well. Oscar would not recover well from the anesthesia. Carrie explained her response in part, “A moment like that, you don’t think will ever happen to you.” There is always that risk when anesthesia is involved and for someone like Oscar, who was medically fragile, it is an even bigger concern. That experience was, of course, frightening to Carrie as Oscar had to stay in the hospital to recover. She explained that her “heart dropped” as she saw Oscar struggle to recover this time around. MRI testing is always performed in a search that includes Batten Disease as a possibility. Doctors can generally see differences in a child’s brain that may cause them to look further through other means. The doctors could see that something wasn’t right with Oscar’s brain. They explained the things that were difficult to understand but it was obvious that Oscar had a major issue going on. Carrie recounted all of the testing that he had to endure in addition to those already mentioned. They included included Scans and Xrays, EEGs and 24-hour EEGs, Lumbar Punctures, and well, you name it! The results of the EEGs would reveal that an enormous amount of seizure activity was going on in Oscar. The poor guy had to be such a trooper and one has to wonder what he was thinking during all of this. Still, He smiled brightly on every occasion that he was able to do so. Such an adorable little man he is. What a little warrior and a handsome prince!
As 2018 came around, Carrie would have other concerns as well. She said that it was necessary for them to move to a different place. This was, in part, because she could no longer manage getting Oscar up and down stairs. Another factor was that they were not allowed to make any adaptations to their dwelling place that would benefit Oscar. Carrier said that they contacted the housing counsel to see if they could help with this. After a wait, of course, they were able to help Carrie find a new place for them to live and it would fulfill their needs. It had been fitted with a hoist for Oscar and it had wheelchair access. The only problem was that it was further away from family and friends but still, this made Carrie feel like things were falling into place. In addition to the new dwelling, Oscar was given a spot at a new specialist unit at school in March of 2018. This was huge for him and Carrie said that the staff there gives Oscar so much love and respect. They are wonderful and provide him the bests days that he can have! But still, there was not a complete answer as to what was going on with Oscar. That would soon change though and the change would happen as a letter was received in May of 2018.
Carrie was informed through the mail that Oscar had an appointment with the pediatrician at Glangwili on the 22nd of May. They also had an appointment scheduled for a week later at Cardiff Hospital to see Oscar’s neurologist. They assumed that the purpose of the later appointment at Cardiff was to discuss the findings of Oscar’s test. It was, however, at the earlier appointment with the pediatrician that the outlook for little Oscar would be changed forever. This, much to everyone’s despair. Carrie, and company, had arrived early with Oscar and had taken a seat in the waiting room. Much to her surprise, they were called in to the appointment room in only a couple of minutes after arriving. This was just supposed to be a routine appointment with the pediatrician.
However, immediately after arriving, the doctor informed Carrie that the results were back and that through gene testing it was determined that Oscar had the CLN7 variant of Batten Disease. Carrie described her response and what she was thinking as she was given the news,
“It was true, it was all true and it was real. He had this horrible disease that none of the doctors or us knew anything about. I was wrong, things like this do happen to every day people, it was happening to us. To my Oscar. My heart dropped and I felt one tear drop from my eye. All I really wanted to do was crawl up into a ball and call my dad. I felt like a little girl again, scared and afraid.”
And yet, Carrie knew that for the good of Oscar, she had to Continue on with the appointment. Her role in Oscar’s life would become even more vital than before. She continued to tell me about the appointment,
“I knew I had to listen to the doctor and take in information as it was so important to know exactly what we can expect and the next steps moving forward. So, I wiped away my tear and pulled myself together. I asked as much as I could about this disease and the doctor told me all he could. I looked over to Jake (Oscar’s father) and he was silent. I could just tell he was as heart broken as I was. In a way, it wasn’t a surprise as we knew it was the only thing they were testing for now. But also, it was so unlikely that I almost made up my mind that he didn’t have it. Now I was wrong.”
Oscar has Late Infantile Batten Disease(CLN7). It is the gene that has been identified by researchers as CLN7 that is responsible for this variant. Both Carrie and Oscar’s father Jake were carriers of this gene and that is why the disease is rare. To the best of my knowledge, CLN7 Battens is one of the most rare variants of the disease. This is an aggressive form of the Battens and as far as I know, there is not a clinical trial that is yet available. Oscar and everyone that loves him would find themselves to be on a journey. He would become a warrior and a prince of a rare kind.
With receiving the news about Oscar, Carrie wanted to share his life story with others. This, in order to let people know what an entirely special little man he is. He is so very special. Just look at that smile. It is undeterred, even with everything he faces, and it is one of his trademarks. Carrie started a blog page on Facebook and she called it “Oscars Story.” She keeps people informed about events in Oscar’s life while sharing the struggles, as well as, the joy that he brings to people that know him. Talk about on the job training. As Oscar’s mum, Carrie has certainly been through much. Carrie said that the months following the diagnosis were “hectic”. According to her, everyone got involved as the number of Oscar’s appointments only increased. These were with specialists and Oscar’s palliative care team. Dealing with the issue of palliative care was especially difficult on Carrie and she explained why that was, “This was hard. We had the talk about an end of life care (plan). Oscar was only 5. How could we possibly be planning end of life care for him? It didn’t seem real, but it was. They told us that the sooner he had one in place the better, so we needed to think it over and just prepare for it. Again, how can you prepare to put a plan in place for Oscar’s end of life? I was feeling exhausted and stressed.”
In fact, Carrie began to have issues with her heart because of the stress. The doctors would give her the “all clear” on the health of her heart and they told her that she needed to stress less. Her feeling in response was “how is that possible?” The good thing is that Carrie had since begun a new relationship with her partner Simon who was around to help her with many of the physical tasks. He was also a big help mentally for Carrie.
This was a good thing because the challenges with Oscar became more complex. The following describes what she and Oscar’s father Jake would have to endure (not to mention Oscar). It also gives examples of the types of things that are faced by some of those who care for these beautiful children (they are so beautiful). This is all of what Carrie had to say, “Oscar began to stop sleeping, he was jerking A LOT, and he started to develop some choking issues. He was missing a lot of school and he couldn’t manage his food properly. Giving him all his medicine was a task (and he had a lot). He has medicine for his muscle stiffness, seizures, reflux, sleeping, dribbling, and constipation. Oscar was then given an Ng tube. This alone was terrifying. Me, Jake, and Jake’s mother all had to learn how to give medicines, do flushes and feeds, etc. We had to look out for important signs of infection or any issues like the tube going into his lungs. I was petrified. If I got anything wrong, it was my fault. And I couldn’t do that to Oscar. But then, two months later, I could do it with my eyes closed. You certainly get used to it, and now Oscar can have his medicine and nutrition without an issue.”
In addition to all of this, Oscar was pulling out his tube on accident quite a bit and this meant trips to the hospital at Glangwili. This, in order to get a new one inserted. It would happen mostly on the weekends and while he was at his father’s house. Is Oscar worth all of this trouble? Dear God, yes! Just look at him. Whatever it takes!
What are the things that we have talked about in this blog? The things that we see in these families that face Batten Disease? Each story begins with the joy that comes with welcoming a child into the world. Sometimes we touch on the expectations that one might have for the life of that child. Then, things begin to happen that no one would expect to happen. There is, all of a sudden something very wrong and a search for answers takes place. Sometimes that search takes a very lengthy period of time and sometimes the puzzle is put together more quickly. The search takes place while the child suffers setbacks and symptoms that leave people confused and dismayed. When the answer is found it brings with it devastation that has to be experienced in order to be understood (I don’t mean to be overly dramatic, but this is really the truth). Devastation gives way to determination to do everything that is possible. This, in order to provide the best and brightest quality of life. To provide the happiest and most loving home. To make the most out of the worst situation while making the sweetest memories in the process. All, while remaining broken hearted. Through this, we observe undying love and a bond that is so strong and so sweet (Such is the bond between Carrie and Oscar). We see ordinary people that are asked to do something that is extraordinary. This is not even to mention what we see in these children who become little heroes. In them, we see resilience and the display of the indomitable human spirit. What we see are little (sometimes a little older) warriors that can subdue us with their innocent smiles and laughter. All while they battle so bravely. All of this is what we see in the life of Carrie and Oscar, Just like we see it in Ashley and Caleb. These are things that have been observed recently by my friend Holly and these are the things by which she is so deeply moved. I am amazed when I watch from a distance the things that to others may seem like just a matter of survival.
Oscar’s needs continue to increase in complexity. Carrie was waiting on a new wheelchair for Oscar at the time that she sent me their information. She was also waiting on more testing before Oscar recieves a PEG. Carrie tells me that this is a tube that carries nutrients and medications directly to the stomach lining. You see, Oscar has had a terrible time with reflux issues. It is so difficult to consider that an end of life plan for Oscar has to be put into place but, in fact, it does. I just cannot know what it is like for such a young mum like Carrie to have to go through this. I am glad that she is surrounded by people that love her and Oscar. What a character builder this is. What a wonderful mum and person Carrie is as she has set aside everything in order to care for Oscar. Is he not adorable? He sure is! The following are some of the things that Carrie had to say about Oscar and the love that she has for him,
“He is the most beautiful child in the world. He is always happy, despite everything he goes through and he is a real hero. He is my hero. He has taught me so much, and changed me into a better person. This disease is awful, but it makes him ten times more special️.”
“Most parents regret not spending enough time and appreciating these moments more as their children grow up so fast. I was given the opportunity to love him so much, and to appreciate every single day I get with him. If there’s one good thing to come out of this, it’s that.”
“Our future is still unknown. We know Oscar has got a short life. And every time he loses a skill, or deteriorates a little more, I’m reminded of it. But what I do know is that I have some time left with him. In that short time I will spend as much time as I can with him and make the most of each day. I will try to make Oscar smile and laugh as much as I can, and I will remember these happiest moments we make together, forever.”
Carrie Baugh, you are incredible! There is no way that I can capture all that is taking place in the life of this amazing little man and his mum. The varied emotions that are felt, as well as, the many stresses of dealing with the disease. There are also many sweet details that I would not have room to list. I did my best, however, and there are some things that I am absolutely sure of. The impact of Oscar’s life on Carrie is one that will last a lifetime, and her own experience is one that others will benefit from. I am convinced that while these kids lives come about through a rare set of circumstances, they are by no means an accident. They have real purpose in coming to these families at the time that they do, and their impact will be felt forever by those who love them. They are truly amazing! These are the things that we’ve talked about and they are the things that I will continue to make people aware of as I have opportunity.
Blogger and Advocate
“There were no awkward stares or whispers. Everyone went about their business around us. As I was kneeling beside Kristiina and comforting her, one of the mother’s came over and squeezed my shoulder, asking if there was anything she could do or help with. I soon came to know that wonderful, sweet woman as Laura Betts. For the first time in forever, this felt ok! I didn’t feel like the freak at the circus everyone stares at. We were totally overwhelmed by the warmth and acceptance of everyone around us. It changed our perspective of our situation for the better, forever! And finally, we didn’t feel like we had to do this alone anymore.”
While attending her first
I may sound like a broken record to some. This, because I tend to repeat myself many times over from one story to the next. I do this for a reason. It is because I want to place emphasis on the things that I feel are important to mention. Some people that read these stories, do so on a regular basis. Some even wait for the next one to be written. There are others that scan each story and look for the highlights, while others read them in their entirety. Perhaps some look on to check out my work. This, to see if my comments are based on fact. Others, just know about the blog’s existence and are happy to know that I care. I do care greatly! I am happy to have people viewing the blog for whatever the reason they have chosen to do so. Greater awareness was never my primary reason for doing what I do although It has helped with that. My purpose has mainly been to honor those who battle Batten Disease, as well as, to share the struggle that is associated with it.
I am at the point where doing this has become a part of who I am. The work that I do has never been for profit although I have gained much from writing these stories. These kids have taught me that I love to write. (Whether or not I am well written is up for the readers to decide). I started writing about these priceless warriors as the result of a chain of events in my own life. It is so easy to create dramatic occurrences through the written word and I don’t want to do that here. Simply put, I became aware and one thing led to another. The events that followed changed my way of thinking. Anybody that knows me, knows how much I love my son who has special needs. As I write this, I am leaving in a couple of hours to go out on a work trip for several days. I hate leaving my guy behind, as well as his mom, but I do it because I sometimes have to. Just knowing he is in the next room gives me comfort. What would it be like if I no longer had him there? Our time together is always priceless. I have no idea as to which one of us will make it to the finish line first because his outcome in life is unknown. What if I did know though? What if a doctor had sat down in front of me and had given me the news? The news that my child has a terminal disease for which there is no cure? That is what a family faces when they are told that their child has Batten Disease.
The climax of their story brings with it the devastating news that they will one day have to say goodbye. How can it be that there is no cure? Change is taking place but it seems to be happening at a snail's pace. Each of these stories contains the dramatic outcome of a search for answers and I include these details on purpose. The initial shock and grief that is so understandable eventually gives way to determination. This, to do one's very best for their child in helping them to battle Batten Disease. I am always amazed by the details of undying love and sacrifice. These families are made up of ordinary people that are asked to do something that is extraordinary. All of a sudden, these families find themselves being part of a community that was previously unknown to them. While it is unimaginable for most of us to go through such an experience, it is understood by those who make up the Batten community. Once the community is discovered, these families become part of a larger group that understands what each are going through. All of a sudden, they are no longer alone and in a state of confusion. The heartache is still there but they are no longer alone. That is what my friend Monica found out at the time that she attended her first Batten conference. I was allowed to attend this event for the first time this year and it was an experience that I will never forget. These kids are absolutely priceless and I am amazed by the stories that these families create by their caring acts of love and affection.
This next story, like the others, is about a family. It is a story about a community that they would discover after a search for answers. What once seemed like a problem that could be solved would actually become a journey that they would take together. Out of these circumstances, a princess of a rare kind would become the center of their attention and her life would change theirs as could no one else's. Monica Perreault’s life would take a course that she never would have thought about. She would experience events in life that one could never imagine. We all experience things in life that are good and bad. Things that are difficult and that bring challenges that are not expected. There are things in life that test us and that require courage. Events in life change us and they make us who we are. Sometimes, other people benefit from the changes that are made in us. Those changes may be affected by a single person and very often, they are. Things usually begin as two people meet and that would be the case for Monica as she met her future partner Robert. They would meet in 1992 through a mutual friend and they began to date. This would take place in Scarborough, Ontario which is in Canada. This is the place where Rob and Monica grew up and it is the place that this story begins.
They were young and It didn’t take long for Rob and Monica to fall in love. What almost always follows is the desire to have a family together. Such was the case for this young couple and their heart’s desire came in the form of a baby boy. Their first child, which they named Matthew, was born in October of 1993. They had a baby boy and they were so excited as they welcomed him into the world. This young couple’s life together had started to take shape with the addition of little Matthew into their home. There would be challenges ahead but they felt that they were ready to handle all that life would bring their way. Rob and Monica had recently moved a few cities away from family and had tried to make a go of it. They found, though, that they missed being close by. That is when they made the decision to move back home. They went back to Scarborough and moved in with Rob’s grandmother who they lovingly referred to as Nanny Cassels. It would not be long after that when Monica received some exciting news. She was pregnant and the best part was that she was going to have a daughter. How exciting this would be for any young mom. Their little family would be complemented by the addition of a little girl, and they had the perfect name in mind.
The time came for the family to welcome their next little bundle into the world. Baby Matthew had been born by C-section but it was time to give a normal delivery a try. Monica would endure five hours of trial labour when it had become too much for her to bare. With a sense of urgency she would tell the the doctors, “I don’t want to do this any longer! I can’t wait another minute! Get her out!” Monica stated that the delivery was a little bit rough as she had a reaction to the small amount of morphine that was in the epidurals. As is the case when you deliver a baby, it is always a labour of love, and the the birth of Kristiina Lynn Cassels was no exception. She was born on the day of May the 15th in 1996, and she came into this world as pure perfection. Kristiina was born at Ajax-Pickering Hospital in Ajax, Ontario Canada. She was delivered, as her big brother had been, by Cesarean section. That means that she was perfectly shaped, and oh, so beautiful. She had all of her fingers and toes and she was covered in brand new baby skin. She felt small in Monica’s arms at eight pounds, 11 ounces. This, being almost three pounds lighter than brother Matthew. Monica had to have been beaming with Joy as she held her baby girl for the first time. Rob and Monica felt that they had the perfect size family now, and they had a boy and a girl! They must have had thoughts and feelings about their future life together with their two beautiful children.
Kristiina’s name, which uses the letter i three times, was not a misspelling as the spelling had reason behind it. Monica reflected concerning her daughters name, “We had chosen to spell her name differently than most to reflect her Finnish background on my side of the family. Her name was perfect and unique, just like her!” Kristiina was unique from the time of her birth and her life story would remain that. She was perfect in appearance at birth and she is, to this day, uniquely beautiful. Monica had no idea, at the time, as to how this little person would impact her life but that impact is being formed continuously. Monica stated that Kristiina was a much different child than her brother Matthew was. She had, in example, a problem with Colic. Kristiina would cry a lot and she was hard to get settled down. Monica joked that she had learned the fact that the sound of a vacuum cleaner calmed Kristiina. This would quiet her down and she described what she did as a result, “Needless to say, I vacuumed A LOT! LOL!” As Monica’s little princess continued to grow, she graced the household with her presence. She was reaching all of her milestones within the normal timeframe and she was developing just like any other normal child would. Monica told me what she had seen in her daughter and it would be wrong to not let her tell you what that was. This is what she said, “She was bright, delightful, and had an immense amount of energy! She had a giggle that touched my heart each time I heard it. Her eyes were so blue that I used to drown looking into them. She was my princess!”
Life at this house continued to move on in the normal fashion. Rob would continue to work full time in order to provide for the family while Monica looked after the children. Things were working out for them as they continued to grow as a family. They would move into their own semi-detached home in September of 1999, to a place name Brampton, Ontario. The couple would ask Monica’s parents to move in with them. This, in order to make things more affordable and to receive help with raising their children. Life was overall quite good for this young family and Kristiina was on target in her early childhood development. It was, however, in her third to early fourth grade year that she started to develop problems with reading. She was also having trouble pronouncing certain words. Monica stated that one example is the word binder. Kristiina would instead pronounce the word as blinder. They didn’t panic over this as Matthew had similar struggles early on. Monica said that things would straighten out in Matthew and that it would happen as if “someone turned on the switch”. Monica thought that Kristiina had inherited the issues with pronunciation from her. This was because she herself had spent many years in speech therapy. Their little darling would continue her education in regular school through an education plan that would accommodate her needs more fully.
We are all changed by the circumstances that surround us. Things that are good, and also those things that are life’s struggles, can change our situation as they impact our future. The same would be true for Monica and her family. It would happen as they would experience the loss of her father in November of 2003. This would shake up anyone and I am sure that the entire family must have been greatly affected by the loss of Matthew and Kristiina’s grandfather. In fact, Monica’s mother and sister would move from the family’s home by the following July. This, because the memories of what had happened became too much to bare for her mom. This would, of course, require adjustment, and that is something that happens for most of us from time to time. Life would continue for a while but then an event would take place that would be a sign of what was to come for this family. It happened as Monica was returning home from working the night shift. She had since taken on a job of her own in order to help out with the family budget. It was December the 6th of 2007, as Monica returned home, that she did so to the sight of emergency vehicles in the front of her home. Monica describes the scene as she returned home, “I came home to police cars, fire trucks and an ambulance in front of our house. I remember time standing still as I got closer to the house and panic rose in my throat. Our next door neighbor met me getting out of the car. It was then, my worst fears came true. They were all there because of Kristiina!”
The image that this leaves in my mind is so moving as I think about the love relationship that exists between Monica and Kristiina. According to Monica, Kristiina had fallen and had gone into a seizure that morning before she had returned home from work. This, as she looked out the front window, waiting for Monica to return. Testing was run on Kristiina after she was rushed to the hospital for follow-up. All of the results came back normal and she was released to return home with her family. It was, however, only days later that Kristiina would have another seizure. Further testing was done, but again, it came back without any findings. Monica said that as the third seizure occurred, they demanded that Kristiina be seen at the Sick Kids Hospital in Toronto, Ontario, Canada.
Yet again, the testing came back showing everything to be normal. Kristiina had an aunt on her father’s side of the family that had a seizure disorder. The doctors felt that the two conditions must be related but still, this left the family feeling helpless and confused. This was their princess. She was so much the center of their world. How upsetting this must have been! The only positive that came out of this was that Kristiina was referred to a pediatric neurologist. Kristiina would see a neurologist at Trillium Valley Hospital in Mississauga, Ontario, Canada. Dr. Conrad Yim turned out to be extremely qualified in handling Kristiina’s situation as he was able to get the seizures under control quickly. She would, however, continue to have breakthrough seizures even while on medication. Monica described how all of this left them feeling as they witnessed the seizures, “They were hard to watch her endure and left us all exhausted. They were so unpredictable and as a result Kristiina suffered many falls and bruises.”
Life for a family often becomes more complicated in general as the years pass by. Throw into the mix of events a child that is suffering from recurring seizures while not having an answer for why it is happening. Well, that can create some real stress in a relationship. All of a sudden, you have this one person that requires so much attention and as a result, other relationships may suffer. Whether or not that was the complete story in Rob and Monica’s case, it was definitely a huge factor. It was April in 2008 and by that time the relationship between Rob and Monica had broken down. According to her, she and Rob had become much different people. It is sad when this type of thing happens but the reality is that it does. This part of Monica’s story, though, would eventually have a very happy ending. In the course of time Monica would meet someone that would be the perfect match for her. That being her future husband Adam Bearman. The relationship started at work in friendship,, but as Monica learned more about Adam her feelings for him would deepen. Monica was struggling with her situation as far as Kristiina was concerned. Having a child with special needs can be so difficult and it often takes the right type of person to deal with the circumstances. Adam was that type of person and as time went on he would show Monica much about her situation. You see Adam had been around people with disabilities for a good portion of his life. In fact, he had even played wheelchair basketball for almost four years. Monica stated that Adam was almost more comfortable around Kristiina’s seizures and special needs than she was. People always come into our lives for a reason and such was the case with Adam coming into Monica’s life. Instead of complicating the situation, he would make Monica’s life easier. There is more that could be said here but in concluding what Monica had to say about Adam, she stated that his own experience was responsible for helping to “change her way of thinking.” Monica would elaborate concerning Adam’s help as she told me the following, “He (Adam) told me ‘the only limitations Kristiina has, are the ones you place on her’. It changed things for me and in me. I looked at what was going on with her differently. I stopped wrapping her in bubble wrap (figuratively speaking of course!) and I let her do the things she wanted to. He helped me to let go and stop molly-cuddling her and it allowed her to become independent and grow.”
Adam and Monica would continue to face the circumstances surrounding Kristiina’s situation together. The puzzle pieces that were an actual diagnosis for Kristiina were yet to be put into place. Adam showed great leadership, unconditional love, and support during the time that it took to find an answer. He was by the families side, and they would not be alone in the struggle.
Monica would make the decision to move with Kristiina to Burlington, Ontario Canada. This because she felt that the school system there had opportunities that were better suited for Kristiina’s needs. In fact, Kristiina loved going to school in Burlington. She made new friends and she had fit in well in her new surroundings. As 2009 rolled around, Kristiina was also doing well when it came to seizure activity. Monica said that at that time seizures were few and far between. They were checking in with the neurologist every six months and her EEGs were showing that no seizure activity was present. Monica stated that Kristiina was so healthy that she was getting sick less than anyone else. She was helping with the dishes and she was so happy. In fact, the whole family was happy and Adam and Monica were happy together! Things would change as Kristiina turned fourteen years old, and as she started high school. Stress and overstimulation would prove to be triggers for seizures in Kristiina. According to Monica the stress of high school started taking its toll on Kristiina. She started having seizures every other month or so. This number was a lot for her. Monica said that the grand mall seizures were especially hard on her daughter as they left her exhausted for as much as 24-hours. She would go on to tell me that it was at about this time that Kristiina was showing signs of regression. The regression would be followed, after a while, by a slight improvement in the affected area. It was obvious that Kristiina’s situation involved more than just a simple seizure disorder, but what was it? A possible diagnosis was mentioned several times but none of them could be proven to be correct. Hereditary Seizure Disorder and Globally Developmentally Delayed were two terms that were brought up as possibilities. Monica said that the possibility of crushed blood vessels in the left frontal lobe and oxygen deprivation were used many times as reasons for Kristiina’s delays. How frustrating it had to have been to not know the answer for what was happening and yet, to have to watch these thing take place. It is very possible that feelings of isolation may have set in as well. This because of misunderstanding by those that are not affected.
Adam had brought a daughter from a previous marriage to the relationship and her name is Sammantha.Together, Kristiina and Sammantha would become big sisters to another beautiful baby girl. Her parents would name her Khloe Lynn Bearman and she was born in April of 2012. Monica stated that the bond between Kristiina and little Khloe is indescribable. She went on to say that it is beautiful to behold. I get the feeling that this entire blended family was extremely close to one another from the beginning. It appears that the difficulties that have come with Kristiina’s condition have only served to make them closer to one another. That is the way that it should be!
It was early December of 2007 that Kristiina had her first seizure and much had happened since then as 2014 rolled around. This is when the neurologist was prompted to have further testing done on Kristiina. He had blood work and metabolic testing done, but Monica said that all of this revealed nothing out of the ordinary. He would continue to have EEGs performed, and this time, the results came back abnormal. This would cause the doctor to dig deeper. He wanted to have genetic testing performed. The search for an answer as to what was taking place with Kristiina was starting to narrow, and soon, the family would know. The puzzle pieces would soon be put together. This, much to the family’s dismay. Monica stated that a call to Toronto Western Genetics Lab was made, but they were told that there was a years wait to get tested, How could that be? She would go on to say that there was no way that she would wait that long. I wouldn’t have either! Monica did an online search for another lab, and she found one named Hamilton's McMaster Genetics Lab. She asked how long the wait for an appointment was in an urgent situation. The lab contacted Kristiina’s neurologist to verify her information, and still, it took four months before blood samples were taken. This, at least, was better than waiting a year. It was in April of 2015 that samples were submitted at McMaster Hospital in Hamilton, Ontario Canada. It is hard to believe what would follow and the time that this family would have to wait to get an answer. Life always moves on whether or not we want it to and to remain patient is so difficult. This, when you are facing a challenge such as this family was at that time with Kristiina. Adam and Monica were informed that it would take, in all likelihood, until December to get results from the test. This was because it would take four to six months to get government approval for the genetic testing to get paid for. Oh my! Yes, Life would have to move on as they waited.
Something really exciting would take place as they did wait. Adam and Monica would become man and wife! Adam had been there unconditionally and he would continue to be the rock of the family. It was in December of 2015 that the couple would tie the knot officially. They did things all fancy like, of course, and princess Kristiina would have her place in the ceremony. She would be one of the bridesmaids and would be dressed like the princess that she was. Monica said that Kristiina beamed proudly while wearing her red dress. I just bet that she was a sight to see! As this was happening, it was also time to inquire about the results of the genetic testing that was done on Kristiina. Monica felt that it was time, but the genetic counsellor wanted to consult with Monica, as there was a problem with the testing. You see, the samples had become untestable. They had somehow become contaminated. Perhaps by improper storage or by being stored at the wrong temperature. Can you imagine the level of frustration that must have risen as a result of hearing this? This meant more blood would have to be drawn and the family would have to wait again for the results. Fortunately, they had previously gained the approval for funding of the cost. Even so, as April of 2016 rolled around, they would have waited a year to learn the results of the testing. At this time Kristiina was 19 years old, soon to be twenty and Monica stated that the entire experience had been a real rollercoaster of emotions. Adam and Monica attended the appointment with Kristiina also being present as they received the news. Monica stated that she should have known that the news wouldn’t be good by the way it’s delivery started. The geneticist and counsellor sat down in front of the family and here is what was said between them as recounted to me by Monica, “Uhh.. Yeah. The results are back.. and we found the reason.” I remember saying “So you know what’s going on? You have an answer?!” And she said “Yes. It’s a rare, neurodegenerative, lysosomal storage disorder.. (pause).. fatal, incurable.. pause.. disease. Batten’s Disease, specifically CLN2. It means the brain cells don’t dump the waste, and it builds up and the cells die resulting in the regression of skills that Kristiina is experiencing”.
And there it was. The answer for what was plaguing Monica’s princess. I think that most parents believe that they are facing something that is fixable once the cause is determined. At least, they have that hope within them until they hear the news that brings with it pure shock and utter despair. Monica puts their reaction into her own words with the following, “None of us had ever heard of Batten’s Disease. My brain stumbled over those words one by one in order to understand what it all meant. Wait! fatal? That means resulting in death! Incurable? That means there’s no cure! My eyes stung with tears. Adam excused us from the room. My daughter was just issued a death sentence. I was devastated!! Thank you God for Kristiina not understanding a word of that appointment! I had so many questions! How? Who does she get it from? (the answer was) It’s a recessive gene. She inherited a copy of my rare gene and a common copy of the gene from her Dad. They went on to explain more stuff which just sounded like babble to me as I was too preoccupied in my own thoughts. They were both very sympathetic to our situation and offered to do whatever they could to help.”
Kristiina was diagnosed as having CLN2 Battens, or Late Infantile Batten Disease. This, even though she was quickly approaching twenty years of age. In her case, she was defined as being atypical. This, because in most cases, this variant of Batten Disease strikes people that are much younger, and therefore, are young children. At first, the geneticist thought that she had Juvenile Batten Disease because of her age, but the results did not lie. The doctor and counselor proceeded to furnish Adam and Monica with some information on the disease that they had put together and directed them to some websites that they felt would help. They also said that testing could be performed on other family members when they were ready. Can you imagine the rush of emotions as their world was quickly turned upside down? Monica continued to share what she was feeling at the time as she stated the following, “ I had not been prepared for this diagnosis at all. Not just the diagnosis but also what would happen to my princess. She would go blind, she would lose the ability to walk, she would lose the ability to eat and then eventually die!! I hid from the world for a few days as I tried to gather the strength and courage I needed to go on.”
Through everything, Kristiina would continue to be her normal cheerful self. Being a parent to a special needs person, my heart just naturally goes out to Kristiina and her family. Seizures and delays are things that we are familiar with, but the awful diagnosis that is Batten Disease is something beyond what we have experienced. It had to have been so terribly difficult to receive the news that Kristiina was a Batten warrior. I am sure that breaking the news to others while watching their response was a challenge as well. They first broke the news to Monica’s mother and her sister Janet. Then, they did so to Kristiina’s school teacher and the rest of the staff at her school. They were crushed as Kristiina was muched loved by everyone that knew her at the time. She is still much loved by all who know her. Monica said that as the school staff listened, tears streamed down all of their faces. I myself, have shed tears as I have poured over the details of these stories. These kids are priceless beyond comparison. As was mentioned earlier, Kristiina’s twentieth Birthday was approaching quickly as all of this was taking place. Her Birthday would come and go quietly. Monica made comment about this stating that it just seemed, at the time, that they had just taken another step closer to her daughter’s death. They did, however, celebrate the occasion at home, and made good and sure to make their princess feel special. Oh God, how truly special she is! Again Adam would take the lead as the mood in the house became somber. Monica said that she was having a difficult time coming to terms with what life had brought there way. She said that she would find herself falling apart often and with great ease. How very understandable! She would go on to tell me how Adam helped in this situation, “Adam persuaded me there was no time to sit around and wallow in self pity, we needed to act. We needed to learn more so that we could make these years, or however much time we had together as a family, special and memorable for us all!! I began researching Batten’s Disease. Looking for other families I could reach out to. The world felt so big and I felt like we were so alone in this.”
Monica started making phone calls as Adam continued to work to provide for the family. She looked for provincial and municipal programs and organizations that might be a help to them. She also posted a plea in one of her mammas groups on Facebook. As she did so, she was shocked to find out that one of the mom’s had a nephew that was battling Batten Disease. How could this be? Through all of what would follow Monica would learn that they were, in fact, not alone. No, not at all. There was a worldwide community made up of families that were facing the same set of circumstances. Supported by social media and other forms of communication, these families were anchored together by organizations like BDSRA and BDFA in the UK. These families were and still are able to understand and support one another as no one else can because only they can fully understand what each other is experiencing. They know the details of what takes place as the disease progresses and they are able to know what each other is feeling on an emotional level. You have to walk in a person’s shoes to know for yourself! Adam and Monica would find Bev and George Maxim of the Canadian chapter of BDSRA and very quickly, they made plans to attend their first Batten conference in July of 2016. Monica said that it was the best thing that ever could have happened at that point. They were able to connect and learn so much about Batten Disease from the many professionals that were in attendance. My opening quote comes from an event that was experienced as they attended the New Family Mixer at the conference.
The gathering was overwhelming for Kristiina because of the large number of people in attendance and as a result, she experienced a seizure. I’ll repeat what was previously quoted at the beginning and include the remainder of what Monica had told me, “I had never experienced anything like what followed. Adam immediately ran back to our room for her rescue meds. Everyone at our table sprung into action! The two men lifted the table out of the way and gently placed Kristiina on the floor on her side. The one lady calmly stated her seizure had started at such an such a time and it had been just over a minute. There were no awkward stares or whispers. Everyone went about their business around us. As I was kneeling beside Kristiina and comforting her, one of the mother’s came over and squeezed my shoulder, asking if there was anything she could do or help with. I soon came to know that wonderful, sweet woman as Laura Betts. For the first time in forever, this felt ok! I didn’t feel like the freak at the circus everyone stares at. We were totally overwhelmed by the warmth and acceptance of everyone around us. It changed our perspective of our situation for the better, forever! And finally, we didn’t feel like we had to do this alone anymore.”
And with that, Adam and Monica discovered that they were not alone. They had the overwhelming support of a big bunch of wonderful people to whom they could relate. People like Laura and Barrett Betts, as well as, many others. It was, no doubt, an amazing experience for them! Over the course of the last couple of years since the diagnosis, there have (I am sure of this) been many close bonds created between the Bearman family and many of the others. The year 2017 brought with it occasions that were reason to rejoice and also things that brought sadness. It was 2017 that Kristiina would graduate from high school with a Certificate of Accomplishment. Monica said that they were surprised when they learned that the Parliament for Burlington Ontario presented Kristiina with the Citizen of the Year Award! She is so deserving of that! Life after high school, though, would bring many challenges for the family. Kristiina would be denied access into several life skill programs because of her diagnosis. Monica said that they would continue to receive denials from the coordinators and she shared an example of the type of thing she would hear from them, “I’m really sorry but we are not equipped staff wise to deal with her seizures. She is a high risk student and a real liability”. She went on to share with me her feelings and also what a typical response from her would be, “I felt like they were talking about Kristiina as if she was a car or a piece of property!! The kind denial emails were always a slap in the face that angered me. I would fire back a nasty email stating how unfair it was to Kristiina to be denied and that it was them losing out in the long run for not giving her a chance.” Who could blame her for her feelings of frustration?
There was an event that brought much sadness to Monica and Kristiina and it had to do with an old friend of Monica’s. Her friend’s name was Lindsay. Monica had hired her to be Kristiina’s PSW and she performed this function a couple of times a week. Kristiina really enjoyed their time together. They would go shopping and at one point, Lindsay introduced Kristiina to a church based group called The Friendship Club. They would, for example, do things such as going bowling together as a group. Kristiina had been seizure free for almost a year and a half as of December 2017 but that would soon change as tragedy struck both families. Here it is in Monica’s own words, “I had been getting Kristiina ready to go out with Lindsay, as we did every Wednesday evening. When Lindsay didn’t show up or text us to cancel, we didn’t know what to think. The next day, we found out that Lindsay had struggled with her own dark demons for sometime and had lost the battle. I was devastated!! What was I going to tell Kristiina? I took a couple days to come to terms with the news myself before I broke the news to Kristiina. We explained to Kristiina that Lindsay had looked fine on the outside, but was sick on the inside, and she had passed away. We told Kristiina that Lindsay was very sorry and that she loved Kristiina very much.”
Lindsay was much more to Kristiina than just her Personal Support Worker. She was Kristiina’s good friend and she was someone that was loved and trusted, just like family. Monica said that it was obvious that the stress associated with the news was too much for Kristiina. She became angry and upset over it and as a result, she suffered a seizure the next morning. The seizure left her worn out for the next few days. Monica said that Kristiina has never completely gotten over the event. This is certainly understandable.
As April of 2018 rolled around, a really good thing would happen. Monica would learn about a day program that would show great promise for Kristiina and she would learn about it from one of her momma friends on Facebook. Monica went almost straight to the program and talked to the coordinator whose name was Bill Murray. She had a lengthy conversation with Bill and, much to her amazement, she found out that he knew what Batten Disease was in detail. This is because one of the former, long time clients there had the disease. Ironically, it was the family of the mom that had initially provided Monica with all of the information about BDSRA and Batten Disease. Monica said that it felt as if this was all meant to be and, in fact, it was as Kristiina was accepted into the program on a trial basis. In time, it would prove to be a great fit for her. The name of the program is Accent Music Therapy and it resides in Burlington, Ontario. Monica says that Bill Murray and the staff there have handled Kristiina’s seizures like seasoned professionals. Perhaps the other day programs could learn a lesson from them! Monica is so thankful for the opportunity that has been presented to Kristiina by Bill.
Monica says that, in many ways, life for them is just like any family. Adam works hard and does a great job of providing for his family. Kristiina’s big brother Matthew lives outside the family home while Khloe and Sammantha still live with mom and dad. They both attend school. For Monica, there are all of the normal responsibilities. Housework and preparation of meals are a big part of her day but the biggest job is being a mom. Specifically, a mom to three daughters. One is a warrior princess and that of course brings with it special challenges. Monica stated that Kristiina cannot be left alone for more than 30 minutes at a time and so she needs to be vigilant. Their activities as a family has to take Kristiina’s special needs into consideration.
The Bearrmans have to be prepared for anything and they need to refrain from activities and conditions that may trigger a seizure in Kristiina. Their social life is affected because most people either do not understand their situation or they are uncomfortable with being around a person with special needs. Both Adam and Monica each have a few friends that they socialize with and their closest family members give them lots of love and support. This would include Monica’s mom Helen and sister Janet. Adam’s parents and other family members are a part of the Bearman’s support system as well. One very noteworthy individual is Adam’s sister and her name is Krystal (Bearman) Blackborow. Krystal had moved in with the Bearman family about a year ago and their relationship has since blossomed. I will let Monica tell you what she means to Kristiina and the family in her own words, “She’s always there for our family. She’s grown very protective of Kristiina, as she is of all her nieces. She helps me out a lot with the girls! I’m so glad I live with my sister-in-law. she’s my best friend! We love her!” That is, of course, a wonderful thing!
There is nothing that this family would not be willing to give up for Kristiina. Monica says that her love for her daughter is unconditional. She is completely empathetic towards Kristiina as she continues to teach Monica so much about the important things in life. Knowing that her time with Kristiina may be limited makes each day count towards forming priceless memories. Monica stated that each smile, each giggles, each kiss, and each hug are priceless to her. Kristiina is her princess and her presence in Monica’s life is a gift that she doesn’t take for granted. She reflects on what she sees in her daughter and what she has been taught about life through her circumstances, “I admire her (Kristina) for her strong will and determination. She never quits trying to do her best! She loves to help everybody! She has this spark about her that is indescribable!! She’s funny and loves attention! She’s my hero! It’s hard to think to think about the future when you’re not sure about what’s to come. Adam has helped me deal with the anxiety that arises in me when I think about the future. I have learned there is no sense in worrying about tomorrow. We take life one day at a time and live for today with no regrets and no looking back. There will always be challenges in life. We do our best to deal with them and just take it as it comes.”
It would be difficult to not consider the things that Batten Disease has taken from Kristiina and her family but Monica says that they have a lot to be thankful for. Kristiina still has her eyesight and her ability to walk and talk! She is able to eat and drink by mouth and any Batten parent will testify to the fact that this is a big deal. As is always the case, a Batten parent thinks about what it would be like to lose their child to this cruel and relentless disease. Like many of the families that battle Battens, the Bearman's cling to the hope that is provided by current treatment that could be made available. Here is what Monica had to say about the subject, “We are hopeful that one day Kristiina will receive the life changing enzyme therapy. For now, we will advocate for her right to quality of life and we will educate others about this disease in hopes of Health Canada approving the treatment that she so desperately needs and deserves. This whole situation has also opened our eyes to the sad realization that these treatments and cures, not only for Batten’s Disease, are more so about the politics and money involved.”
I was flattered by the fact that Monica approached me at this years Batten conference and introduced herself to me. She had been a regular reader of my blog and she wanted to work together in doing Kristiina’s story. Monica is another incredible mommy! There was so much going on at the moment that she approached me and I had failed, at that time, to walk to their table to meet Kristiina. My huge mistake. Hopefully, next year if I can make it to Denver. Looking back at it now, I had seen Kristiina (or Krissy) in the midst of all of the Batten kids and I could tell that she was a crowd favorite. This, I can tell from what I have seen, and also from What I have been told. This being that Kristiina is entirely special.
I know that it is often difficult for Batten parents to recount the experience that is the Batten Journey. This includes sifting through old photographs that help to paint a picture of what life was like before Batten Disease. As I was close to finishing this story, eight children had gained their wings in the span of about two weeks. Flying away together as a group seems to happen frequently with Batten children and I know that the community was shaken when it happened this time. Batten parents were hugging their kids extra tight when this took place. Monica was also deeply affected by this loss and she had to power through near the end of working with me. She has my profound respect and appreciation for this. Kristiina is doing so well and will continue to battle. Advocating for her treatment is of the greatest importance. These kids (and sometimes young adults) are so special and it is difficult for me to find words that are adequate to describe how I feel. The parents, however, know for themselves how these warriors tug at your heart.
I am a friend of, and an outsider to the Batten community. I still do not know, nor can I fully understand what it is like to be on a journey such as Batten Disease. With every new story, I remain amazed by the care that is displayed and the love that is undying. Another ordinary family that has been asked to do something that is extraordinary. Nobody will impact the lives of this family like Kristiina will. She will forever be a part of what makes up the whole and the impact of her life on others will be without end. Such a princess is she!
Blogger and Advocate
“I have always called Mya my little butterfly because her beauty has always awed me. Like when we see a butterfly and we are awed by their beauty. Also, as time went on the meaning grew. Especially when I heard this song (She’s a Butterfly - Martina McBride) and dedicated it to Mya.”
I have said this before. Spreading awareness concerning Batten Disease was not my original intent. Things just kind of snowballed, and one thing led to another. Yes, this has become my little niche’ for spreading awareness, but greater awareness is just a byproduct, if you will, of what my actual objective is. I do what I do in order to bring honor to those who battle Battens, and also those who have done so in the past. The stories were simple in the beginning, but they have now grown in size and complexity. Often times, the details are, oh, so sweet. This, in large part, because of the love that is shown towards these young warriors.
Some will say that I have no idea what Batten families lives are like, and that I have no right to be involved. With others, I have gained acceptance, and I have been shown their appreciation for what I am doing. In the former case, I have to agree that I have never experienced what a parent goes through when they are in the midst of the struggle that is Batten Disease. Caring for someone that battles Battens brings about circumstances that not many will experience. These are normal people that have been asked to endure something that is extraordinary. It is tragic when a parent is told that their child has a disease for which there is no cure. Even worse, when one is told that the disease is fatal. I have used the term “A Beautiful Tragedy” in the past as an analogy and I was able to get away with it. I am going to use it again here. When something tragic happens, it brings with it terror and dire circumstances. Nobody wants a tragedy to take place. Sometimes, though, beautiful things come out of tragedy that would have otherwise never surfaced. People work better together in those times and are generally less selfish. Strength and determination are often on display during difficult times. Resilience is at full strength. Love is displayed without hindrance. The type that care separate themselves from those that care only for themselves. Heroes are made and strong bonds are created. These things, however, may often be only viewed from a distance. Those in the midst of the tragedy may be too overwhelmed by the circumstances to see anything but the heartache.
I never want to create drama that isn’t there by the words that I choose to use. That being, as I write these stories. I only want to share my observations. Those that care for children with Batten Disease deal with the daily struggles and the heartache that is attached. I can identify with some of it as a parent to a special person, but only to a small degree. The parents of these children are often stuck in the muck and the mire as the disease progresses, whereas I can only form opinions from a distance. When I look at someone that has lost a child to Batten Disease, it is almost as if I can sense what they are feeling. There is no way, however, that I can feel the pain associated with that kind of loss.
Being hidden from the daily circumstances, I see, from a distance, the sweet details. Undying love in action. Heartbrokenness mixed with resolve to do your very best. Caring for an innocent child or young adult as they battle Battens. The bond of affection is intensified by hours of care and time spent together. These little ones become our heroes, and they are due that honor. I received the nicest comment about my last blog that I wrote after attending the Batten conference In Nashville this year. It came from a veteran warrior mom who had lost her daughter to Batten Disease all the way back in the mid 70s. Susan Lesko Klee had this to say to me after reading the blog entry, “Thank You for sharing your beautiful thoughts, and for loving our children so fiercely.” I love the word fierce as it is used in relationship to the type of feelings that surround these kids. I Googled the word and this is part of what I found. A usage within the definition for the word says something like this, “A feeling, emotion, or action, showing a heartfelt and powerful intensity.” The words powerful and intense are both perfect in describing the type of love that I see being displayed by those who care for these children. It is difficult for me to not feel it as well after observing so many people putting that kind of love into action. Children with Batten Disease produce these types of feelings. This, is what I see. This next story is about one such child.
Jessica Nicole was alone, and she was left to deal with some extraordinary circumstances. This, as it was time to welcome her first child into the world. Doctors had seen some abnormalities in her expectant child through an ultrasound that had been performed. As a result, it was decided that it would be best to induce labor. She had been carrying her baby for forty weeks and the doctors didn’t want to wait any longer. The time was 8:55 in the morning of October the 2nd, 2008. That was the time that her baby girl came into the world. Her mommy would name her Mya Nicole, and from that point on they would forever share the same middle name. Never had Jessica experienced an event such as this. She held her brand new baby in her arms, and she was her very own. Mya was perfect, and she was perfectly beautiful. She was covered in brand new baby skin and she was, to Jessica, the most lovely little thing that she had ever seen. Who could argue that this was the case? Just look at her!
Jessica would tell me that she had never felt true love before like she had felt at that time. This all took place at Forsyth Medical Center in Winston Salem, North Carolina. It happened, however, that her joy over the experience was quickly turned into feelings of confusion. This because the staff took little Mya away from her mommy in only a moment after she was placed in Jessica’s arms. I can only imagine what a whirlwind of events this must have seemed like to her. Mya needed to be examined further because of the problems that were detected in the ultrasound. Little Mya was taken to the neonatal intensive care unit (NICU) where they would discover that she had several serious issues that she was facing. The least concerning was that she had a club foot on her right side. This is something that could be corrected by surgery. Along with that were some more serious issues. Her little spine was shown to be abnormal, and she had a missing kidney on her right side. The largest issue that baby Mya faced was with her little heart. She had a Ventricular Septal Defect with double outlet ventricle. This means that Mya had a hole in her heart, and an abnormality in the plumbing within her heart. Barely into the world, Mya would already be a little warrior of a princess. None of what she was facing would cause Jessica to think less of her infant daughter. She described how she felt as she became the mother of little Mya, “...when I saw her, I didn't see a baby. My baby with defects. I saw the most beautiful child I’d ever seen. She was gentle and sweet.” There is no doubt that the bond was instantaneously strong. She had carried little Mya for nine months and she was here. The love that she felt for her daughter was powerful, and the problems that were present would not deter her! Jessica would stay by Mya’s side, refusing to leave her baby.
Baby Mya would do well while in NICU but then would contract Sepsis, which is an infection of the blood. This can, of course, be very serious. Little Mya was placed in a room within the pediatric intensive care unit (PICU) with Jessica by her side. There was no family present at any time nor was Mya’s biological father there. Jessica was just eighteen years old at the time, and she was without a clue as to what to do. The situation would have been very desperate except for the attention that they both had received from a very caring group of nurses. They were at Jessica’s side to give her comfort and relief when she had feelings of being overwhelmed by the situation. Mya would recover, and within a week it would be time for Jessica and Mya to leave the hospital. Alone and only eighteen years of age, Jessica would be confronted with more bad news before leaving. A doctor pulled her to the side and explained to her what he felt, at the time, was the probable cause of Mya’s health issues. This would only add to her increasing anxiety. The doctor went on to explain that he felt little Mya had a condition called Vacterl Syndrome. Otherwise known as VACTERL Association, this is a genetic disorder that affects many body systems all at once. The issues that little Mya was facing would cause doctors to fit her into this category, and she was officially diagnosed with the disorder two weeks into her life. According to the doctor, the condition is one that is very rare and there are many health professionals that do not know much about it. The biggest concern at that point was Mya’s heart. The doctor had a prediction that by age three months, Mya would suffer from congestive heart failure. He recommended that Mya be watched closely by a cardiologist through regular visits. The doctor would add that her heart had already started to weaken. He expected that little Mya would not make it past 6 to 7 months of age if the condition of her heart did not improve.
I can only imagine what it would be like for a young girl, who had been through so much already, to be given this kind of news. To look at the little person that she had brought into this world with great expectation. She could have been defeated by what she had heard, but instead she chose to have faith. Jessica described what she was feeling, “My heart dropped. I was a mess, but I was determined no matter what, Mya would be okay. We would get her healthy. God has a plan for my child.” In fact, He did have a plan and still does. This all weighed on Jessica’s mind as she left the hospital with baby Mya. It was time to go but what would she return to as she went home? Jessica lived, at that time, with Mya’s father, but the situation was not a good one for her and her baby. She needed to leave. This, on top of all of the new complications, they needed to move and find a new place to live.
Jessica reached out to social services as she could not think of anything else to do. It was no accident that she would be put into contact with a very wonderful and loving family that would help and assist in a huge way. It would be easy enough to mention their last name, but let’s just call them, well, The Angel family. Angels do exist you know. They are God’s special agents, and they are often sent to help. Most of the time, we don’t even know that they are there. However, sometimes they make their presence known, and it is unmistakable. The later case is what Jessica would experience as the Angel family took her and Mya into their home. It would be well worth it as this would require them to move from Virginia to Kernersville, North Carolina. Jessica and Mya would live with them for a few months until they were able to get on their feet and they were a tremendous help and support to them. Mom Angel is a registered nurse and her help with Mya was invaluable. She would attend Mya’s many appointments, and every surgery that she would require. This family even introduced Jessica to their church, and they provided so much support to her. Jessica credits them with helping her to be a better parent as they modeled these skills in their own home. They provided a shoulder for her to cry on, and they remain a huge support to her.
Support is what was needed as the challenges for Mya would continue. At three months of age, little Mya experienced congestive heart failure. This, just as the doctor had predicted. Heart failure meant that the pumping power in her little heart was weaker than what was needed to maintain proper blood flow. The doctors needed to perform reconstructive heart surgery on Mya, and that would include patching the hole that was about the size of a dime. The doctors wanted to hold off until Mya gained weight and would do so until she was seven months old. At that time, she was twelve pounds in weight and they could no longer put off the surgery. Her life was in jeopardy and they needed to take action. She underwent heart surgery at Brenner Children’s Hospital in Winston-Salem. Jessica waited hours as the Angel family sat by her side. Jessica said that she was a nervous wreck as she waited approximately six hours while the doctors worked on her baby’s heart. Mya was then pulled out of surgery, and it seemed that she had wires coming out of her everywhere. Jessica described her reaction which was certainly understandable, “I weeped and weeped. I just wanted to hold her and know that everything was okay.” More waiting, and then Jessica was finally let back in to the recovery room. Mya had a huge scar on her little chest and she was asleep, heavily sedated. Still, her appearance to Jessica was like that of a precious angel. Jessica said that days would go by and then the medical staff would begin to take her Mya off of medications, and they also removed her from machines. She must have been so relieved as she observed the results. As Mya awoke, she had a smile that was ear to ear, and it lit up the room. According to Jessica, it was like the surgery had never phased her. She would go on to describe the results as baby Mya recovered from heart surgery, “She began to roll over, crawl, and meet milestones as expected. She was such a happy baby and one that is full of personality and spunk. Even to this day she is that.”
Mya would need surgery to correct the other issues that she faced. She would go on to have seven surgeries on her right foot and a tethered spinal cord surgery. All of this took place by age four. After this, Jessica felt as if life for Mya would be smooth sailing. Children are just by nature, beautiful. Their features are pristine, and they are free of the lines that are brought on by the worries of life. Innocent fun as life’s lessons are taught are the order of the day. Jessica’s label of her daughter as being a butterfly was spot on. It is certain that she brightened any room that she occupied, and having her in your presence was pure joy. She had already been through so much, and had come through with flying colors. During these years Jessica would marry but the relationship wouldn’t last. Life happens, and things do not always work out the way that we would want them to. The big silver lining is that out of that came Mya’s little brother whose name is Cameron. Jessica is a person who has faith in God. She felt that He had His hand on her life even in spite of some of the difficulties that she had faced up to that point. Mya had turned five years old, and by this time her life had already touched the lives of many. That would include a family of angels who Jessica still considers to be her own! She decided that it was time to move back to Virginia and to be with family. So it was in December of 2013 that she moved back home, taking little Mya and Cameron with her. Upon returning home, Jessica would enroll both of her children in daycare, and both would do great in that setting. Mya was a late bloomer and didn’t start kindergarten until August of 2014 at age six and a half. That is when signs of a behavior issue started to surface. Little Mya was initially diagnosed with having ADHD. This seems to always be the first conclusion that people come to. Jessica said she was told that it was actually in the families best interest to pull Mya out of school. This was only two weeks after starting. The teacher felt that Mya had issues with defiance, and that she was not able to pay attention. After all this little girl had already been through. How disappointing this must have been for both Mya and Jessica.
Jessica stated that she was hurt by this, but proceeded to pull Mya out of school. She did so under protest and would soon place Mya in a private school. This, hoping that her daughter would get the help that she needed. The signs that there was a larger problem continued to develop, and they must have left this momma feeling a little bit weary. Even in private school Mya had issues with retaining information. Not only that but she also had forgotten the things that she learned in preschool. She could not write her name, do her ABCs, or her 123s. She could not even identify simple objects, and could barely hold a pencil. Her difficulties were great enough that Mya would have to be held back the following year. Jessica would have opportunity to place Mya back into the public school system at a time where she had moved to a different county within Virginia. In spite of what she had heard about the quality of education within the public schools, Jessica would find that they worked best for Mya. She said that at one point, she was able to find a teacher that really “got it”. Jessica would fight “tooth and nail” to get her daughter placed in special education, and Mya would eventually be given exactly what she needed. She loved school, and she loved to socialize. It would bring Jessica a measure of comfort to eventually find things that worked well for Mya. This, even though the symptoms of a greater problem would continue to develop.
Mya is a beautiful little girl for sure. She could really pour on the charm, and Jessica has the pictures to prove it. She would pose for Jessica whenever the camera was on her, and Mya was, and still is to this day, momma’s butterfly. Socially, she had proven to be in the butterfly category, and mom has always considered Mya to be a rare jewel. I have to say that I completely agree with her. The relationship between a mother and her child is a very special one. It is just my observation that there is a special quality of a bond between a mother and her daughter. The bond between Mya and Jessica was very strong. Especially after all they had experienced together. This would come into play as Jessica began to notice that Mya would no longer look her in the eyes. This began to happen as Mya had turned seven years old. Jessica was on to something and she described her reaction, “It drove me insane and worried me so much. I addressed it to her psychologist and she said not to worry.” Jessica’s thought was, “easier said than done.”
Symptoms of a disorder that was unknown to Jessica would continue to develop in young Mya. It was as if a puzzle was being poured out on the table and the pieces would have to be put together. Jessica would begin to notice changes in Mya’s vision. She would have to hold objects close in order to see them well, or she would use her peripheral vision to look at things. Jessica would, of course, take Mya to see an eye doctor, but the results would be less than perfect. The doctor would tell Jessica that Mya’s eyes were fine, but he would prescribe glasses in hopes that it would help. Mya broke them in a week. The problems would just continue to mount. Jessica would see more problems that were taking place with her butterfly. Mya was struggling to write, and retaining information was becoming much more difficult. How disheartening and frustrating this must have been for Jessica. This is not to mention how frustrating it was to sweet Mya. This was all taking place as Mya was going to be starting the first grade, and Jessica felt that she needed some answers. Just as with most every mom, she knew something was wrong. As she would put it, “I knew in my heart something was not right.” At that time, Mya was still seeing a psychologist, and during an appointment Jessica was asked if she noticed Mya having staring spells. Jessica was surprised with being asked this question. That is something that she had never noticed with Mya. The psychologist questioned whether Mya may be having seizures or not. As a result, she would refer little Mya to a neurologist at UVA (University of Virginia) Hospital in Charlottesville, Virginia. This was a two hour and 45 minute drive, but Jessica would have traveled any distance to put more of the puzzle together. She wanted the best care possible for Mya and a diagnosis for something that was reversible. She felt, at that time, that whatever was going on with her daughter had to be fixable.
Something good would happen for Jessica and her children while this was taking place over the course of time. A male friend named Tracy had contacted Jessica, and they began to talk about things. They had known each other for seventeen years, and their relationship would develop into something that was more than a friendship, although it would also remain that. Jessica had just finished moving into a nice place to live with her two children when they began to talk. They would date for a while, and would introduce themselves to each others children within a short period of time. The pair hit it off right away, and their relationship would blossom. Life can bring with it difficult circumstances at times. For some the challenges are greater than they are with others. Jessica shared with me concerning much of what she had faced personally up to the point that Mya had come into her life. I can tell you that it was more than a lot of people have gone through. Of all the challengers that she had faced up to that point, the journey that she would embark on with Mya would be the greatest challenge of them all. I have so much respect for her as a person. She is also, to say the least, an incredibly caring mommy. The challenges would only become bigger, and Tracy would become the biggest means of support for Jessica on an emotional level. This, both with handling the struggles of the past and also the challenges that would come in their future. Jessica refers to Tracy as her ”best friend and soulmate”. According to her, Tracy is a hard working guy, and he loves her children as he would his own. Tracy would become a huge help with Mya, and he loves to make her laugh. For this reason, he and Mya have a special bond. Both Mya and her brother Cameron refer to Tracy as their daddy. Jessica has told me that he is in all honesty the only one that the kids have ever known. Tracy’s support would be much needed as Mya’s situation continued to become more complicated.
The struggle would continue and the puzzle would continue to be pieced together as the family met Doctor Heinen, a neurologist, in October of last year. Jessica would refer to her as a wonderful woman and a Godsend. She is the kind of doctor that goes above and beyond in her patient to doctor relationships. This was especially evident as Mya came into her office for the first time. Jessica would tell me that Dr. Heinan fell in love with Mya instantly and she was eager to help in every way that she could. Dr. Heinan’s face would light up when she entered the room as another bond was being formed between her and Mya. Jessica had prayed over the initial appointment as the day arrived, hoping that answers could be found. She stated that the appointment seemed long and her nerves were shot as time moved on. Finally the doctor said that an EEG should be performed to which Jessica was in full agreement. The results would come back showing an abnormality. Mya was, in fact having seizures and she would receive an initial diagnosis of Epilepsy. She was prescribed medication and would do well on the meds for some time. This must have given Jessica a certain feeling of relief. People with Epilepsy can often lead a close to normal life. Even so, to be an epileptic on top of all she had already been through. How much could one little person go face in a lifetime? Any feelings of relief for Jessica would be short lived and overall she was still unsettled with things. The puzzle would soon be put together and the picture would present the biggest challenge of them all.
Even with an official diagnosis of epilepsy, Jessica had left that first neurology appointment not feeling quite right. She described what she was feeling, “After that appointment I never felt right. I felt like God was tapping me on my shoulder trying to prepare me for something, but I was too busy and stubborn to listen to Him. I didn't want to believe there may actually be something more that was wrong with my child.” This would lead her to make a decision. It was right before Christmas of 2017 that Jessica decided that she needed to quit her job in order to keep her focus on Mya. She had, up until then, been working as a manager of a fast food restaurant for five years. Jessica knew in her heart that Mya would need her in a big way. This as, being a mother, her instincts were in full effect. It was the day after Christmas that an event would take place. This would lead to the next stage in determining what was taking place in Jessica’s butterfly of a little girl. At the time, Mya was sitting at the table she began to be held in a jerking motion. She wouldn’t move or speak and it was obvious that she was seizing. The decision was made to load her into the car and to rush her to UVA hospital as quickly as they could. As they arrived, Jessica told the staff that they were not leaving until every last test was run on her daughter and some answers were available. They would be there for four days.
Doctors and other staff would run a 48 hour EEG, an MRI, and Mya would be seen by an eye doctor. They still would leave with no answers. The only information that they would receive is that Mya’s brain volume appeared to be smaller than usual for someone her age. Jessica was told, though, that this was nothing to worry about. It would soon be known, however, that the low brain volume was due to degeneration. The tension had to have been mounting for both Jessica and also Tracy as he stood by her side. This darling little girl had been through so much already in her young life, and there was more that was developing. It was the very next day after leaving that Jessica received a call from the neurologist. They wanted a genetic panel performed on Mya. This was to be performed on February the 26th of this year (2018). They would, of course, go get the blood drawn and it would not take long to get the results. Jessica would receive the next call on March 30th stating that she needed to come to UVA hospital very soon. The results were back and they made an appointment for April the 4th. Jessica stated that they had never got her in that quickly before. Once they had arrived, the family was escorted quickly to the appointment room. It also did not take long for Dr. Heinan to arrive, and with her was a genetic counselor.
Jessica said that she watched the doctor and that she observed her facial expression and her body language. Jessica knew from what she had seen that the doctor was going to tell her something painful. This had to of set both Jessica and Tracy on edge. Jessica said that Dr. Heinan grabbed her hands and looked her straight in the eyes. She could see that it was difficult to tell her what she had to say as she proceeded to speak. I’ll use the very words that were given to me by Jessica as they were told to her, "Ms. Hardy, Mya’s test came back and we found some abnormalities. Mya has NCL which is also referred to as Batten Disease. This is a terminal illness."
Most parents that are given the news that their child has Batten Disease have never heard of it. They go on a search for answers thinking that whatever the problem is, it can be fixed once it is identified. It is a fact that Jessica had known what Batten Disease was as she received the diagnosis. This because she had followed a page on Facebook about a young girl who had an NCL (neuronal ceroid lipofuscinoses). Jessica felt that it was almost as if God had taken her to the page in order that she would be informed about Batten disease. Certainly, nothing could prepare her as the diagnoses was given to her. The following was her response as she received the news, “Everything else she (the doctor) said was a blur to me and my heart shattered. Everything around me stopped and I remember saying no no no, then saying I knew it I knew it I knew it. Crying, I looked at Tracy and he was crying. I got up and I grabbed Mya and held her. I asked Dr. Heinan, "there has to be something we can do. She said Mya has CLN1 and there was nothing to cure it, only meds to help with symptoms. I felt so many emotions at one time. Why was my child, who had already been through so much, having to have this horrible diagnosis and this outcome? This isn't fair. I took it very hard and became very depressed.”
Just like it is with every parent that receives this awful news, there is the initial shock and range of emotions that comes with it. “My child! What do you mean we can’t fix this?” The initial trauma subsides and it is replaced with a determination to do the very best that one can for their child. Hope, acceptance, heartbrokenness, pure love, and a bond that is so strong that it will never be broken. These are, in all likelihood, the things that are experienced. These events and the changes that follow are things that Jessica has experienced as well. She shares these words, “I am finally getting out of the depression and learning to take things a day at a time while enjoying what life of Mya’s I have left to share with her. I try to do my best.”
I have said it before and I will continue to hold fast in my belief. These kids change people like none other. Their resilience, strength, and determination are so inspiring. How they go about changing the way that we view the things that are important. How they teach us to keep going on our worst days. The special memories that they create. Yes, even from a distance, I can see that there are not many like them. If I am affected like this from a distance, how much more those who are close. I feel that Batten moms (and dads too) experience things in life that not many people will. Jessica shares more about Mya, “My life definitely changed. At first, like I said, I did not take it well. I believe the outcome of all of this will be the beautiful Legacy Mya will leave behind and that legacy of her’s I will continue to share. Also through her legacy Batten Awareness will be spread. Research on Mya may provide a cure? Whatever the outcome, Mya will have served her purpose and fulfilled God's will for her. She will be able to help others through her story of strength (Mya doesn't let anything stop her), her determination, beauty, and joyfulness.” I have been touched personally by each of these kids that I have followed and none more than I have watching Mya from a distance. She has had to battle right out of the starting gate and has been through so much. What a precious little warrior princess she is!
Jessica’s perspective on things is spot on. I think that it reflects the feelings that are held by many others within the community that she is now a part of. Working through the heartbreak, she looks for the positives in her situation. Here is more of what she had to say. In this, she includes anticipating going as a family to Mya’s Make-A-Wish trip, “Even though I watch my child become weaker, and now having issues with stiffened muscles, knowing someday she will not be capable of walking. I hang on to my faith in God and I watch Mya fight like she has since day one. She wears a smile like nothing bothers her, and she is so happy and loving. Next week she will go on the biggest trip and wish of her life. To see her smile and enjoy it with her brother and us, her family, will be all that matters to me.”
As we go through life, one of the most important things that we have are the memories that are made along the way. I have no doubt that with a Batten child, making memories is so very important. Both for them and for their family. That is why the Make-A-Wish Foundation is such a special organization. Not only are memories made but the warrior prince or princess is held in a special place of honor. This, as they should be. Tracy, Jessica, Mya, and Cameron would go together as a family to Walt Disney World in early August of this year. Jessica told princess Mya about the trip as soon as she was accepted for it by the organization. She did this so that Mya would become excited as she anticipated leaving for the trip which meant so much to her. Mya would have a send off party before going, and she looked all the part of a princess, and she definitely was, and is, a butterfly. Mya especially enjoyed the light shows and fireworks. This is not to mention interacting with all of the Disney characters. Like most of us, the family does not have a ton of money laying around. This made the help that Tracy and Jessica received with the trip so very much appreciated. Jessica shared that Make-A-Wish truly made their dream come true! You just know that was the truth when it comes to Mya.
This is a family of a rare kind and Mya will always be a giant part of who they are. No one else will have a greater impact on this family than Mya will. I want her to battle forward, just as she always has already. There will be further challenges but my hope is that Mya’s life will draw the people that love her closer together. I have the deepest respect for any man that takes on himself the responsibilities that Tracy has. He loves Jessica’s children and he stays by Jessica and her children’s side regardless of the challenges. Tracy refers to Mya as “his baby girl”. He has been a huge help to Jessica in the time that they have been together, and she has said as much. She says this concerning Tracy, “When I felt defeated and could not move on he has picked me up and carried me through this. Sometimes when I did not have the strength.” Mya’s little brother Cameron is six years old. Jessica says that he is extremely smart and is also a very loving child. She refers to the bond that exists between Mya and Cameron as “inseparable”. Jessica feels that Cameron was sent by God to help with his big sister and who could argue with that? She considers both her kids to be a blessing even with the challenges.
Yes, a fierce kind of love is in order when it comes to these children. Anybody with any amount of feeling has to be moved by the details. Writing these stories has become a big reason for my waking up each morning, and I will continue as long as I am able. This, because these kids show us so much that is of great value. They absolutely amaze me as do their families. Ordinary people that have been asked to do something that is extra special. Progress is being made but for the families, it couldn’t come soon enough. The stories are in a big sense filled with tragedy, but the details of undying love and commitment are very beautiful. These little warriors are extremely special and I am moved by what I see!
I want to tell you a little more about what I see as I close. I have a close friend on Facebook named Christen who I will often talk to about this blog. She is always a great source of encouragement to me and a big supporter of what I do. I was sharing a little about this particular article with Christen and I forwarded a picture of Mya to her. Her initial comment was that Mya is a beautiful princess. She did not have in mind the fact that her mom calls Mya her little butterfly but I am sure that she can see what I see. That is what her mother sees. Mya is beautiful just like the most beautiful butterfly you could imagine. With her long dark hair and olive complexion, she is a little beauty. Jessica had made a short video for me of Mya doing her best to say hello to me, and it provided one of those moments. In the video, Mya was working so hard to do the things that her body was not cooperating completely in doing. She would get the job done though. It was amazing, and precious, and I shared it with Christen. This is all of what she said after watching it, “Oh my goodness, she’s so precious. She tries so hard, and she’s so sweet. I just want to hug her.” My reply was simply, “You see why I do what I do (Writing this blog)?” Christen said in response, “I absolutely do. I understand totally.”
Christen gets it and she sees what I see. These young warriors; boys, girls, and young adults just tug at your heart like nothing else. Yes, loving them so fiercely is the right thing to do. This, because they so deserve it, and because they just cause it to happen, if you are the right type of person. I am that type. I can see that Mya is a butterfly, and that, she will always be that. She is a butterfly.
Blogger and Advocate
“This child, even though he was not able to see, nor walk or talk or even eat by mouth. He could not even sit up and he required total care at this point but still, he had this glow, smile and laugh that was unlike any I had ever known. He was happy and so brave. These children even with this debilitating and terminal disease that brings daily obstacles and continuous setbacks . They are heroes, superheroes, and MY heroes. They take what comes and they keep fighting and never give up and all the while maintaining this surreal strength unlike anything I've ever seen. Their families are together strong, and with what they have to come to terms with, knowing the final outcome. A life forever changed”
Kristy Thetford Prince
The image is etched in my memory and it will forever be there. I had followed my Batten princess in her journey and I knew from what I was seeing that she had taken a turn for the worst. The post on social media told the story. This created in me a sense of urgency which caused me to develop a plan. I had to meet her and it needed to be soon. As a family, we had never traveled away from the west coast but we were going to make it happen. This warrior princess made me aware but only because she shared a hero type status with another hometown hero. It was through following the legacy of a fallen Navy SEAL and a memorial created in his dedication that I had first found her. We would make the trip which would include me diving this memorial. Did I forget to mention that it is under water? I had to take diving lessons and at the time, we were in the process of selling a house. There were many things that had to fall into place and everything would come together. We told my son Benjamin, who has special needs, that he was going on the bus with wings. He did so well. The entire trip was magical and my life had been changed by the experience. This all left a lasting impression on me.
I would never look at things in the same way ever again. I had been made aware and I had met the one who was responsible for that taking place. She was beautiful and I would soon mourn along with all of those who followed her. This, as she passed into eternity just three months later. Why Batten Disease and these families? Why do I continue to hold them in such high regard, and why do I pay so much attention to this one childhood disease? My perspective in life had changed once I was made aware, and these precious Batten warriors have become much of my focus. There are many other rare diseases that affect children and also many forms of pediatric cancer. I believe that, in many ways, the families that battle Battens represent all of the others very well. The sacrifices and the hardships are a common theme in the lives of all of those who have a sick child and the challenges on an emotional level are difficult. Often times, we wished that we could take their place as they battle. It just seems so unfair that someone possessing childlike innocence would face something like Batten Disease. As I have continued to write these stories, my appreciation for what these families face together has intensified. Often times, I will use my own experience as the parent to a special needs adult to try to relate. I am always very careful to point out the fact that my experiences pales in comparison to what is faced by these families. They have my profound respect and appreciation. I find Batten Disease to be an especially brutal disease and people need to know that it exists. They also need to know what these families go through and I try to accomplish this through what I write.
My writing came out of the experience of following my personal Batten princess’ journey and also the legacy of this Navy SEAL. At one point, I had decided that it was time to start connecting with other Batten families as I followed their children’s journeys. My friend Sandy had recommended that I start blogging about these little warriors and it was some of the best advice I have ever been given. My love for these children has grown with each additional story that I have done and my connection to the Batten community has become an integral part of my existence. I love being of assistance to these families in any way that I can be and it is a privilege to be involved. One of the things that I had taken notice of as I followed more families is the Batten conference that is put on yearly by the Batten Disease Support and Research Association (BDSRA).
I thought about how much I would love to go to one of these but at the time it seemed off limits to me. After all, we are not a Batten family. Attending one of these events seemed to be something that was only available through a right of passage. On one hand, I was happy that such an event existed for the families, but on the other hand, I was a little envious from not being able to attend. What would it be like for you? By that I mean, what if you were passionate about telling these stories and had done several up to this point in time? Would you not want to be in attendance and would you not want to meet the people that you have written about? To meet all of those beautiful kids, all in one place would certainly be a chance of a lifetime. This is why I am so thankful for my friend Alicia Headrick. Almost everyone that is involved in the Batten community should know who Ally is at this point. She has one of the most beautiful little girls and her name is Lydia. Princess Lydia has CLN2 Batten Disease and her story is one of the ones that I was privileged to be able tell in my blog. I titled the story, “Lydia, I Love You”. Alicia is a very bright and articulate young lady and she more than provided me with the right amount of material for getting the job done. We worked together and I think that the story turned out very well.
As this years conference was approaching, Ally contacted me and told me that I should attend. My response was as I have already stated. I am not a part of the Batten community and do not really have a right to be there. She remained persistent, letting me know that people other than members of Batten families would be in attendance. At the time, I had been visiting my dad and I was talking this over with my son’s girlfriend. She also encouraged me to check into it. After returning home, I contacted BDSRA through the conference page and I was simply told that I was welcome to attend. I had learned a while back that people at the organization read my blog and I was happy to know this. Apparently, word travels and my name was familiar to them. To date, there has not been many things that have been more fulfilling to me than working as an advocate on behalf of these families. I wanted to go to the conference to meet the people that I have grown to care about. I wanted to observe what takes place and I wanted to learn more about the Batten community. Most of all, I wanted to be in the presence of all of these precious warriors. Remember, I had traveled a great distance to meet just one. Of course, she was very special!
Life has become very busy for me. On top of my work in the aviation field, there are all of the normal responsibilities of a husband and father of a special needs adult. On top of this, writing has become my passion and my number one spare time activity. I tell you this so that you know that the time passed very quickly between making the reservations and flying to the conference. Before I knew it, it was time to meet some familiar faces in person. As with any new experience, there is a certain amount of tension involved. You think to yourself, how will I fit in? Will I feel welcomed here? It was up to me to make a positive impression on the people I would meet. From the very beginning, those faces that were familiar to me started to appear. The first familiar warrior that I spotted was Sir Dante’ with his mom Marlo Schinell. I didn’t hesitate to say hello. Marlo had told me in the past that her son was a real teddy bear. I could tell from the very beginning that this was true. What a completely special guy he is. I looked for Dixie Bergeron and her son Jacoby and found them. I spotted Crystal Alger, who’s grandson Seth (Seth and The Healing) is in heaven, and I said hello! Pamela Cameron of Casen’s Crusade is someone who was a big help to me in the beginning and I wanted to find her. I had written a story at one point called “The Caring Type” and it was about Pamela’s grandson Casen. Casen was the most charming little man that you might come across and he is now the most charming heavenly angel, I am sure! It didn’t take long for me to find Pamela and to say hello. I would go on to spend a lot of time with her and Crystal out front with many of the others. I learned a lot by just listening.
What I observed from the very start is that which I knew to be the truth. There is a bond that exists between these families that is rarely duplicated. This, to me, is completely understandable. After all, who out there in the general public can say that they have faced the same set of circumstances? Who has walked in a similar pair of shoes? Batten families understand each other’s plight like not many others can.These families, also, all share in common the same commitment to their children. Undying love in action. They have dealt with the same emotions as they watch their children battle. The whole thing resembled a family reunion and in fact, it was that. One thing that you learn in life is that being a family does not always happen by sharing the same bloodline. I wanted to see all of this for myself and that is a big reason for my wanting to be there. It was everything that I expected it to be and more.
As I have already stated, I had followed many of these families through social media and it was such a pleasure to meet a lot of them in person. There were, however, so many that I either didn’t know or had not followed as closely as I would have liked to prior to being there. Batten Disease really isn’t that rare, is it? I wanted to meet more people in the community so I did the only thing that I could. I mingled as best as a shy person like myself can. I met some new people and others that I should have known. Well that’s the funny thing. I have gotten into the bad habit of “accumulating” names on my friends list without getting to really know people. My mistake. As I was mingling, I saw a family who had a handsome young guy with them and I walked up to introduce myself. They were very friendly and we began to talk. I checked a few minutes into the conversation to see if I could find them on Facebook. As it turns out, they were already on my friends list. I am talking about Mike and Leah Brochu. Their son’s name is Neil. What an extremely nice couple they are! And Neil? He was amazing. You see Neil loves to hug, and not just a little. You just mention the word hug and the guy throws his arms open. He is absolutely the best hugger that I have ever met and he is such a sweet kid. The Brochu family is special and I am so glad that we had a chance to meet. We were able to chat on a couple of different occasions and it was an absolute pleasure. I made sure to get a hug from Neil ever chance that I was able to, and I am glad that I did.
As I had previously mentioned, I have Ally Headrick to thank for encouraging me to attend the conference this year and her having done so is very much appreciated. I knew in advance that she would be attending with her parents, Wayne and Lisa Headrick. I first saw them by the check-in window and Ally said, “Hello, we are the Headrick family.” I was like oh goodness, I know who you are, lol. I really enjoyed talking with all of them at the reception that first night. Ally’s dad is a working guy like me and we enjoyed talking shop a little. Wayne has just been treated successfully for cancer and that is something that we also have in common. Only in my case, my battle happened many years ago. Lisa was, of course, very nice and you can sure tell that she loves her princess Lydia. We had chatted many times over Instant Messaging and it was so nice to meet her in person. I really enjoyed the chats that me and Alicia were able to have out front and all I can say is this. Everything that I had felt about her as a person was found to be true. She has been through a lot as she has helped little Lydia to battle Batten Disease. In the process, she has shown great courage and determination to do the best that she can for her daughter. Yet another amazing person and mom, and I am so happy that we got to meet in person. There are so many others and one is someone that I knew very little about until the weekend of the conference. I would leave knowing so much more about her and her dear family.
I was walking through the hallway near the conference rooms and I looked off to the side where I saw a smiling face. I said something like, Amanda? It was Amanda Lay who was yet another friend that I had met online. She has two beautiful daughters and both have CLN3 Batten Disease. What a challenge this must be. We had chatted briefly a couple of times online but not really much more than that. I had no idea at the time, but I would leave the conference completely impressed with this lady. She was there at the conference with her mom Dessie Lowe, her very significant other Jerry Newport, and of course, her two daughters Abby and Shayla. Me and Jerry hit it off right away as we are both former Navy men. Jerry, in fact, served in the Gulf war and that type of thing always impresses me. What really impresses me is that Jerry has taken on the role of father to Abby and Shayla. Both of these girls could be a necessary inconvenience to some, but not to Jerry. He loves both of them to the moon and back, and I can’t say that I blame him for that. Both, are very special! I really didn’t have that much exposure to Shayla as she was occupied elsewhere but I spent time being around Abby on a couple of occasions. There is no way that pictures could do her justice. She is extremely special. Watching the interaction between Amanda and Abby was amazing. Even though Abby’s ability to express herself is limited, she was able to communicate in a way that doesn’t require a lot of words. The love that exists between this mom and daughter was a sight to see. This all left a big impression on me. One that is lasting. I am sure that Amanda’s mom, Dessie, is the anchor for the family in many ways. At least, that was my impression. She is a really neat gal. Like mother, like daughter!
And speaking of really neat gals, I didn’t even know that she was paying attention. I had made an announcement on my Benjamin’s Daddy Facebook page stating that I was planning on attending this year’s conference (I had nothing better to do, lol). Anyhow, I received the nicest response in the comments from Bobbie Lewis (Riddle). She has one of the most beautiful little persons for a daughter and her daughter’s name is Tayla. Tayla has CLN2 Late Infantile Batten Disease. I have no way of knowing who reads these stories unless I am told. Come to find out, Bobbie has been reading the blog on a regular basis and she has been very supportive of my efforts. She referred to me as “the famous Batten blogger” in a photo that we took together. Now it has never been my goal to be famous and I doubt that I am that. Even so, I am happy to know that someone that is held in such high regard thinks that highly of me. I enjoyed having our picture taken together. Especially when little Tayla was in the frame. Bobbie put Tayla in my arms so that she could have a picture of the two of us together. What a compliment that was. I really enjoyed meeting Jay Riddle and sharing a couple of short conversations with him. Together, these people are a very charming family of four, and you know, there isn’t anything that they wouldn’t do for little Miss Tayla. That would include leaving their home and extended family in Australia. Bobbie is a school teacher that has put her career on hold to care for Tayla. Fortunately, Jay’s career field involves working for an American based company. This allowed Jay to do a transfer and move his family to the United States. Their hope was that by doing so, they would be able to get little Tayla in to a clinical trial for Batten Disease. I am so pleased to be able to say that this became a reality. Tayla is now undergoing enzyme replacement therapy and she is rocking the house with it. Yay Tayla! I am so happy to know and to have met the Riddle family in person.
There are not too many things that are more enjoyable than having a meal together with friends and family. This was definitely true at the conference. The food that was served was, for me, well worth the price of admission. Not only that, but seeing all of these Batten families gathered together was priceless. I enjoyed talking to a few of the families I had just met and I very much enjoyed getting to better know those which I had become well acquainted with through social media. I spent time with Marlo Schinell and Dante’, as well as Dixie Bergeron and her rockstar son Jacoby. Jacoby is quite the character. Alicia Headrick was there with Miss Lydia and Jacoby made sure that Lydia’s face got wiped clean to his satisfaction during the meals. Awesome Job Jacoby. I already had a ton of respect for Dixie before the conference and that was only helped by meeting her in person. She has done so much for Jacoby regardless of whatever circumstances she might be facing at any given time. I got to meet her daughter Myracle as well and she is quite the little lady. Her and Jacoby are, of course, very close and that was evident in person. Marlo is another awesome Batten mommy. She is an educated person but she has set aside her career ambitions in order to care for Sir Dante’. As I said before, Dante’ is a real teddy bear of a guy. I Don’t know what got him going at dinner, but he started laughing hysterically and it was so funny. His laughter is infectious!
I had just finished dinner soon after the start of the conference and I was headed for the exit. I happened to look at one of the tables where I spotted yet another smiling face. Of course, I had to stop and say hello. This person turned out to be Laura Johnson and she was sitting with her daughter Hannah. I had been friends with Laura through Facebook but I had only chatted with her a couple of times. Her lovely daughter had been diagnosed with CLN3 Batten Disease only two months prior to the conference. Laura felt strongly that she needed to be present with Hannah at the event. When it comes to Batten mommies, I am amazed by their determination in doing what often needs to be done for their children. They both wanted to be at the conference so badly and Laura had started a GoFundMe account for just such a need. This, in order to travel to Nashville. She had no idea, even until right before the conference, as to whether or not they would make it to Tennessee. People responded generously and they helped to make the trip possible for this warrior princess and her mom. What is amazing to me is that Laura would drive her and Hannah for thirty hours straight in order to get there. I have a nickname for moms like this. I call them Tiger Mommas. Dixie Bergeron fits into this mold perfectly and Laura is yet another fine example. There are so many that I have grown to respect. I am so glad that Laura and I got to meet and talk. Hannah is amazing. She is soft spoken and really sweet. She answers questions well and seems to be accepting when it comes to her condition. The resilience of these kids is something that I find so inspiring. I don’t yet know Hannah’s complete story but she seems to accept the fact that she is legally blind. Hannah really enjoyed doing crafts and making the things that she was so proud to show everyone. She seemed so happy to be there and I really enjoyed spending time with her and her mom at the conference. I am sure that it was no mistake that our paths crossed and it was such a pleasure to meet both of them. Their life together is a story that I would love to tell someday soon and our meeting is something that I will not forget.
There were a few surprises for me at the conference and this is one that I never would have suspected. I was in the lobby at the beginning of the event and I was surprised by the appearance of a very special young man. I looked down and who would I see but Daniel Brown of Daniel’s Defenders! There he was in person with his handsome face and thick brown hair. I am so glad that he brought his parents with him so that we could also meet. I had chatted with his mom Lori Anne Marie on a couple of occasions and I am also FB friends with her husband Tony Brown. Theirs is a journey that I have followed for some time now. I was happy to meet both of them and that was something that I hadn’t expected to happen. It was nice to meet Daniel’s dad. We talked a little bit about family life and each of our lines of work. Lori has done a fantastic job of sharing Daniel’s journey with all of us through the Daniel’s Defenders Facebook page. She is an outstanding Batten mommy and I loved the fact that I was able to meet her as well. Their devotion to handsome prince Daniel has been well chronicled through Lori’s posts. As a family, they help Daniel battle Battens and they will not give up! Yet another inspiring family and it was a real privilege to meet them. In fact, there were so many wonderful families there. Why are they wonderful beyond what a normal family would be? It is because of what they face together and how they care so much for their warrior prince or princess. Some families have both.
Another one of these dear families is the McCorkles. That would be Matt and Misty playing the part of Batten Dad and mommy. Their handsome young warrior man is Micah and Micah has a awesome big brother named Maddox. It was such a pleasure to meet this charming young couple and their little prince. It may be a bit of a stretch but there was a small connection between us. You see, they are from the great state of Louisiana and that is where I spent much of my time in the military. In my case, I spent my time near New Orleans, whereas they are from the Northern part of the state. I have some very fond memories from that time in my life and so I appreciated the fact that they are from the Pelican State! As a family, they had to transplant themselves to the state of Ohio so that little Micah could receive the enzyme replacement that is available to kids with CLN2. They have since returned home because the therapy is now available at their place of origin. How wonderful! We talked a bit about my blogging and also about the things they face as a Batten family. We talked about Louisiana too! The best thing that we talked about was Micah and what a special guy he is. You know what? These kids are all so special and they all have a big piece of my heart. It was so nice to meet and talk to Matt and Misty. It was really special to meet Micah the prince!
I could go on forever, talking about these families but what about the hosts of the conference. In fact, I wonder. Where would these families be without BDSRA? Certainly, social media is a huge outlet and way to connect the families together. I am sure that a lot of them simply pick up the phone and talk. It is as I had said earlier. The conference is just like a family reunion, and in fact, that is what it is. The conference is such a big undertaking and it would be enough if BDSRA existed just to support the event. They do, in fact, do so much more and I am certain that having such an organization helps to bind the Batten community together. Just think about what it would be like on an emotional level to be told that your child has Batten Disease and not knowing where to turn. You find out about an organization that can offer support and they provide you with the resources that you need at the beginning. I am certain that BDSRA helps to connect newly diagnosed family members to those that are farther along on the journey. Then, there is the research support that they offer. Research doesn’t happen without funding and having an organization such as this is vital to that cause. I am also certain that they provide a connection between the various researchers, as well as, people in the medical community. I am new at this but I am certain that the Batten Disease Support and Research Association is a huge support to the Batten community, and that Dr. Margie and her staff have a crucial role in the battle against Batten Disease. There needs to be a voice so that people can hear and that is a big part of their mission as well. I was so thoroughly impressed with the conference and it would not happen without them. I know that there are some key people that serve on the board and that they help to steer the organization in the proper direction. I have never been a witness to any event such as the one I attended over this particular weekend. I may be a little biased in that though.
In the beginning, I had not purposely sought to have a role as an advocate for children that have Batten Disease. I just found that I loved telling these families stories. I know that there are people that are more capable than I am and so I appreciate every family that gives me the opportunity to write about them. The most recent story that I have done about a family here in the States is the one about little Conner Beish from Maryland. I called the story “That Kind of Love - Conner’s Story.” and it was one of the most complete stories of them all. This is only because Conner’s mom Hollie did an outstanding job of providing me with the information that I needed. It was wonderful to be able to meet Conner in person and I am so glad that he brought his mom and dad with him. Jeff and Hollie Beish turned out to be the kind of people that I thought they would be. Extremely nice! Conner’s older brother Jaxon is the best and I would have liked to have said hello to him. He was, however, always on the move. Awe, that’s okay Jaxon. Together, these folks are the kind of people that I would love to have for neighbors. Just seeking to be an average family, they have been asked to do things that most average families will never have to deal with. Jeff puts in long hours at work to support his family and that is something that I can relate to. I really enjoyed talking with him. Hollie is a special mom who takes care of business and she, without a doubt, loves her kids immensely. It was very special to meet this family in person after having written their story so recently. Conner is such a handsome little guy and as a family they are all Fighting For Conner! You can certainly understand why they would!
I cannot always put it into words as to why it is, but these Batten children do something to me. Perhaps it is my own position as a parent to a person with special needs combined with the feelings that I have about the plight of these children. The way that they battle and show us their resilience. They just amaze me, and to have so many in one place was truly special to me. I was so happy to meet the Swindall family at this year’s conference. Again, I had Kirsten Swindall on my friend’s list but had never had any interaction with her online. Her daughter Brianna is yet another very special warrior princess with CLN3. I also got to meet Kirsten’s husband John and their other daughter Hope, briefly, at dinner. The Swindall family are natives of Tennessee and I found them to be extremely pleasant and very personable. It is so easy to see the love that they have for their daughter Brianna and I can easily see why. I was able to spend some time talking to her during breakfast time, twice, and that was truly special. Brianna was very happy to show me the crowns that she had made and she wore them proudly in her hair. She also loved playing with her spinner toys and she loved the food at the buffet breakfast. This princess, with her bright red hair, was so happy to be living life in spite of all that had taken place and she was a joy to be with. Brianna is just so sweet and accepting. I enjoyed my conversations with John and Kirsten. At one breakfast in particular, Kirsten was able to share with me much of what life has been like and what all the journey has entailed. This is yet another regular family that has been asked to do something that is extraordinary. That is to care for the most special of persons. Meeting people like the Swindall family is the big thing from the conference that will leave a lasting impression on me.
Of course, I had a lot of time on my hands being by myself over the four days that I spent at the event. It took me a little bit to figure out where “the crowd” hung out. I found them outside in front of the hotel and this is where I spent a lot of my time. I loved hanging out and just listening to everyone talk about life and what it is like for them. Some of these folks have children that currently battle Batten Disease. Others have children or grandchildren that have gained their precious little wings. Pam and Crystal (my two buddies) were always there and I loved hanging out with them. I also loved having Dixie and Jacoby around. Dixie is a hoot and she is one strong Batten mommy. I loved it when Marlo and Dante’ would show up for a bit. If you haven’t figured it out yet, meeting Marlo and her rockstar son were a big deal to me. Dessie Lowe spent time out front and I enjoyed our conversations. Jennifer Whitfield Vaughn was frequently there too. She has the most darling little warrior princess for a granddaughter and her little name is Jayline. There were many others. Some I introduced myself to and there were others in which that wasn’t necessary because social media had done that for us. These are all special people that have been asked to do something which is much more than ordinary to me and so I listened carefully. I am by nature fairly withdrawn until I get to know someone and I would like those that I didn’t reach out to, to know something. I want everyone in the Batten community to know how much I appreciate them for all they do as parents and caregivers to the most priceless children in existence (I’m injecting my personal feelings here).
I do not have words that can adequately describe what being in the memorial room was like for me. I am sure that it would be very different if I had lost a son or daughter who had battled Batten Disease. The memorial service itself was both very moving and very fitting. It is so important to never forget those who have battled. I was able to view the names of those children that I had followed closely, as well as, some whose names were very familiar to me. I saw the name of the one who had made me aware (the most beautiful name) and those who were her contemporaries. I do not not remember the gentleman’s name, but I was able to talk with one of the people who were responsible for the memorial’s creation. He himself had lost a child to Batten Disease. He told me all about the memorial and shared a lot of interesting facts about some of the names that were on it. This has all served to deepen a sense of responsibility in me. What an absolutely special group it is that makes up the Batten community. These kids change people’s lives even though their time on this planet is limited. I have tried my best to capture this fact in my writing.
There is no way that I could adequately express to you what this entire experience has meant to me. You may have guessed that being at the event has left a lasting impression on me. I was told that there were over 400 people at the event and it would be impossible to mention everyone that I came in contact with. Please know that each family is looked at in a very special light because of what the Batten journey entails. There are a few others that I must mention. It was so nice to meet the Hiltman family. Both Jay and Stacey had some very kind words for me and it was so appreciated. Both of them were very personable.They have done a wonderful job of sharing their son Nathan with all of us, as well as, sharing the journey that they are on as a family. It is easy to see that Nathan brings challenges with him but he has so much charm and he is an extremely special guy. I don’t know where the connection with Edward Scissorhands originates but he is apparently one of Nathan’s favorite characters. That is so cool Nathan and it was so nice to meet you and your family!
The hotel was quite big and had a lot of stories. That meant that unless you wanted to climb stairs, you had to wait at the elevator. I met a few different families while I waited. Previous to the conference, I had started to follow a page called Little Batten Warriors but I had not yet spent a lot of time on the page. This is because I generally am involved with one family at a time as I write. The page is about the journeys of both little Kaleb Diaz and his sister Mia. They both have CLN2 Batten Disease and both are undergoing enzyme replacement therapy. So in reference to the elevator. I was waiting and had an opportunity to say hello to Kaleb and his father Alex. I have to admit that I goofed up and mistook little Kaleb for a girl when I first spotted him with his dad. Why the mistake? Well you see, this entire family happens to be very attractive and they have let little Kaleb’s hair grow out. Kaleb is a very cute little warrior man and his sister is very very adorable as well. I saw both their mom, Barbara, and little Mia at the conference but we were both too preoccupied to make contact with one another. Barbara did, however, contact me after the conference had ended via Instant messaging to express her appreciation for what I am doing with my blog. I can’t tell you how much I appreciate things like this! I did spend a few minutes talking to Alex and it never would not have happened if it was not for that wait at the elevators. I hate the fact that Batten Disease exists because of the way that it affects young families like the Diaz family. I do, however, so admire the way that they handle the challenges that have come their way and I promise that I will pay closer attention in the future. It is so nice to have met the Diaz family. Little Kaleb and his sister Mia are precious and priceless and they are each, a little warrior. One is a prince and the other, is a princess. Alex and Barbara also have a daughter who is unaffected by Batten Disease. Her name is Jaylen and she is now thirteen years old.
Oh my gosh! I could go on forever. It was so nice to meet Kayla Neveri in the Lobby at Starbucks. Even though our time together was confined to a single conversation, it was special and we continue to converse online. This, as she shares about her little princess Breanna who has Battens. I enjoyed meeting Michelle Wheeler who is a very nice lady. She has a handsome grandson named Login who has Batten Disease. I am glad that we had the opportunity to speak with one another!
I ran into a couple in the elevator on the first night of the conference and their names looked familiar to me. It was Dean and Melissa Pollman and they just happen to live in a city that neighbors the one I live in here in Oregon. This is another couple that I have never chatted with online but there we all were, in Tennessee, at the Batten conference. We had a nice conversation at the reception and I hope to see them again sometime. Very nice couple indeed! The Pollman’s have a beautiful daughter who has CLN1. Her name is Haley and she was not in attendance at the conference. The Pollman’s have set up a foundation for Haley and they also have a Facebook page for her. The name of the page is Haley’s Heroes Foundation. She is a most adorable little girl. Please check out her page if you haven’t already.
It was on Saturday night that I was approached by a dear person named Monica Bearman and she obviously knew about my blog. In fact, she was another Facebook friend that I had never made contact with. She later introduced me to her husband Adam and they turned out to be really nice people! Monica expressed interest in doing a story about her princess of a daughter whose name is Kristina. You just know that I could never pass up an opportunity like that! I look so forward to the knowing more about this family and it was a pleasure to meet them.
There are others that I could mention and I hope that you will forgive me for not doing so. I absolutely have the utmost respect for all who love and care for these children.
It was huge for me to be able to meet Tracy and Jennifer Vanhoutan at the conference. For them, the battle against Batten Disease is personal. Not only did they have a handsome son named Noah with Battens, but also a darling daughter named Laine. Both are now beautiful angels. Tracy and Jennifer remain active in raising awareness and in trying to bring about change. Tracy has served on the board for BDSRA and they are both extremely knowledgeable about Batten Disease. This couple is pure class, through and through, and it was a privilege to be able to meet them! The community that they remain a part of is very fortunate to have them both in their corner.
Let me say that I am always looking to connect with new people within the Batten community and also those who advocate for these families. I ran across Kristy’s name at some point on Facebook and I sent her a friend’s request. She was somehow connected with a charming Batten child named Bentley Betts, however, I wasn’t sure how that was at the time. I came to find out that she was Bentley’s home care nurse while he was nearing the time in which he would complete his journey. Kristy Thetford Prince’s life was changed as a connection was made with Bentley and his family. I think that Kristy and I both took notice of each other’s involvement in the Batten community. This, as we noticed each others activities on FB. Both of our lives have been greatly affected by these kids as we follow their family’s journeys. As Bentley’s nurse, Kristy was deeply connected to this little warrior of a young man. Her’s was a first hand encounter of a very special kind. This is how she would put it to me, “From the moment I became Bentley’s nurse, my life has never been the same. In saying that I mean I never knew about the disease ‘Battens’. I read and studied about this disease before I started with Bentley but It still did not prepare me for the extraordinary experience that changed me from that day forward.”
My own experience cannot be compared to that of Kristy’s but what I have experienced through writing these stories is notable. Having read multiple accounts and attempting to put them into words that do the stories justice has changed me. Kristy talks about her feelings towards Batten families and I can only say that I try to relate to her as best that I can. This is how she finished her comments as she shared from the heart concerning these families, “I love them and I pray daily for these children and their families. I strive daily to remember to be thankful and I keep Bentley close in my thoughts and I draw my strength from him. When things seem tough on my end, I see his smiling face. I hear his laugh and I know he's in Heaven running around and whole and I know I shall see him again one day.” It was an absolute pleasure to be able to meet Kristy at the conference. It was also so nice to see little Bentley’s parents, Barrett and Laura Betts, at the event. My conversation with Barrett was brief but meaningful and it was nice to meet in person. I am sure that things haven’t been easy for this young couple and I am so glad that they could be in attendance and be surrounded by members of their Batten family. It is easy to see that Kristy and the Betts family share a bond that won’t be broken. The bond comes from a shared love for little Bentley. These children change people. I know that to be the case with both Kristy and myself. This is not even to mention the impact that they have on their family members and others that are close by.
Being at the Batten conference and connecting with many of these families in person is an experience that will stay with me. Many that I met have made a lasting impression on me, and their kids? They are, to me, the most special children on the planet. I will never be able to completely put it into words but I know it to be the truth. This is the way I feel. I am so grateful to the folks at BDSRA for allowing me to attend the conference. It was so nice to meet Dr. Margie Frazier in person and to shake her hand. I also appreciated meeting Tracy Kirby. She made me feel welcomed at the event! The venue could not have been anymore spectacular. I am glad that these families have each other and I am also glad that they have other people in their corner. It is wonderful to know that the Batten Disease Support and Research Association exists to serve those who are so deserving (Funding, Research, and support for these dear families as we look for a treatment and cure). I will always consider it a privilege to be involved in the Batten community and in the lives of those who Battle Batten Disease. The stories that I have been allowed to work on have left a lasting impression on me. Can you tell? I hope so because it is true. Being at the event was a special time and a time that I will never forget. More than anything, these kids and their families will always be etched in my memory. Thank you allowing me this special privilege and thank you for taking the time to read about the experience.
Blogger and Advocate
“He is my heart. When he laughs I laugh, when he cries I cry. His good days are my good days and his bad days are my bad days. I have so much love for him that many days it hurts, pains me to think that one day he will not be here with me.”
Why would you continue to read this blog and the stories that are in it? They are all the same. Aren’t they? Another child with Batten Disease. You say to yourself, yes, I get it! Sounds horrific, but their stories just all seem to be the same to me. Not only that but the blogs just seem to get longer and longer. The length shouldn’t matter to those that are interested. You would take the time to read a good book. Wouldn’t you?
A young mum from Plymouth in the UK is faced with a dilemma. How can she possibly care for her son who has Batten Disease? This, while being alone and trying to establish herself in life. It is no accident that her very own mother is trained in the type of nursing that is needed to care for this most handsome little warrior. She performs, in this case and in my opinion, a very selfless act in that she turns over the care of her son to her mum. The bond between the son and grandmum is incredible and the most beautiful story is knitted together through the love that they have for one another. This quiet professional of a lady leaves her profession in order to care for this precious warrior day and night. They literally would cling to one another until this incredibly beautiful angel of a boy was ushered into eternity. Their story is perhaps the sweetest ever told.
Later, and from four Thousand miles away in Chattanooga Tennessee, a young mother is devastated by the findings. Her daughter has a disease for which there is no cure and, it is fatal. All of a sudden her princess has a life expectancy of no greater than 12 years of age. This mom’s search for help leads her to a webpage by which she learns of a clinical trial that offers hope for increased longevity. This, in hopes that a cure can be found. Like the handsome prince in Plymouth, this princess has CLN2 Batten Disease. This trial would provide a replacement for the enzyme that was missing in her daughter’s brain.The only problem was that it was not available where she lived. This help would require that she move seven hours away to Columbus Ohio. Without hesitation, she packs up and leaves behind everything that is familiar to her. She very bravely does what is necessary to get her daughter what she needs to battle the disease. She told me that “anyone” would have done the same thing. I don’t believe that is true. The bond that has been created by facing this battle together, away from home, has become incredibly strong. This little warrior princess is the center of her momma’s world and for good reason. Just one look and you would know why.
The same kind of bond would begin to be developed in Jamestown North Dakota. This, testified to by a women’s actions. A single mother of six children fights to find the answer for what is taking place with her youngest daughter. She struggles, initially, to find a neurologist that will look beyond the obvious. To her, it was obvious. There was more going on with her princess than just the seizures associated with an elevated temperature. Her efforts would find her with a new doctor and testing would take place that would reveal the answer. Her baby doll of a girl has a disease that her mom, in all likelihood, never knew existed. She, also has Batten Disease. The same treatment that was available to the princess from Tennessee was also available to her daughter. The only thing is that the treatment was only available many hours away at the Mayo Clinic in the state of Minnesota. Impossible to move there, she did what she had to do. She would make three trips in two weeks just to set up the treatment, then came the time. Leaving her other children in the care of family, she packed her little girl in the car and made the trip to Mayo for the treatment. That same evening her daughter was released and they made the long trip back home in the dark of night. This, so that mom could attend her oldest daughter’s last track meet of the season. That very same day. She had missed all the others because of her quest to get the answers that were needed for her Batten princess. Her’s is another story worth telling (They all, are worth telling!)
There are so many others. Whether or not there are difficult circumstances attached to the story, each one is significant. I have shared, in the past, a comment from a well known Batten mom named Bekah Bowman. It was written concerning this blog and it would be impossible for me to put into words how much it still means to me. She obviously speaks from experience as she comments, “While you share ‘batten stories’, each family has their unique journey of traveling through such brokenness and every one of those kids and their families have something valuable to teach the world. I love that you continue to share my fellow batten family's stories. They are all incredible stories of courage, pain, joy, beauty, grief, love and more. All deserve to be told.”
All do deserve to be told and will be told as long as I am able (I know there are others that are more capable). This next story is no exception. It doesn’t matter what the individual circumstances may be. Each of these little warriors brings with them challenges that have to be met by the parent or caregiver and most often by an entire family. The biggest challenges seem to be the ones dealt with on an emotional level. As I was beginning to receive information from Hollie Beish concerning her son Conner, she was sitting at the hospital with him. You see, Conner was in the process of receiving his 26th infusions of the chemical that is named Brineura. This chemical takes the place of a missing enzyme that is responsible for cleaning out wastes that would otherwise allow the progression CLN2 Batten Disease. The treatment is done every two weeks. It is easy to tell by doing the math that Conner has come to his one year anniversary since he started receiving the infusions. This was a really big day for him and his family and I know that they are thankful that such an anniversary was possible. This after going through the range of emotions that are connected with being told that your child has a fatal disease for which there is no cure. The treatment, though, was available. As should be the case, these stories most often begin with two people that meet and fall in love. It was no exception for Jeff Beish and Hollie Blades. It all began as Hollie was working as a manager at a local drugstore in Middletown, Delaware. Jeff “just happened” to be the delivery person for Coca Cola at her store. They would often chat with each other when their days at work came together, and of course, there was an interest in one another. This did not escape being noticed by Hollie’s friend, and coworker, who decided to help things move along. She asked Hollie if she would be interested in dating Jeff if the opportunity came about. To this Hollie gave a big yes in reply. Hollie ended up receiving Jeff’s phone number from her friend and the couple really continued to hit it off. They were engaged to be married about a year later and then became man and wife in October of 2006. As their relationship developed, Hollie would make the move from her home state of Delaware to the neighboring state of Maryland. This was where Jeff lived. Hollie’s parents would end up moving there as well in order to be closer.
As with any other couple they had talked about what their plans and dreams for the future would be. There was of course so many things discussed and they both agreed that children were part of their future. Jeff and Hollie both wanted two kids but they took some time first to enjoy their new life together. Their plans began to come together when they welcomed a brand new baby boy into this world. This would take place in June of 2009. They named him Jaxon! He was so perfect to this happy couple. He had all of his fingers and toes, and he was there very own son. Couples look so forward to that expectant day and this day belonged to them and Jaxon. As Jaxon grew, so did the challenges that he brought to this couple. He was so full of personality and also very sweet. Jaxson was the center of attention and he made sure things stayed that way. He stayed on the move from the first time that his legs took flight and he kept his momma busy all throughout the day when they were together. Jeff and Hollie loved being parents and so they continued to work towards completing their family.
The couple did not know at the time but they would soon begin a journey together as their family grew. This was to be a journey that neither of them knew existed. It all began as Hollie found herself pregnant for the second time. They were delighted with the fact that there would soon be a playmate for Jaxon and their family would be complete. The day arrived. It was August the 18th, of 2012. That was the day that a very special addition to the family made his arrival and they named him Conner. Conner was born at Anne Arundel Medical Center in Annapolis, Maryland. He made his appearance into the family a week before he was expected. This because Hollie was having issues with high blood pressure. The doctors felt that labor should be induced and so they did. The delivery went well and before they new it, Hollie was holding Conner in her arms. He was so adorable to look at. He had reddish hair that would later turn to brown and he was so perfect. His older brother had been a big baby and looked to be three months old at birth. However, Conner, by comparison, was much smaller. He looked like a tiny newborn with lots of wrinkles and holding him made Hollie a little nervous. With the exception of a small concern initially over Conner’s breathing everything went just fine. As a baby, Conner was very laid back and lessor of a challenge than his big brother had been. He was a go with the flow kind of a little man and that suited his mom and dad just fine! He completed them as a family and it seemed that everything had come together for Jeff and Hollie. Who wouldn’t agree with that? They were two people that loved each other and they loved being parents.
Not only did Jeff and Hollie have the family that they had wanted but, as the boys grew together, they thrived together as brothers. Just as it was with Jaxon, Conner developed normally and he would reach all of his major milestones. The only thing that was noticed was that his speech was not developing normally as it had with his peers. This, as he approached two years of age. It was at that time that his pediatrician recommended that Conner be placed in speech therapy. Conner would continue to develop normally with the exception of his speech. In that area, he continued to struggle a bit. This, However, was not a cause for alarm. Often times, as we are growing up in a family, we have older siblings that we look up to. This would become the case with Conner when it comes to his older brother Jaxon. According to Hollie, Conner really looks up to Jaxon and did so from the beginning. Sometimes, big brothers gain a hero type status with their little brothers who look to emulate them. This is true in this case because Jaxon is an amazing big brother and he has been from the very start. There were no jealousy issues with Jaxon when his new little brother came into this world and he was so excited to have a brother. In fact, he looked for every opportunity to help with Conner. As a result, there has been an incredible bond formed between the two brothers. Jaxon loves his little brother and the feelings go both ways. Jaxon is Conner’s hero in a brotherly sense. When Conner was a toddler, he would follow Jaxon around and try to do the things that he had seen his big brother doing. Hollie said that, as a result, Conner became a daredevil. There were plenty of bumps and bruises for Conner and several mini heart attacks for her. Hollie sums up the connection between them with the following, “Conner has and still does think Jaxon is awesome. He looks up to him and loves him so much. When Conner gets hurt he will often ask for his bubu (that’s what he calls Jaxon). Bubu comes to the rescue and makes him laugh, forgetting about whatever caused him to cry.”
You get the picture, right? Normal family life is taking place. Sweet memories are being made and plans for the future are being thought through. Every family has their struggles but some are called to challenges that are above what one would expect. This family was in a good place and one would expect things to continue on that way. Things would change though. It was October of 2015 when Hollie received the most frightening call a mother might could get. Conner had a seizure while at school and had been taken to the hospital. As is often the case, a first time seizure is labeled as a febrile seizure. This is the type that is associated with an elevated temperature. This is what the staff at the hospital concluded in Conner’s case. The family was told that this was not a big deal and they were sent home. There were, however, more seizures that would follow and, according to Hollie, these seizures were unprovoked. As would be expected, an appointment to see a neurologist was made for Conner and with this, the search for answers would begin.
Once at the doctor, an EEG was ordered and as you might have guessed, it showed that something in the findings was abnormal. As is most often the case, the journey begins with a diagnosis of epilepsy. You can imagine that this would be so disheartening to any parent. All of a sudden there is a health concern that will greatly affect a child’s life into adulthood. Epilepsy is something that could be overcome and it most often is. Medication was prescribed for Conner and it held the seizures at bay until May of 2016. As is the standard when epilepsy is diagnosed, an MRI was ordered. That had taken place in February of the same year and the results were received a couple of weeks later. Conner’s MRI showed some things that caused concern and as a result, the doctors wanted genetic testing performed. The team of physicians were on to something. They felt that there was something that tied everything together - the speech delay, the MRI results, and the epilepsy. The family would one day find that to be the case, but for now, the search continued. As May of 2016 rolled around, Conner had another seizure like the ones he had experienced before and his medication was increased slightly. This is normal for the control of seizures, however, it was later that month that there was a new development. Conner started having a different type of seizure. Can you imagine what it must have been like as Conner’s parents? How the level of concern continued to increase? How hard is it to carry on with life when you have this type of distraction and concern? Conner began to suffer from atonic, or as they are commonly referred to as, drop seizures. Again, the medication that he was receiving was adjusted. This, however, would not help stop the drop seizures that were occuring. In fact, this was the advent of the scariest time for this young family. I will use Hollie’s own words to explain, “The summer of 2016 was horrendous! Conner had seizures like never before and they lasted so long that he had to be hospitalized multiple times and receive rescue medication each time.” As someone that follows these kids journeys, It is difficult enough to read about the timeline of events that are part of these stories. What is it like to live through the events themselves? Can you imagine laying your child to bed at night after experiencing these things on a daily basis? This, while not yet knowing the cause. It must have provided many a sleepless night.
It was imperative that Conner’s medication be adjusted again. As a team, the doctors and family decided to re-introduce a medication that Conner had been taking before to control the seizures. This got the long and very frightening ones under control. It, however, did not get rid of the drop seizures. The search for answers would continue and with that came a new diagnoses. The doctors diagnosed Conner with Doose Syndrome. This is a rare form of epilepsy that is characterized by seizures that are difficult to control. It can also cause learning disabilities and so it may have seemed like the proper diagnosis at the time. It was, however, just another step in this journey. Hollie said that while all this was going on, they were working on getting the results of a second round of genetic testing. The first tests came back with no findings and so the doctors wanted an epilepsy panel performed. They would do so and the results would raise some big concerns. The panel looked closely at the genes that affect a person’s tendency towards epilepsy. According to Hollie, the panel looks at any genes that, when affected, causes seizures. Some of the genes that were looked at are linked to Doose Syndrome. The testing also checks the genes that come into play with Batten Disease. They would not get the results from the 2nd set of genetic tests until October of 2016. That was a year after Conner started having seizures. With the results, Conner’s family received word of a possibility that Hollie would call “downright scary!”
They were told that Batten Disease was a possibility when the results came back, but also that it was unlikely to be the case. Jeff and Hollie were also told that more testing would have to be performed to rule out Battens. This time it would involve genetic tests for Jeff and Hollie. Conner would also need additional blood work done. They were encouraged to not go home and research the disease. In the meantime, receiving the results would take three months when it should have taken only about three weeks. You can only imagine the extra anxiety that this caused this young family. There were multiple mistakes that were made by those responsible for the testing and blood had to be redrawn. I had made a remark to Hollie about how stressful the wait must have been. Her reply says it all, “Yes, very difficult! Our family was falling apart and we were all so stressed out and worried. Conner was also declining rapidly during that time. I hoped the results would come back saying he didn't have it, but I knew in my gut it was Batten. Being in limbo was the worst. Watching him decline and not knowing how to help him or what to do was impossible.” I don’t know why doctors tell a parent to not research the disease. They should know that every parent will. Hollie described what she had seen when she did some research and also, what her response was, “I saw the words fatal, genetic, no cure, no treatment, progressive, average life span 12 years old. I was scared to death and hoped with all my heart it was something else, anything else.” They would actually receive the news over the phone. How very impersonal. This left them devastated and angry and who would think anything but that this would be the case. People sometimes make mistakes but It took way too long to get the results.
According to Hollie, Conner had two variants on a gene that caused NCL (Neuronal ceroid lipofuscinosis), or as it is commonly referred to as, Batten Disease. In order to conclude that this was possible, both Jeff and Hollie would have to be tested. This because they would both have to be carriers in order for Conner to have Battens. Blood was also to be drawn from Conner to see if he was deficient in the enzyme that is missing when Batten Disease is present. The testing was complicated by the fact that Hollie carried a variant that had never been seen before. After what must have seemed like forever, the news that they had received over the phone was so disheartening. Conner had CLN2, Late Infantile Batten Disease. It took over a year from the time of the first seizure until they finally received the diagnosis. How the tension must have increased with time and circumstances. The results of Conner’s tests came back the following January. His enzyme level was deficient and he was given the diagnosis of Battens. This makes me wonder what the Christmas season must have been like for this dear family. I have done other stories where the outlook for these families goes from bad to worse to the unimaginable. One can live with epilepsy and even with Doose Syndrome. We cannot, however, imagine what it is like to finally be told that your child has Batten Disease. A disease that they had never even heard of. And, it was fatal.
What is it like to receive this kind of news? What was it like to end the phone call and to be left there alone together in the silence of the family home? By instinct, knowing that difficult times were ahead, and yet holding out hope that the news could be better. That hope dissipated by that which you wished you had not heard. Hollie described what she was feeling in the most telling fashion, “I remember feeling like I couldn’t breath, my legs were like jello holding up what felt like 500 lbs. I felt sick to my stomach, nausea took over. It was the worst day in my 35 years on this planet.” There is a common theme that runs through all of the stories that I have done so far. It starts with the devastating news that brings total shock and despair. Your child has a fatal disease for which there is no cure. Then, after processing the information, determination takes over. With that comes the will to do your very best for your little warrior prince or princess. Undying love and tender care become the order of the day. This would all begin with Conner’s parents as they processed the information. Like every parent or caregiver mentioned before, they would begin to do what is necessary. This is how Hollie would put it, “Now I will say, a few days later, I felt a weight lifted. We now knew what we were facing and what to do next. It was time to fight for Conner’s life! The results came on a Friday and we spent the weekend taking it all in but on Monday it was time to get to work. It was time to fight for this precious little boy and that is what we did and will continue to do.” And they would, in fact, continue to fight for Conner. This would take the help of family and community of caring people from all walks of life.
Like with most every family confronted with this awful diagnoses, Jeff and Hollie searched for what they could do next. How could they help Conner fight? The answer came in the form of a clinical trial that would replace the enzyme that was missing in Conner’s body. This was taking place in Ohio at Nationwide Children’s Hospital. It would involve traveling over 7 hours one way. If Conner was to be accepted into the trial, it would require traveling that distance every two weeks for each cycle of the infusion. The family first traveled to Ohio in March of 2017 and learned that they could not yet get access but that a spot may be opening soon. They also learned that they may have to wait for approval of the drug from the FDA and that wouldn’t happen until the end of April. They had to deal with the fact that their little man was digressing this entire time as they waited. This had to produce many anxious feelings for this mom and dad. Still, they did everything that they had to do to make sure that Conner would be approved. This included making sure that he had all his therapies while constantly checking on the approval of the drug. Finally, on April the 27th of 2017, they received the call that brought some exciting news. The drug, which is named Brineura, was now FDA approved and Conner was accepted into the trial. How exciting!
Conner had his surgery to have the port placed in his head at the end of May and he would receive his first infusion on June the 1st. His first time with receiving the infusion did not come without a struggle as swelling around the port was an issue. It took nine tries to access the port. Poor little Conner screamed with pain which in turn made mom, of course, cry out for her son. It was an emotional day but those things wouldn’t happen all the time. The process would become easier but that didn’t mean that there wouldn’t be sacrifices made. As I previously stated, the trip for each infusion was around seven and a half hours one way. Like with every child that undergoes the process, it has to be performed every two weeks. Conner and his mommy would have to be away from home for three days each time. Of course, Jeff would have to continue to go to work for his family and Jaxon couldn’t miss school. This would mean time away from one another and I am sure that would be difficult. This would take place for six months before any changes would happen.
Driven by love, a young mother is undeterred by time and distance. She sets aside the life that she knows and does what is necessary to help her precious warrior battle. This, as dad holds down the fort while working to provide. There are very few kinds of love more beautiful and none more compelling than this. It is that kind of love.
Although a routine would be established it would only be made easier by those who provided help and support. Those people would include family, friends, the medical staff, and the drug company. Things would even go well with approval from the insurance company. Hollie’s father, Bruce, had recently retired and was able to accompany her and Conner much of the time on these long trips. (There are many other stories that are similar in terms of time to travel. You see, Batten Disease, CLN2 variant alone, really isn’t that rare. From London to Ohio, there are others who make that trip in varying distances. Some have even left behind the home they love.) One person that needs to be mentioned is Conner’s older brother Jaxon. All of a sudden, the number one son in the house needed to, in many ways, take a back seat to his younger brother. This because Conner needed all of this extra attention. Jaxon still is an awesome older brother and Conner’s hero. Jaxon would be assured of his family’s love for him in this difficult time through words of encouragement and affection. This, while receiving the explanation of Conner’s circumstances. Fortunately, the family had the help of Jeff’s sister, Lisa, during Hollie’s absences as she lived right down the street. What a blessing! It would be a very difficult time for the whole family but Conner was so worth it. Just look at him!
Hollie stayed committed to to this demanding routine for as long as it took before a transition to Children’s National Hospital in Washington DC took place. Conner was to be the first child with CLN2 to receive the infusion and the staff would train specifically for him. He is so worth it. There is now one other child being treated at the facility in addition to Conner. This is what Hollie said concerning the change in hospital, “The transition from Ohio to our home hospital in DC also went smoothly. Children’s National trained and prepared for many months and it was clear they worked hard to make it as smooth and easy on us as possible.” As stated at the beginning, Conner has just reached his one year mark. That means that he has been receiving his treatments in DC for half of that time. I can only imagine how much it must mean to the hospital staff to be able to help Conner in this way. I just bet that they are very attached to him and the people there must look forward to seeing him every two weeks.
A Staff of dedicated and caring medical professionals train specifically to treat a child in desperate need of the care that he receives. Their team is part of a new treatment center within the U.S. and the response to the opportunity is the same as the rest. Their response to Conner is that he is so worth it, and you know what? He absolutely is!
How is Conner doing? According to Hollie, Conner is doing very well with the infusions. They saw some decline in him at first but he has actually made some gains as time has moved along. Hollie said it was at the four month mark that Conner began to become more vocal and alert. He hadn’t declined any more as of eight months and his vocabulary continued to thrive, as did his level of alertness. He is now seizure free at twelve months and his tremors have decreased a lot. His dexterity has improved and he can reach for objects as well. Conner walks with a gait trainer and can crawl short distances. He is even able to stand at the couch. He can kneel without assistance and sit up by himself for short periods of time. If it wasn’t for the Brineura, I venture to say that none of this would be happening. In Hollie’s own words, “I can’t imagine where he would be right now without it.”
Conner was granted a wish through the Make-A-Wish Foundation and this took place in April of 2017. Conner is a little Star Wars fanatic. How cute! His wish was to do battle with Darth Vader at Disney World and he would, of course, get his wish and then some. Although he didn’t battle Darth Vader at Disney he would do so at his send off party that was attended by the 501st Legion. The members of the 501st make appearances wearing authentic replica costumes of Star Wars Characters, like Darth Vader. These guys are first class and their presence made Conner’s day. While at Disney, Conner attended the Jedi Training Academy. He appeared on stage with Darth and ended up battling another Star Wars character while at Disney. The family stayed at Give Kids the World Village and they would attend Universal Studios and SeaWorld as well. You just know that Conner and his entire family had a blast. I think that it is so wonderful that there are people in this world that show that they care about these kids the way that these organizations do!
What is life like for this family going forward? This is part of what Hollie had to say, “Batten Disease has greatly affected our family in a variety of ways. In a way it has brought us closer. When you know your days together are numbered it makes it that much more important to spend time together making memories. We all help each other and make sure Conner is as happy and healthy as possible. We understand each other and what we are going through.” I always feel that it is a wonderful thing when the struggles in this life draw us closer together. I feel that is the way that it should be. I know from experience that having a special needs child in the house affects siblings. It stands to reason that Jaxon’s life has been impacted by his brother’s plight. Hollie says that they try to make Jaxon’s life as normal as possible but she has expressed the fact that Jaxon is having to grow up faster through all of this. As I mentioned earlier, Jeff and Hollie do their best to provide a foundation of love and understanding for Jaxon. I just know from my chats with Hollie that Jackson is a rockstar of an older brother and he will continue to be a hero to Conner. I don’t know this for a fact but I am thinking that I am on the right path by telling you this. I think that there will be a little bit of a reversal of roles as Jaxon sees his little brother continue in the battle. I think that Conner will grow to be Jaxon’s hero. I don’t think that there will ever be another person that will impact Jaxon’s life in the way that Conner’s life will. Of this, there is no doubt!
There is stress far and above what would be experienced by other families. As one might imagine, the financial concerns are greater. The fact that Hollie is unable to work because of Conner leaves a larger burden on Jeff. He is responsible, as the breadwinner, for the families income and and many of Conner’s medical care expenses. Dad has to work long hours but I have to believe that he assumes this heavier burden, willingly, out of the love that he has for his family. He looks to be a wonderful husband and father and I think that we would be good friends. Hollie is a warrior mom. She is left to care for Conner and to attend to his basic needs. There are always appointments to get to on top of all of her other responsibilities. Because of all the extra demands, both financially and otherwise, they are not able to do as many things together as other families might. When they do go out, there are restrictions and it requires extra planning. Certain pieces of Conner’s equipment has to go with him. Some of the activities that are only appropriate for Jaxon are attended by one parent only. Hollie has had to grow in the area of time management and she is always on the go. By her own admission, she still struggles in this area at times. I know that she sometimes wishes that things could be easier, but just like Jeff, she loves her family and she does what is necessary to get through each day. She has learned how to prioritize things in her schedule in order to accomplish every task that needs to be performed. She explains it in this way, “My priorities have definitely changed with all of this. I do housework and run errands while the kids are at school that way when they are home I can focus on them. I spend the majority of my time with my immediate family, mostly at home where Conner has everything he needs and is happy and comfortable. I do not stress the small stuff like I used to. As long as everyone is happy and taken care of, all is good.”
As a family, they take each day as it comes and they do so for each other. Looking too far ahead causes Hollie to feel overwhelmed. She is very practical in the way that she approaches the circumstances that surround them. Hollie has this to say, “I just do what I have to today and leave tomorrow’s worries for tomorrow. I take problems as they come and look for solutions. I have no way of knowing what research or treatments are in the future so I just hope that something changes before it’s too late for Conner. The same goes for challenges. I take them as they come and I smile and do my best to stay positive through them. We discuss them as a family as they come and help each other face them and figure them out together.” As a couple, Jeff and Hollie keep Jaxon in the loup but they are careful in what they say to him. Jaxon loves his little brother, no doubt, to the moon and back! Both of their kiddos are very special. Each in their own way. Hollie adds the following concerning her two special kids, “I am amazed by Conner’s strength and bravery! I am so proud of Jaxon. He is empathetic, caring, and sweet. I am impressed at how he is handling everything.”
The people within Jeff and Hollie’s community have been of great help to this family. They live in a small town and the people there have been very supportive. They know who little Conner is and awareness is being spread through his Facebook page which is called Fighting for Conner. His Batten Disease shirt is popular and a lot of people wear them for Conner. In fact, just about every teacher at his school has one. I know that he is a very well loved little man by all who know him. Friends and family worked together to put on a huge fundraiser for Conner and the Star Wars characters from the 501st Legion were there to help! They stay in touch with the family and also let them know anytime there is an event so that Conner can be there. Conner is such an amazing little man that he is even loved by Darth Vader. Go figure! Hollie says that thinking about all of the support that they have received still causes her emotions to get stirred up. She has this to say in regards to the outpouring of love and support they have received, “We have truly been blessed with so many amazing people surrounding us with love.” These kids have a way of turning regular people into those which are amazing!
A Family, a town, a medical community, a teaching staff, and even a legion of Star Wars characters. They all work together in support of one incredibly charming little man. Why? Because, he is so worth it. This most handsome little hero produces, in all of us, that kind of love and for that reason, he cannot be denied.
Again from Hollie in closing, “I love Conner as any mom loves their child. I would do anything for him, give my life to make him well, to allow him to have a typical childhood where he can run and play, talk, and read and write. However I will say, this horrible disease makes it easier for me to just love him without worry. I don't have to worry about him doing homework or chores, behaving badly or disciplining him. I just get to love him and play with him, and have fun. He gets to do what he wants and I can give him everything without having to worry if it will impact him negatively, all the stuff I have to worry about with my 8 yr old.”
I am the parent of an adult who has special needs. He has been with me practically every day of my life for the last 28 years. The bond between us is incredible. He needs me and his mother for some of the most basic things and I think that has increased the bond between us. Demonstrating the love that I have for him is one of life’s greatest pleasures. I only make comparisons to what a Batten family goes through in an effort to relate to them but afterwards, I remain honest with myself. I really don’t know what it is like to be told that your child has a terminal disease. I think that part of my reason for writing is because it serves to help me understand. I continue to be amazed at the level of devotion that these parents show their children and I also am amazed by these kids that I have grown to love. Their resilience and the things that they teach us about themselves and those of us who follow them. They change our perspective about what is important. What is to be valued. They are able to impact people’s lives like none other. At least that is the way things are from my point of view. This may seem a little out there, but I believe that these kids are sent to us for a purpose. Their lives, even if cut short, will impact people as none others would after a lifetime. They change us. This is where my heart is and this is why I write. For this reason, Conner is my hero also!
Blogger and Advocate
Where am I? What kind of a title is that? Well you see, I am in between blogs about Batten warriors right now. There will be more on the way if everything goes well and do I expect it to.
The last two blogs were quite a bit of work. Lucy Faith did a fantastic job of providing me information and I thought that everything went well. The more information that I am given, the more detailed the story will be and I was given her family’s complete information. Wow, what they have been through just to bring four children into the world. That, even before Battens came into play. The families in the UK that have children with CLN2 Batten Disease are dealing with so much right now. The threat of having treatment for their children removed must be devastating. To have that hanging over them is a huge challenge in addition to the one they already face. My thinking when I approached Lucy was that they, as a family, are now so well known. Perhaps it would be of no use to her and Mike to work on a blog with me. I was, however, pleasantly surprised at her response and I was extremely happy with the way that it turned out. It was a lot of work but well worth it.
I first started this blog in order to share the experience of being a parent to a special needs person. It also was for the purpose of sharing some of the things that I had experienced as a result of reading a particular book named FEARLESS. I have shared in the past that it was because reading this book about a Navy SEAL that I eventually became aware of Batten Disease. How is that possible? Well, you can read The Blog About My Blog, and that would fill you in. It’s right here on the home page. Just scroll down and you will find the link. I had heard the term blog before I started blogging, but at that point I didn’t really know what one was. I think that most blogs have daily or weekly entries but this one is different. Most blogs have information that people may find useful for living life or perhaps they exist to share thoughts or opinions. This one is different. The original content shared my experiences about my following the legacy of a Navy SEAL. It also talked about how I traveled to see his hometown and also to meet the little girl who first made me aware. There was a time when I was wondering what or who I would write about next. It would be a bore to most people if I was to write about myself. The question of what to write about next didn’t last long. Through a series of events, I started reaching out to families who had children with Batten Disease. Since that time, I have never been without a family to blog about. This blog is different because it tells the stories of families that go through the Batten journey. It does so from an outsider’s perspective with the help of the families themselves.
You may ask why am I doing this. What motivates me? This blog has become my life’s work outside of my normal job. I put an awful lot of time into it. If I am not writing, then I am doing something else in preparation to do so. It has been an amazing experience for me. The stories started out being somewhat simple. I have learned a lot along the way about gathering information and how to process large amounts of information. I have brought my son Benjamin into the discussion several times and have talked about the special bond that exists between us. I have talked about what it would be like if we no longer had him. A large void would appear in our lives if he were to be missing all of a sudden. We have no idea when that will take place or if it will happen in our lifetime. For the Batten family, things are different. For them, there is, in a big sense, a course of travel that is unavoidable. The actions that I see on the part of the parents are to me unparalleled in terms of their devotion to their child. Undying love is still a phrase that fits so well with the Batten community.
A young and single mother uproots herself. She moves away from her family in order to help her daughter receive a form of treatment not available in her area. This, knowing that it will only extend her daughter’s life in the hopes that a cure can be found. A cure for a terminal disease that she, up until recently, didn’t know existed. She is helping her little girl to battle the disease and the results are wonderful. During a chat about the whole experience, she tells me that anyone would do what she has done. You can’t tell me something like that and expect me to believe it. This same mom told me that she “loves her daughter so much that it hurts” (I am not done using this statement). That is the kind of thing that causes my emotions to get stirred up. I think about these things in the most inconvenient places. At work, while I am with my co-workers, driving down the highway, or while I am getting ready to pull up to full service gas pumps. As a parent to a special needs person, I can tell you that the level of devotion, and often, the sacrifices that I see in these families are well worth my time in writing. In fact, I need to do this!
Batten parents will sometimes maintain their own blogs concerning the things that they and their child are going through. I highly recommend that you patronize their blogs if you want first hand information. I hope that you will read both mine and theirs though. The things that I write are a combination of information given to me by the parents themselves and my own observations and feelings. There are some things that I can relate to directly and there are other things for which I can only try to show maximum empathy. The empathy that I have is not something that is forced but rather, something that I desire to possess. Let me tell you something. I do possess it. I am stating the truth when I tell you that I love these kids. I hope that you don’t mind. The same is true when it comes to their families. It is just the way I roll. I have my base of supporters of course. I have had several people express to me their appreciation for helping to spread awareness about Batten Disease. I so appreciate everyone’s kind words. I do like the fact that I have carved my own niche for spreading awareness but that was never my intent. My main reason for doing this has simply been to honor people within the Batten community because I have been so touched by their stories.
I get a variety of responses when I tell people about the blog. I think that most people are not able to place themselves in my shoes. This is so different than what the average person commits their freetime to. Sometimes, I will be talking to someone, explaining what I write about, and they will have this reflective look on their face. It is like they are trying to process what they have just been told. It is a little different but my heart is really in this. I look at life a lot differently than I used to. My life isn’t so much about self indulgence anymore. Don’t get me wrong, I can certainly be that way at times. It is true, however, that my short and long term goals are different. I now have an additional purpose or calling, if you will, in my life. I have had it suggested a couple of times that I should take up writing about wounded warriors. That would not be out of the realm of possibilities for me. I could contact the Veterans Administration or a veterans group and volunteer my time in working with warriors of a bigger variety. You know what though? I wouldn’t want to quit doing what I am doing currently. I would only want to add to it.
I have a really good friend who a lot of people know as Christy Gerrell Bac. She is the mom to a beautiful young girl named Amy. Amy is battling CLN8, Batten Disease. Christy’s husband is a different kind of warrior. Retired from the Air Force, it was discovered that Chad had cancer in his colon. Chad is battling hard and many people are praying for him in this most difficult battle. Can you imagine what it is like for Christy? How does she handle having two thirds of her small family sick like this? I don’t have a complete answer but she tells me how she handles it, in part. Simply put, she pours herself into writing and she writes extremely well. I cannot believe the level of talent that she possesses although I know that people like her exist in other places. Christy really deserves to have her books go through a publisher. Her ability to create characters and to carry them through in length just amazes me. In fact, I was perhaps a little bit jealous of her God-given talent. Then I thought to myself, It’s not that I don’t have an ability to write. It’s just that I am more of a journalist than I am a writer. Christy is one type of writer and I am another. If I had her level of talent, I swear that I would no longer be turning wrenches. What I do though, does have its place. My place now, and until there is a cure, is with these families for as long as they will allow me. By the way, Christy is self published on Amazon. You have to read her books on a Kindle, which I happen to have. I have been reading one book of hers after another. Just keep in mind that they are not for kids.
As I have already stated, I have a passion for telling these stories. I do so while pouring as much feeling into them as I can while also maintaining accuracy. I don’t know this for a fact, but it seems like the number of people that look at the blog on a regular bases is declining. This is not a big factor in my motivation to continue. I can see that people are reading the stories as they appear in the blog and that is good enough. It is nice though when I see that a lot of people are reading the various stories in between the times that new ones come out. That means greater awareness. I do see evidence of that at times! I can imagine that my detractors will think that it is useless to continue to read these stories. They may be thinking that, well here we go again. Just another story about a kid with Batten Disease. It is my belief that each one of these stories is unique and each one of these families stories are worth reading about. Whether we are talking about a well known family that has a lot of support in their local community or a single mom that is struggling to make ends meet. They all have to deal with this dreadful disease. The pure devotion and undying love exists in both sets of circumstances. Batten disease has no prejudice although certain variants may be more prevalent in certain places. Different stories, different circumstances, but the same pure devotion with varying but touching details. Let me also add this. You can put into the place of these families those who are dealing with different forms of pediatric cancers and other rare and fatal diseases. Pure undying love and devotion exists in many other cases.
So I have received an overall positive response from the Batten community when it comes to these stories. I also know that there will be those that are skeptical of what I am doing. I suppose it is true that, for whatever reason, the blog is not for everyone. Like I said earlier, some parents have their own blog. That’s wonderful. Others, just want to share their journeys on their own. Some just want to keep everything in private. You are certainly within your right to not participate. Please know though that everyone has a story to tell. Everyone that is dealing with Battens or has dealt with it had plans for their family. Those plans have been interrupted and their lives have been changed. If you don’t mind, I would like to tell your story. The heartache that is mixed with tender details. I have had the privilege of blogging about a lot of families now. This is so appreciated. There are some that I have talked to and some that I would like to approach. Some have said no thanks and that is certainly okay. I have not gotten back to a couple of families that I have previously talked to. I am so sorry about that. PLEASE! If you would like to be included, let me know. Don’t be afraid to ask. Also, Just because I send you a friend’s request it doesn’t mean that I am doing so just to get a future story. I follow the journeys of these children just to be a support to others. If an opportunity to write about that journey becomes available, then great! Again, if I ask and you are not interested, just say no thank you. Honesty is always the best policy. There are always those who say yes. If you change your mind after doing so, just tell me and there will be no hard feelings. The journey is a difficult one and all of you come first. Do you have some junk in your trunk that you don't want shared? Well, don’t we all? I just want you to know that I always keep things positive. I always say that “Life Happens” and the stories revolve around these little warriors. Plain and simple. I realize that it is sometimes difficult to share an experience that is so disheartening. I completely understand. Your kids and their stories are so sweet though. Please give it some thought.
Okay, so here we go, gulp! The next subject is putting ads that would generate a profit into the blog. Let me start by emphasizing something. This blog has never been about making money and it has never been my plan to do so. We are talking about the lives of innocent children and young adults and I never wanted to use that to increase my income. I have never contemplated putting ads into the blog just because of the way that things might appear to people if I did that. The reason I bring it up is because I would like to know what people in the Batten community think about this. It does cost money to maintain this blog but it is a cost that I gladly assume because I love what I do. The reason why I think about the ads is because of what could be done with the money. The people that know me best, know that I love to give to families that are in need. Lets face it. Dealing with this disease is very expensive. The thing is that I am the sole breadwinner at this house and so my ability to give is not that great. I don’t know how much money would be generated by the blog. However, if it was to become self-sustaining and I was able to give to research and those in need, well that would be a good thing. Wouldn’t it? I don’t know. Maybe that would complicate things and my fear has always been that it might drive people away. I would try to make the ads suitable to the types of items used in the Batten community. Your opinion would be greatly valued. It’s just a thought and I could go either way.
So where do I go from here? I will write as many stories as people will allow me to. In the beginning they were pretty simple. The stories have grown in length and have become more detailed. There is more commentary from me and more information about your sweet warriors. I hope that more people will say yes as I continue to reach out to the community. They are your stories and you have a say as to the content. The writing style is mine but I will work with you. I will repeat the fact that being Benjamin’s Daddy has lended itself to my sensitivity towards your children. My challenges pale in comparison to those experienced by all of you but I try to understand as best I can. It wasn’t that long ago that I loaded my little family on an airplane and traveled over 2,000 miles so that we, in large part, could meet the Batten princess that made us aware. It might sound hoaky to some, but my life was changed by that event. I have heard stories about the Batten conferences that take place each year and I have seen pictures taken at the event. I have even written about it in spots and I think about how incredible it would be to see it in person. I have always been a little jealous of the fact that I couldn’t be in attendance when they are going on. To be there and to meet so many of these little warriors in one place. Wouldn’t it be awesome if I could go? Well it would be for me and you just never know. I may show up sometime. Until then I will continue to write for as long as I am able. Thanks for following along. There is more to come!
Blogger and Advocate
“The date the 13th of February 2015. A day that will stay with us forever. I can remember the color of the walls, even the smell. As we sat in that small room with Ollie on his daddy’s knee we were told that our child has Battens Disease, that there is no cure and that life expectancy is between six and twelve years. Ollie was just four years old! Our world fell apart in that moment. We were told Battens Disease was so rare that they had never come across a child with it before.”
Part of what makes these blogs is selecting the photos that I feel work well with the story. I think they help make the articles what they are. Choosing the best ones makes a difference. Don’t you agree? I’ll be looking through people’s photo albums (with their permission of course) and I will spot just the right one to use at the beginning of the blog. I’ll see it and say to myself, “That is the one. Right there!” At that point, the picture becomes associated with the title of the blog. The photo that you see here, at the top of this blog, is the one. It is amazing and it has been my favorite for some time now. There have been many a photo taken of Ollie and Amelia, but to me there is not another one like this one. It is a priceless work of art. Lucy has a way of working the camera. She knows how to capture the charm of her two little warriors so that we can all enjoy them from far away. This one paints a picture. It’s a picture that needs no words to tell a story. It portrays a close bond between a sister and her brother but they are not just any pair of siblings. They are on a journey together and I just bet that the bond between them is incredibly strong. With everything that they face, they do so together. This picture gives the appearance of Ollie needing comfort. It also appears that Amelia is providing it. This picture is simply beautiful and it goes well with a beautiful story.
I noticed a comment from one of the Batten parents when I first started writing these blogs. It was simple and it stated the following. “He’s writing about us.” I think that my feelings at the time were, “Why yes, I am. And for good reason.” It has been well over a year since I started and my feelings haven’t changed. Every day I go home to my sweet wife and my special needs son, Benjamin. The bond between us is strong and it is to me, an incredible thing. I have made this point over and over but I will do it again. My sensitivity towards my son has lended itself to the fact that I have a sensitivity to kids with rare diseases, such as Battens. There is always something really sweet about a child’s dependency on a parent or caregiver. The feelings that surround these stories are an addiction of mine. One that I have no intentions of giving up. What is it like to be told that your child has a disease that is terminal? That is a question that I have thought about often. Thinking about what these families face often brings me to tears.
This is a story about a young couple that fell in love and wanted a family together. Their dreams would come true but the fulfillment of them would not come without some big challenges. Mike and Lucy met and were inseparable from the time that they did. They made plans and worked at achieving goals together. This included achieving career goals and having the family that they dreamed of. Lucy gave birth to three sons and they were so excited. Mike and Lucy had no idea that a prince was in their midst but there was something really special with this little man they named Ollie. The bond between him and his family was instantaneous and special. Gosh, he was cute. A future heart throb. While things seemed like they couldn’t get any better, they did. The entire family was elated with the news that a princess would soon make her appearance into the household. Although she gave everyone quite a scare, she arrived into their midst and she was so completely perfect. From the time that Amelia arrived, all were instantly in love with her. This was for good reason and one that could clearly be seen. From the moment she began to smile, her smile became infectious.
They thrived as a family and life was so good. The first eighteen months had passed since Mike and Lucy’s family was completed and the future looked so bright. Mike was working hard and was in the process of building his business. The same tenacity and work ethic that had made him great on the football field also made him great at work and at being his family’s provider. Lucy had finished her education and she was poised to one day return to work. For now, the house was so busy with four children happily involved in all their activities. The king of which was football. The sounds of footballs being kicked were sometimes followed by things breaking as the sport was practiced inside as well as outdoors. Somehow, mum and dad didn’t always mind as much as one might expect. After all, the kids were doing something that was loved by all. You couldn’t walk very far without tripping over a toy or two. Kids have to have toys you know! There was laughter. Plenty of laughter and fun. No doubt, there were outings together to places that included grand mum’s house. Accompanied with all of this were thoughts of what the future would hold for all four of their children. Sporting and school events. Summer and winter vacations together. Perhaps Ballet recitals and other sweet things for the princess in the house. Graduations and successful careers. Weddings and a sprawling family as the size increased with grandkids. The list goes on.
It is too often that things don’t go as planned or as one might think they should. Troubles come our way. Things we would never expect to happen do happen and that sometimes brings devastation.That is the way it is with Batten Disease. Life already has it’s share of difficulties and we deal with them as they come to us. A rare disease such as Battens brings with it concerns that are on an entirely different level. Mike and Lucy had no idea how different of a course their lives as parents would take. Almost all at once they found themselves being part of a community, worldwide, that they didn’t even know existed. This part of their story will not only explain what would happen but also, how they would handle the unforeseen. Things would change forever as problems began to develop with their prince they named Ollie.
Lucy stated that it was around the age of three that they noticed some things in Ollie that caused concern. One thing was that he had some delay in the development of his speech. This had occurred in Ollie’s older brother Micky as well, however, Micky had caught up with the other kids when he started school. When they mentioned Ollie’s delayed speech to health professionals they felt that he would catch up just like his older brother did. While there, they also mentioned the fact that Ollie was a bit clumsy but as you might guess, the doctors felt that Ollie was just being a boy. Ollie was attending preschool at the time and he just loved it. He was hitting all of his milestones, with the exception of the delayed speech and potty training had been no problem. Mike and Lucy must have been relieved by the fact that the doctors felt everything was just fine. That was until the month of September 2014. Ollie had his first seizure.
The day had been very normal and Ollie had attended his preschool. He had done well that day and he was happy when he was picked up. As they had returned home, Lucy had noticed that Ollie felt warm and he had a temperature when checked. Her concern for him was great enough that she brought him to get checked out by medical professionals. It was found that Ollie had an ear infection and so antibiotics were prescribed as the family was sent on their way. Ollie returned to running around with his brothers after receiving a dose of the medication and all seemed to be fine again. It was later that night, while bathing, that trouble would strike. It was then that Ollie began to have a seizure. Lucy stated that it was the most terrifying thing that they had ever witnessed. Ollie actually slid under the water while seizing. Fortunately, Lucy was close by and screamed for Mike to come quickly as she pulled Ollie out of the water. Mike flew into the room as Ollie continued to seize. Lucy said that it was as if time had stood still while all of this was happening and who could argue with that. Mike continued to care for Ollie as Lucy called an ambulance. The seizing stopped in a few moments as they waited, but as it did, Ollie stopped breathing. What must have been part of Mike’s training as a former lifeguard kicked in and he started to perform CPR on his very own son. That is amazing calm in the midst of chaos. Lucy said that flashbacks from that day still haunt them.
She went on to describe to me what she had seen happen with Danny and Micky as all of this was happening, “I can remember looking up from Ollie, who was laying on the floor, into the eyes of Danny and Micky. They stood in the doorway watching and pure fear filled their young eyes. But even so, they knew what to do as they picked up Amelia who was still a baby. They flew out of the door to our friends next door.” By Mike’s quick actions, Ollie began to breath again. Of course, Ollie would get checked out by doctors who came to the conclusion that he had experienced what is called a febrile convulsion or seizure. This is a type of seizure that is associated with an elevated temperature. Mike and Lucy were assured that these are common in children and that 9 out of 10 times, they are never repeated. Ollie was discharged from the hospital the next day, much to the families relief. Their little Olliebobs was okay! It was, however, only hours after returning home that Ollie would have another seizure. According to Lucy, they were told by the hospital that it was a convulsion related to Ollie’s infection. That, though, was not to be the case. These seizures were not isolated but rather would become part of a larger problem. Over the next few weeks, Ollie began to have more and more seizures.
It is only fair in considering that Batten Disease is rare, that it may not be diagnosed quickly in all cases. It should, however, be known of in every place that medical science has it’s reach. Mike and Lucy were devastated with the diagnosis of Generalized Epilepsy. Almost all at once, the plans that they had made were interrupted. At least as they applied to Ollie. Questions arose. Would he be able to drive a car or even function normally as he got older. They were at this point, optimistic overall and felt that Ollie’s condition could be handled through medication. It would control the seizures and he could lead a normal life. It should come as no surprise that Ollie was referred to a neurologist because of the seizures. Lots of questions were asked about Ollie’s development and it was at that point that Mike and Lucy brought up Ollie’s issues with his speech and his clumsiness. Obviously this doctor knew the questions that needed to be asked. As a result of the information that she gathered from Mike and Lucy, it was decided that more testing needed to be performed. A lot of testing was performed. This included EEGs, CT scans, and MRI scans. Lucy said that it was as a result of the MRI performed that they learned Ollie’s cerebellum was smaller than it should be for a child his age. Oh man! This is commonly seen in children that suffer from Batten Disease. I don’t even think they knew the disease existed at this point. Lucy stated that it is this part of the brain that controls areas of development such as balance. This was the cause of Ollie’s frequent falls. It was at this point that it really started to appear that there was something going on besides regular Epilepsy. Mike and Lucy were really starting to worry, and for good reason.
The doctors wanted more testing performed. Lucy would tell me that Ollie was tested for a group of conditions through blood testing. Mike and Lucy did a Google search with each one that they learned about, checking Ollie’s symptoms against what they learned. They crossed each one off as Ollie’s symptoms didn’t match. Within a few weeks, they received a call asking them to bring Ollie in for a consultation a couple of days from then.The appointment was made for February the 13th of 2015. Lucy said that she felt relieved at that time, thinking that it couldn’t be bad news coming. This, she felt because they wanted them to bring Ollie in with them. It was, however, as soon as they arrived that they felt something to be very wrong. This because the appointments room was completely empty. This gave them a sinking feeling as that part of the hospital is never empty! It was now though, with the exception of the staff. Lucy said that they had never been to this particular part of the hospital before and she had made note of the appearance of it. It was very white and clean looking. She felt that the area had a funny feeling about it. Like a feeling of sadness. As they entered the doctors room, they were greeted by a number of health professionals. Within minutes of entering they were given the news as Ollie sat upon Mike’s knee. Ollie had CLN2 Batten Disease. They were informed that there is no cure and no treatment. Every time that I have done one of these stories, a dramatic picture is painted by the words given to me by the parents. This, because it is so very difficult for anyone to receive this kind of news. It doesn’t matter how strong of a person you are because no news could be more difficult than this to receive. Lucy’s own words paint the scene perfectly, “Our little boy was dying! I remember looking across at Ollie in disbelief, watching him looking up at his daddy. Smiling, wiping his father's tears away. We asked the doctors where else there was treatment. There had to be something, somewhere in the world. We were told there was none.”
Mike and Lucy were given information about Batten Disease. Then, they were told to take their son home and and enjoy him for as long as they could. They were sent on their way, that is all that there was to it. Lucy stated that the next few days were a mix of pain, sadness, disbelief, and anger. They kept reading the symptoms and felt that Ollie’s symptoms did not match up with them. They began to research the disease, thinking that they couldn’t give up on their son. Mike and Lucy felt that they had been let down by the doctors. As they worked through their grief, they pressed on in search for help with Ollie. Through their search, they found BDFA (Batten Disease Family Association) and reached out to them. They also reached out to families in the United States that were already dealing with the disease. The first person that Lucy had made contact with had a beautiful daughter that also had CLN2 Battens. Her remarks about that initial conversation are worth noting, “That conversation will never leave me. Through this father’s own pain he somehow found the strength to support us. This is something we will forever be grateful for.” This is one of the things that I have observed to be so wonderful concerning the Batten community. That would be the amount of support that these parents give to one another. It is truly something special. Only they can know what they as Batten parents are feeling.
It was a few weeks after Ollie’s diagnosis that Lucy started a Facebook page for Ollie. She had started the page initially to keep family and friends informed about Ollie and his struggle. As a family, Mike and Lucy had no idea, at the time, what kind of impact they would have on the Batten community there in the UK and elsewhere through Ollie’s Army. For that matter, they had no idea how their own lives, and the journey they would take as a family, would be impacted by the other families they would meet. What started as only a way to keep family informed would eventually become so much more. The awareness that would be raised and the people that they would meet is very worth mentioning. The help that they would seek for their own children would also be a help to others with Batten Disease. Ollie’s own need for help would impact other people’s lives. This would all happen as Ollie’s journey continued.
Eventually, through their own search, Mike and Lucy found a specialist in London who they thought could help. They made an appointment to get in as early as possible. Lucy said that the doctor could not believe her eyes after seeing Ollie for the first time. This because, at that time, she had never met a child with Batten Disease that was as well as Ollie was. She was used to seeing children that are farther along in the disease by the time they are diagnosed. The disease is so rare. While there, they would learn that Batten Disease was genetic and, therefore, their other three children had a 50/50 chance of also having it. Mike and Lucy decided to have all three of Ollie’s siblings tested as early as possible. Already dealing with heartache of what had befallen the couple’s darling Ollie, they would be given disheartening news. They were relieved to know that neither Danny nor Micky had the disease. It was, however, revealed that little Amelia also had CLN2 Battens and would one day start showing symptoms. Their princess! The one that they had been overjoyed to receive. She had made her own dramatic entrance into the world and she was just getting started with a new challenge for this family. I had found this quote from Lucy that I used in the original blog. She described what it was like immediately after receiving the news from the medical professionals, “For the second time our world fell apart as we learnt our beautiful princess also had Batten Disease. Amelia had only just turned two, she had been hitting all her milestones, to look at her you would never know. Walking out to our boys in the waiting room, trying to hold it together and act normal has to be one of the hardest things we have ever had to do.”
That was on the 30th of March, weeks after Ollie’s diagnoses. They received the news that their princess also had Batten Disease. Lucy further described the mood within the house as they returned home to digest what they had just heard. “For days all we did is cry. I could not stop being sick due to shock whereas Mike locked himself away from everyone.” This went on for a few days and then Lucy said that they pulled themselves together. I have read similar things from other parents. The shock and devastation eventually give way to determination. This, even though the heartache remains constant. In her own words, “.....there was no way we were going to let this horrific disease take our children without a fight.” So this all raises a couple of questions. How do you handle adversity such as this? What do you do when two of your children, or even just one, is diagnosed with a horrific disease that you didn’t even know existed. Everyone handles things differently. I would think that most every parent is determined to provide the best possible care and also to fight off this monster of a disease as best they can. Some do so quietly as a family and that is perfectly fine. Others share their child’s journey on social media. They want moral and other kinds of support from outside They want people to know who their child is and what the journey is like. They don’t want their kids to be forgotten! Still, others know the importance of spreading awareness about this disease. Much progress has been made but this is no time to quit. The difference between knowing and not knowing is profound. Especially as it relates to the medical community. People in public office need to be aware, as well as, the general public. And then, there are others that take up a leadership role within the community. They do things, not only for their own child’s benefit but also for the benefit of others. This may come from taking advantage of opportunities that present themselves. It can also come from intentionally seeking to take an active role. I believe that almost all of the above applies for Mike and Lucy. They have become very public in their battle against Batten Disease and it has benefited others as well as their own family.
It all began as they searched for help with Ollie and Amelia. Lucy said that they had found a clinical trial that involves Gene Replacement Therapy. Both Ollie and Amelia were accepted, but just as they were, Mike and Lucy learned of another trial that was being done. This one was taking place at the Great Ormand Street Hospital (GOSH) in London. The couple researched the trial and found out that it involves replacing the enzyme that was missing in Ollie and Amelia’s brain. The program was creating incredible results. The only problem was that the trial was closed. Ollie and Amelia met all the criteria for acceptance but the trial was simply closed. Pleading with the doctors was of no use as they were powerless to help. How absolutely frustrating. Grieving over the recent news that two of their children were terminal. Knowing that there was something that could extend their little lives with the hope that a cure would be found. And yet, entrance into the trial was closed. That is when Mike and Lucy reached out to the Batten Disease Family Association (BDFA). With the help of BDFA, they worked towards convincing the pharmaceutical company (BioMarine USA) to grant Ollie and Amelia access to the trial on the basis of compassionate use. Lucy said that it took months and months of midnight calls to America, emails back and forth, bringing health professionals on board. Finally, with a lot of determined effort they were able to organize a meeting with the company and it would be held in Paris. They traveled there, along with representatives from BDFA, to meet with company representatives and others from around the world. The result of this meeting is that the company opened up five places at each trial centre across the planet. That, my friends, is significant and a product of great leadership. It also comes from parents that want the best for their children and will stop at nothing to get results. Lucy put into words their feelings of gratitude towards those that made this all possible, “We cried upon receiving this news. The BDFA and health professionals worked tirelessly to enable this to happen and we will never be able to thank them enough for taking on our pleas for help and support for our two children.”
Even with this decision, the company still had to be convinced that Ollie and Amelia met all of their criteria for the trial. Finally, after many assessments and sleepless nights, both of their darling children were excepted and the process was begun. Lucy stated that her and Mike were so thankful that both of their children were given a lifeline of hope. They also felt uplifted, knowing that their efforts had helped in giving this opportunity to other children across the globe. Ollie had brain surgery in November of 2016 to have a port inserted into his brain. This, so that he could receive the chemical that replaces the missing enzyme. Amelia would join her big brother at GOSH in February of 2017. The infusions take four hours and the family travels from Manchester to London every two weeks. This, obviously, takes a strong commitment as they juggle everything that life throws their way. The results are so worth it though. Lucy stated that they would have traveled to the other side of the world for their kids to receive this treatment. Both Ollie and Amelia are responding well to it. Sadly, Ollie had progressed further into the disease as they worked on getting their children accepted. He could no longer walk unaided by the time the he started the therapy and he was only able to say a few single words. There is no doubt that the enzyme replacement has had a major impact on increasing Ollie’s longevity and general health. The treatment has stabilized the disease in him. He no longer experiences the pain that he was having and his seizures are well controlled. He has only had one seizure in the last 16 months compared to the hundreds he experienced before he began the therapy. Ollie goes to school and enjoys swimming with his daddy. He is a doll of a young man and a genuine prince!
It needs to be noted that what you see with Ollie and Amelia is a contrast between early diagnosis and treatment, and having to wait. Amelia is thriving on the treatment because her Battens was discovered so early and the therapy was started right away. She, in fact, is one of the youngest children to be receiving the the treatment. She started the treatment so early that the only sign of the disease was a single seizure and that was attributed to sickness. Lucy describes the exceptional quality of Amelia’s life. This even though she has Battens, “As far as we are aware Amelia is the only child in the world who should have symptoms of Batten Disease but doesn't. Instead she is thriving, she goes to mainstream school, where she plays and interacts with her friends, she enjoys mark making and phonic lessons. Amelia can learn new information and retain it. She can run, dance, Juno and sing. These are all abilities which she should have lost a long time ago.” Lucy goes on to say that Amelia is learning to count, write her name, and ride a bike. Just like any other child. Mike and Lucy were extremely happy in what they were seeing in both of their Batten children. The enzyme replacement therapy had produced positive results in both of them and so they were excited by the news that the drug being used was approved by both the FDA and the EMA. In Lucy’s own words, they were “over the moon.” Once this happened, however, a new fight would loom on the horizon.
Mike and Lucy would remained involved and Ollie’s Army would continue to become more than a name. As mentioned before, the Facebook page named after Ollie was originally set up to keep family and friends informed but it quickly became much more. Major awareness was created through Ollie’s Army and some of that came with the help of some well known celebrities. I bet that, like the rest of us, they were taken by the appearance of these two attractive children and their family. Fundraising events have been held in the local community and the results are absolutely amazing to me. While a lot of the funds raised have gone towards Ollie and Amelia’s needs, a lot has been done for the fight against Battens in general. A big example happened recently. Ollie’s Army was able to raise and donate £30,000 to help in the research of therapy for the eyesight of children with CLN2 Batten Disease. In case you are curious, I did the conversion and that amount is over forty-two thousand in US dollars. This family is aware of the fact that this research comes too late for Ollie. However, it could help Amelia. They have also done this in the hopes that it will help others in the future. That is so commendable! Fundraising for their children has helped fund equipment and adaptive changes that were made to their home. Some of the funds have helped with the expenses related to the treatment and also help with making precious memories. They have also used funds to help support BDFA in their efforts as well.
There are so many very kind people that have helped in their effort through donations and the giving of their time. One example is the help that they received with the building of an extension to there home for Ollie. Mike was able to receive the help of others in the construction and it became a real community project. This included the installation of a lift and a hoist for Ollie. Lucy describe the feelings of gratitude that she had at the time, “It was an extremely emotional time adding all this equipment to our home that we needed but did not want to accept. This was made easier by the people around us supporting us every step of the way. We even had the children from our street coming to help it was so heartwarming.”
Ollie’s Army holds a Golf Day and Ball each year and it takes an incredible amount of help from others. Their are some really incredible ladies that do this even while working full-time jobs. There is also a Family Fun Day held each year. The event which, includes a football tournament, hosted over a thousand people this last year. This included a few famous faces. Lucy said that all of this would not be possible without the help of their fantastic friends who give of their time. A network of amazing people has entered the lives of the Carroll family. This all, no doubt, makes something that would be almost unbearable, a lot better. Lucy would also tell me that people that were complete strangers to them have reached out to their family, wanting to help with Ollie’s Army. She said that these people have no idea how much their kindness means to Mike, Lucy, and their kids. All of this has helped them get through some very difficult times. The beautiful thing is that as this family is helped, they help others in the process through their own actions. Lucy summarizes the entire experience in her own words, “Ollie's Army was created to raise awareness of this horrific, rare disease. We have met so many incredible people throughout our journey. It's made us appreciate life and helped us to understand that there are so many people having to battle battles everyday.”
There are some notable people that this family has met along the way. Now, I have referred to Mike, Lucy, and their kids as my royal family and they will always be that. (They are every bit as attractive of a couple as those that have belonged to the British Monarchy). As a family, they have been in the presence of royalty. That would be so by being in the presence of Prince Harry. I know that for them, he turned out to be a really neat guy. According to Lucy, Prince Harry first met Ollie at something called the Wellchild awards. You see, Ollie had been nominated for an award as being the most inspirational child. Well, go figure. He won! What happened there was so sweet. Lucy described the seen, “At the award ceremony Ollie met Prince Harry. As Prince Harry was knelt down speaking to Ollie, Ollie who could no longer walk or stand unaided used all of his strength to stand and hug Prince Harry. It was a magical moment which hit the headlines across the world.” That is amazing Ollie!
It would be six months later that Prince Harry would stop in at GOSH to see Ollie and Amelia. What a wonderful thing to do. I think that I am a fan of Prince Harry now. From an ocean and a continent away!
Ollie’s parents had been fans of Ed Sheeran, the musical entertainer, for some time. They passed on their love for his singing ability to Ollie. Ollie loves Ed’s music and he would sing along to his songs. That is until he lost his ability to do so. I know that Ollie’s face still beams with a huge smile when he hears Ed’s music. Ed is not only a gifted musician and singer, but also a great entertainer and his music has seen Ollie through some very difficult times. Ollie’s love for Ed’s music was revealed to Ed through a social media campaign set up by Lucy’s sister Emily. This got Ed’s attention and the entire family was invited backstage to meet Ed while he was performing a concert in Manchester. I can only imagine what a treat that had to have been for the entire family but especially for Ollie. I bet that Ed had really endeared himself to Mike and Lucy for having done this for them. I am also thinking that meeting this family was really meaningful to Ed and I bet he was inspired to be even more of a humanitarian. What a truly neat experience for all involved.
Mike and Lucy recently had the opportunity to appear on the largest morning show in the UK, which is called This Morning. While there for the appearance, they had the rare opportunity to meet the Prime Minister of the United kingdom. Theresa May was also appearing on the show that day and it gave the couple the opportunity to speak with her. They shared with her about the disease that confronted their children and the treatment that they were receiving. Mike and Lucy really appreciated the time that they were given with the Prime Minister. Oh, and why was the couple invited onto the show? That was to discuss the impact of the recent decision by NICE (The National Institute for Health and Care Excellence) to not recommend funding by the National Health Service (NHS) for the treatment that Ollie and Amelia are receiving. This doesn’t only affect them, but also the others that are receiving the treatment. This is the battle I referred to that was looming and it also affects children who are waiting to start the therapy but can’t because of the decision.
Mike and Lucy’s days are busy. There are all of the normal responsibilities that come along with having a family. Mike’s work as a builder and Lucy’s work at home. There are two big brothers in Danny and Micky that are involved in all the normal activities. This includes school and football of course! They have to be the best big brothers because they have two younger siblings that require a lot of attention. There is also a princess in the house and a princess always requires a lot of attention. Especially when yours is a Batten Princess that is undergoing Enzyme Replacement Therapy. Her little self goes to school and does all the “normal” things because the therapy is working! They also have a very handsome young prince at their house. There is a very special bond between him and his family and their world revolves around him. That is because it has to. He is a Batten Warrior and he needs help as he fights. He is a royal rockstar! There are appointments to keep and that is not to mention a trip to London every two weeks. There is always messages and emails to answer. Planning of events and daily entries in Ollie’s Facebook page. Awareness, awareness, awareness! The list goes on and on. Oh, and maybe there is a certain blogger that needs more info to include in his next story. Even with all of this going on, they remain in the lead alongside BDFA in trying to make sure that that Enzyme Replacement Therapy remains funded.
“And now let me tell you about an entire family that has become royalty to me. There have been several famous couples that have been showcased by the Royal Family over the years. In my humble opinion there are none that have anything appearance wise on the couple that is Mike Carroll and Lucy Faith. They are both very attractive people. When an attractive couple decides to have a family that usually means that their children will be attractive as well. It is easy to see that is in fact the case. Ollie and Amelia and their other two boys are all very nice looking indeed. Adorable is more the word that I think of when looking at all the pics. Prince Ollie and Princess Amelia are among the many children that have become the face of Batten Disease.” This was written by me in my first blog about this family, My Kind of Royalty. These words all still stand true. I know so much more about Ollie and Amelia’s journeys, and Batten Disease for that matter, than when I first started. The more that I know and observe about this family, the more impressed I am. They have remained accessible to me even with everything that they have going on. There is another thought from the original blog that I would like to convey to you.
This is what I had said back then, “I can only imagine that Mike and Lucy would prefer that this guy from Oregon USA didn't even know who they are. Like any other couple, they just wanted to be a regular family. Raising their children in the safety of their home. Sharing in the experience of seeing each child grow and develop into adulthood. They just looked forward to seeing each one achieve success individually as they grow together in the bond of family and tradition.” Isn’t that what everybody wants in a family? Things do not always go the way we want though. What do you do when problems arise? How do you respond? I believe that this couple, this family, is a shining example of how to handle adversity. I know that they would give anything to have all four of their children healthy and whole. Mike and Lucy would probably prefer that they didn’t know what Batten Disease is on such a personal level. However, in the face of adversity, they are giving this their all. Aiding in awareness, funding, and research as they battle Batten Disease. Maximum effort with real class! There are so many others in the Batten community that I also have a profound amount of respect for. Yes, it is a tragedy anytime that someone is told that their child has a disease for which there is no cure. However, the details of the undying love and tender care that take place are without equal in beauty. This family will forever be royal to me because of all they have done in the battle against Battens. It is also true that within their home resides a prince and a princess. What once was my conclusion by appearances, is now very evident through fact. Forever Royal.
Blogger and Advocate
Of course Ollie's Facebook page is found easily by typing Ollie's Army into your search window on Facebook.
Is it them or is it us? Is it the feelings that they produce in us or is it that there is something truly special about them? I think that the answer has to be all of the above. It is them, but they also do something to us. Once you give a little piece of your heart to these kids and their families, they come close to taking all of it. Let it happen!
Highlighting a previous blog that might interest you:
Awfully Beautiful - The Life of Noah and Laine
When a family rises from their own tragic circumstances in order to help others...
They became a leading family in the community that they found themselve being a part of.
Take the titles listed below and go to month and year listed in the archives.
January 2017 -
*The Story Between The Lines
February 2017 -
*I Know Just Where You Are
*My Kind Of Royalty
March 2017 -
*The Caring Type
*Face to face: A Fairy-tale
April 2017 -
*No Ordinary Love
*A Rare Friendship
May 2017 -
*Tyrell and The Rare Love Story
June 2017 -
*Seth and The Healing
July 2017 -
*Little John and The Reason
*Sands and The Scottish Princess
August 2017 -
*A Rare Kind of Devotion
*The Giggle Box
September 2017 -
*Sweet Montanna and The Status
October 2017 -
*The Blog About My Blog
November 2017 -
*Lydia, I Love You!
*Kayden And The Change In Plans
December 2017 -
*Those That Are Like Them
January 2018 -
*Nora Skye - I Know Just Where You Are
*Brock and The Priceless Memories
*Jamesy Boy and The Treatment
*The Priceless Princess From Beyond
*Forever Royal - Part One
*Forever Royal - Part Two
*That Kind of Love - Conner's Story
*What I See (She is a Butterfly)
*No Longer Alone - Kristiina's Story
*Things We've Talked About - Oscar's Story
*The Reason Why - Hannah's Story
*Kayla's Perfect Princess - Breanna's Story
*Where Do I Go From Here? - An information only blog
*Warriors Unaware - Mia and Kaleb
*Her Little Story
*Someone Like Raelynn
*Forever Royal - The Final Version
*Awfully Beautiful - The Life of Noah and Laine
*A Lifetime of Love - Tegen's Destiny
*Forever Beautiful - Gabi's Story
*The Miracle Baby - Amelia's Story
*Dylan's Life - A Small Town Story
*Princess Grace and the Challenges
Archives - Use title listed above with the applicable month and year.
Highlights from the beginning of gregster60.com -
Benjamin's Daddy - March and June of 2016 - Revised May 2017. Titled Benjamin's Daddy Revised.
My Fearless Adventure - April and May of 2016
A Different Type of Warrior - January 2017
Larry - February 2017