“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.
“To this day I remember the nurse placing this warm body onto my chest. I looked into his eyes and instantly felt an incredible bond. As quickly as this happened however our little boy who we named Ollie, our Olliebobs was taken off of me. I was not well and was rushed into theatre. A few hours later, and thankfully, I found myself holding this precious baby in my arms again. This time I had two more bundles of joy jumping on my bed desperate to meet the new edition. In that moment I didn't feel like life could get any better.”
A young mum from England.
I may lose some people here but I would ask that you bare with me. I had these thoughts and although I was reluctant to share them in the context of Batten Disease, I felt that I would eventually have to get them into print. The phrase “A Beautiful Tragedy” came to me one day. It, of course, wasn’t a Greg Lopez original. I did not know at the time if I had heard it before so I did a search on the internet. As it turns out it has been used for a name of an album by a heavy metal rock band. It was also used as a title to a movie about a young ballet dancer. Who knows where else it may have been used. On this particular day I had thought of it in the context of Battens.
There is nothing beautiful about a tragic occurrence! Tragedy leaves people feeling hopeless and in shock over what has transpired. Questions arise as to why the events have taken place and why it involved a particular individual or group. It leaves some feeling philosophical about life and some may have questions that are spiritual in nature. I was personally thinking in terms of tragic love stories, like Romeo and Juliet. Concerning love, it is probably never displayed more boldly than it is in times of tragedy. Think about it! The emotions that pour out of these events are just so powerful. Are they not? Batten Disease is so very ugly. Yes, it involves the most beautiful of persons and to me, their stories are the most beautiful. The course of the disease, however, is not so pretty. Even so, the bond that develops between the warrior and their caregiver is like none other. It produces the most beautiful details of pure love and devotion. Extraordinary devotion. As an example, my friend Christina Bouraimi of Greece and her handsome son Theodore. He has Juvenile, or CLN3, Batten Disease. Together, they have demonstrated a Face to Face kind of love and their life together is a rare kind of fairy-tale. There are, of course, many other stories that are also beautiful.
Going through such a thing can make or break a person. Adverse circumstances and events often change people and shape their character. Sometimes for the better and sometimes not. Sometimes the changes in one person can affect another. In fact, some will take the adverse circumstances that are faced and will use the experience to help other people in a similar situation. Think about people that have gone into battle and who have survived. To them, the trials of everyday life are nothing to sweat over. This because their character was formed over adversity and they have learned to not sweat the “small stuff”. Those are often the kind of people that you want next to you in life. Those of you that have been through the Batten journey or who are facing it now, tell me that this hasn’t changed you. I know that it has!
Why Batten Disease? Why keep writing about it? I was, at one time, made aware and I have been passionate about the details of these stories ever since. You may ask, well what about all the other types of journeys that families take together? I really feel that families that battle Batten Disease represent all the other families quite well. Of course, there is a connection that I feel with this community. The Batten journey, and others that are like it, can be the most tragic. This, because it involves innocent children and young adults. I hate Batten Disease because I know that it produces tragic results that reside with the families that have lost that which is the most precious to them. The details of the journey stir up my emotions. This because those details express the outpouring of love towards these young warriors in an unordinary way. It is also expresses the utter love and dependence by the young warrior upon the caregiver. The bond is absolutely like none other. It has to be the most heart wrenching experience when it is necessary to say goodbye. Only the love that was expressed through action remains along with a void that can never be filled completely. Only the sweet memories of the experience can carry a person through such a thing. That and the promise that is often made to carry on and work for a cure. These kids change people and most often it is for the good of others. Yes. It is tragic but many of the details are beautiful. I know that I am a bit naive, however this is the way this outsider views things. I hope that you will allow me this!
It is a tragic thing whenever a child is diagnosed with a fatal disease. It is always a positive though when treatment and progress towards a cure comes about. It is also a positive when you see people get involved and do things that not only benefit their own family, but also help others that are involved in the same fight. Such would be the case when it comes to the couple that is Mike Carroll and his wife Lucy Faith. Anybody that follows children with Batten Disease knows them as Ollie and Amelia’s parents. Ollie and Amelia have two older brothers, Danny and Micky. Neither have Batten Disease but both Ollie and Amelia were diagnosed with CLN2 Battens. They are two of the most adorable little warriors that you will find. Ollie’s Army is a very familiar Facebook page and there is a website with the same name.
I first learned about Ollie’s Army from my friend Sandy. It was when I had first started approaching families about blogging about them that I was told about the Carroll family. I went to the Ollie’s Army page and took a look. I can’t remember what my first thought was but it was something like, “Oh wow, what an attractive family.” My thoughts included the fact that they were from England and I immediately started drawing a comparison between Mike and Lucy and the couples from the Royal Monarchy. I felt that those people had nothing on this attractive young couple appearance wise and so I decided that I would call the blog, My Kind Of Royalty. It was a cute and simple blog, and it was one of my first concerning Batten families. It was meant to point out the things that have status with me. I love professional sports and definitely, I love music. Politicians and other dignitaries, well, I guess I like some of them. These days, I have things other than politics and the news cycle to worry about. The things that are most relevant to me these days are performing tasks at work correctly and my work as an advocate for those who have Batten Disease. I am also a veteran and a long time supporter of our military. You’ll see this in my blog, in several places. Here are some of the things that I had to say in my first blog about the Carroll family concerning the things that are important to me,
“The people that really and truly get my attention are those who battle through hardship and adversity. They can be those who have put themselves in harms way like the members of the military have. Or, they can just be someone that has endured a difficult time in order to rise above their circumstances. These days, my focus has shifted to the little warriors and their families that battle through rare childhood diseases. These kids and their families have become some of my heroes! There are a few childhood diseases that have my attention but the one that has received most of it to this point is Batten Disease.”
My writing and activities on social media are born out of these sentiments. My work has grown out of those first stories of which Ollie and Amelia’s is among the most prominent. I felt that I needed to do a larger story about this family because the first blog was simple in it’s content. There is so much more to their story than what the first one contained. One thing that I noticed while gathering information from Lucy, about her family, is that she herself is very well written. She doesn’t require the likes of me to tell her own story. It is for this, and other reasons, that I so appreciate her allowing me to place her family’s story in this blog once more. From the very beginning, Mike and Lucy have sought to be leaders in spreading awareness as they battle Batten Disease. Like the other families that have been forced to take up this battle, they find themselves in the midst of adverse circumstances. I know that there has to be many difficult days but also ones that provide so many priceless memories. After all, they are parents to both a prince and a princess.
For that reason alone, they will forever be royal to me. Their story would be so beautiful, even if Batten Disease was not part of it. Like with so many of these stories, it all begins with two people that fall in love and this story is no exception to that.
Mike Carroll and Lucy Faith met one another as teenagers. Lucy was sixteen and Mike was seventeen. They were both working at a leisure center as lifeguards in Lucy’s hometown of Poyntown. The town of Poyntown resides in Cheshire, England. Mike lived just up the road in a town named Bollington. Although so close to one another in the places they lived, it was not until this time that they would meet. Lucy thought that it might sound a little cheesy to say so, but for her it was love at first sight when it came to Mike. Isn’t that often the way it happens? In addition to Mike’s good looks, she found him to be incredibly funny. Lucy would tell me that she felt at the time that Mike wouldn’t look twice at her. You know what? I bet it didn’t take Mike more than one look to notice Lucy. The attraction was mutual and their future together would begin then and there. One of the other things that attracted her to Mike was that he had a driver’s license and she was still too young to have one of her own. This means that he was not only good looking but he was also useful! They would go on their first date with a group of their peers and from that time on they were inseparable. From then on, there would be a lot more dates and many late night conversations. There is no doubt that they discussed a future together and all that they would want in life. Things like career goals, marriage, and having a family.
You just know that with both of them being lifeguards, they had to be somewhat athletic. That would be an understatement in Mike’s case as he was a professional footballer prior to meeting Lucy. What do I mean by that? Do I mean like with pads and a helmet? No, you silly Americans. In this country we have incorrectly labeled it soccer, but in the rest of the world the sport is known as Football. Go figure! You do use your feet to kick the ball. In various places throughout the world, Major league Football is associated with clubs that produce future players through a youth system. Kids who show promise are promoted through the ranks until they reach the top. Youths are actually signed to sports contracts. This just amazes me! As an example, Mike had been playing for a club in Everton at age 11 after which he was signed to a two year contract by a club named Oldham Athletic. Mike would be hampered by injury but he continued to pursue his dream of playing at the top level of his sport. It was not only a dream but also a very possible reality as he was just that good. In fact, At one point Mike met a scout that was sent to watch him. The scout was sent directly by the manager of the Manchester United, which is part of the Premier Football League. He was just that good and it doesn’t get any better. Unfortunately, he continued to have problems with injuries. Although he would sign for another club after recovering, he would have to give up the sport as a professional. Football was Mike’s life and a long career in it was his dream, but it was not to be. He would, however, press on with life and this is about the time that Lucy would enter it. Miked continue to play. First semi-professionally and locally after meeting Lucy.
Together, Mike and Lucy would start their family and an entire life together. Lucy had been studying health and social care alongside working a part time job at the time that the two of them had met, She would later receive her degree. Mike would go on to receive training in school to become a bricklayer. Now, we are not talking about brick fences and retaining walls in your front yard, although it could include that. Construction in the UK with brick is obviously very popular in residential and commercial construction. Mike had a desire to, one day, have his own company as a contractor and bricklaying seemed like a good fit. Is that a pun? I am not sure. Anyways, as they wanted a family together, perhaps his children could be part of the business if they so desired. Mike would set up his business later, in 2014. First thing first though.
It is a perfectly good and normal thing for a young couple to want to raise a family. Mike and Lucy would be no exception to that. In fact, they would want a fairly large family as they both loved kids. One of the things that attracted Mike to Lucy was her attitude towards caring for others. At the time that they had first met, Mike had a nephew who was two years old. He observed that Lucy took great care with the little guy and that impressed him. It was obvious that she would be a fantastic mum to his children and we all now know that to be true! We often times do not think about the possible challenges that may come along when the kiddos start to bake in the oven. You know what I mean? There can be complications then and, of course, during their birth. Lucy would face her fair share of difficulties to say the least. She and Mike would face the difficulties together and the whole thing would begin four years after they first met.
It was then that Lucy found herself pregnant with their first child. She did not get off to a very good start as she found herself to be extremely sick. This would be a sign of things to come. Difficulties in her pregnancies. At the time, she was working full-time as a nurse in a baby nursery and she was working ten hours plus per day on her feet. Mike was also working full-time as a builder and they did not yet have a home of their own together. They needed to move quickly. Her sickness would die down and her pregnancy would begin to go smoothly. That is until the 34th week. Lucy started feeling very tired and the doctor signed her off from work so that she could rest. A day after finishing work, she went into premature labor. Their baby boy had decided that he couldn’t wait any longer and he needed to make his exit six weeks early. Lucy told me that as a mother to be for the first time, she was absolutely terrified. This thinking that their baby was going to die. That feeling was probably aided by the fact that the labor room was packed full of medical professionals. The outcome, however, would be challenging but overall positive!
Their little man, which they named Danny (after the song Danny Boy) was born March the 13th of 2007. He weighed 5 pounds and 12 ounces. He was kind of a little guy but that was to be expected with his early entrance into family life. They were lovingly referring to him as their “little bag of bones Danny”. Obviously he was very adorable. So perfect and complete. He had all his fingers and toes plus, he was covered in brand new baby skin. Lucy had her first child and Mike had a son! Football! Oh yes. You just know that dad was thinking about that! No one ever forgets that first child being born and for that reason, Danny will always be special! Danny would struggle to feed and would end up dropping all the way down to five pounds even. Lucy said that he was the tiniest little thing they had ever seen. The staff at the hospital had to insert a feeding tube that went from inside his nose and traveled to his tummy. In addition, they gave him light therapy. Lucy described the love that she and Mike had for little Danny, “The love we felt for him was so powerful. I spent days crying, wishing that I could make him better and take his pain away.”
Things would continue to improve though for Danny. He would get stronger with each passing day and at last, the doctors said that he could go home with mom and dad. The only problem was that because of Danny’s early arrival, they had no home to go to. The final details on the house that they were due to buy had not been finished. Mum and dad to the rescue! They brought little Danny home with them to Lucy’s mum and dad’s until they were finally able to move into their new family home. I am quite sure that they were more than welcome there. Danny would continue to face challenges with his feeding as he had severe reflux issues. He could not keep his feeds down after eating. He was, however, a very happy baby. Lucy said that, much like herself, he was a bit of a late night party animal, Danny liked to stay up all night and sleep during the day. Like any new parent that is adjusting to having a new baby, Lucy thought that life was a little crazy back then. However, the challenges that they had faced up to that point were only the beginning. There was much more that lay ahead.
Life moved forward for this family of three. Mike was working lots of hours as a builder and he had started playing football again on the weekends. They were enjoying being in their new home and Lucy was caring for little Danny. Things were good but Lucy was noticing that she was tired all the time. She felt, at the time, that being tired was just part of being a new mom and it never occurred to her that something else could be going on. It was only six months since Danny was born but Mike and Lucy were given the news that she was pregnant again. This is how the news was received by the young couple, “We were over the moon that our children would be so close in age and even more so when we found out we would be having another boy.” Another boy and a playmate for Danny. Another little football player perhaps. Lucy said that she sailed through the pregnancy. That is until the completion of the 31st week. It was at that time that she felt like something wasn’t right. Lucy went straight to the hospital and it was confirmed by doctors that she was in labor. This time it was nine weeks before the baby was due to be born! Oh my gosh! Doctors gave her two options. The first one was to continue with the labor and deliver the baby. Delivering him this early in the pregnancy would bring a risk of death or other issues for the baby. The second option was to use drugs to stop the labor. They chose to try to stop the labor and thankfully it worked. Their newest little man had hung in there for thirty-nine weeks. He was born one week before he was due to make his appearance. It was on June the 17th of 2008 that baby Michael came into this world to join his family.
They named him after his dad but Mike and Lucy would begin to call him Micky. The name really stuck to him and he has been called by that name until this day. Micky weighed in at 7 pounds and 11 ounces. It was, go figure, a bit of a rough delivery. Micky was a bit battered and bruised, but otherwise, he was absolutely perfect in every way. There is something to be said for staying in your place as one should. Because he was so good, Micky got to go home with his family the very next day. This was very different from Mike and Lucy’s first experience and they were so happy that things went the way that they did. They were home and had two little boys! Lucy stated that once they returned home that the real fun started to take place. These two little guys were only fifteen months apart and as Lucy would put it, she “no longer knew the difference between night and day”. She would also admit that she loved every minute of it. Just as Mike had observed with his little nephew, Lucy was good at caring for kids. She loved it! Not only that but Mike had two sons. He was overjoyed! With the exception of having the same reflux issues that Danny had as a baby, Micky was the perfect baby boy. Things were really shaping up. Their boys were not only a blessing to Mike and Lucy but also to all of the extended family and their friends as well.
What do you think Mike did as the boys continued to grow? What else would he do but teach them about football! He taught them everything that he could about the sport that he loved. Both of his sons started walking just before their first Birthdays. Soon after, sounds could be heard coming from the garden as the boys ran after their father. With the the football being kicked you could hear the word “GOAL!!!” being sounded. Lucy said that she could remember how lucky that she felt at the time. She had Mike in her life and he was wonderful guy. She had two beautiful sons. Her feelings towards how life was at this time went like this, “Life was full of fun and laughter and it was more than I could of ever dreamed of.” They both had career goals and a nice place to live. Along with all of this, they had plans to increase the size of their family. First though, Lucy would return to work as Micky turned one. Life was good and it continued on for this family. One thing was for certain. This couple would have no issues with infertility. Just before Micky turned two years old, Mike and Lucy received the word that she was pregnant once again. Things would move along in the pregnancy and at twenty weeks they received the word that they were going to have another boy. Lucy said that it began to become a running joke that they were going to have their own football team at their house. Who could argue that things were starting to look that way. They were so happy with this news. They wanted to have a large family and they were going to have another son!
As was the pattern for Lucy’s pregnancies, so it was with this next one. Things would go well for the first thirty-four weeks. Once again Lucy would go into premature labor. I wonder what her thoughts were at this time. Something like, “here we go again!” They were given the same option as before and that was to use the drug that is necessary to stop the labor. They went for it as it had worked well the previous time. It did work well only not without complications. Lucy said that all was fine with the baby but she herself had become unwell. She had to stay at the hospital for a few days after receiving the drug but would recover and return home. It was, however, after a few days that that she began to feel unwell again and she had to return. After receiving a few scans, it was decided that they should go ahead and deliver the baby. Their little baby boy was born four weeks early and he weighed 6 pounds and 5 ounces. This took place on the 13th of January of the year 2011. Ollie was born. A prince unaware.
It never ceases to amaze me how mothers remember things about their children that are so specific. Of course Birthdates but sometimes also specific times and exact weights. Mums are like that. They remember specific things that happened with each child and feelings that were felt. I used the following as my opening quote but it fits perfectly here and it is so worth repeating. From Lucy, “To this day I remember the nurse placing this warm body onto my chest. I looked into his eyes and instantly felt an incredible bond. As quickly as this happened however our little boy who we named Ollie, our Olliebobs was taken off of me. I was not well and was rushed into theatre. A few hours later, and thankfully, I found myself holding this precious baby in my arms again. This time I had two more bundles of joy jumping on my bed desperate to meet the new edition. In that moment I didn't feel like life could get any better.”
Due to issues with Lucy’s health, she and Olliebobs had to remain in the hospital for an additional four days before they brought him home. Lucy said that she was thankful for the time that she had with Ollie in the hospital. Four days for time spent just with Ollie. With each one of her boys, there was difficulty but the results made what she would have to go through so worth it. Three boys! She was definitely at this point, well outnumbered, but she was okay with that! Lucy said that even at the very young age that they were, Danny and Micky took very good care of their mum after her return home. She went on to say that they gave the best kisses and warmest cuddles. How wonderful these guys were as they continued to grow. They were and still are, great big brothers and man! Can they kick a football or what?
Mike was continuing to work hard for his family and he had started working towards setting up his own business. As stated before, he felt that it would be possible for his boys to join him in the business one day if they so desired. That would include their new little man that they named Ollie! Lucy continued to care for Ollie and the others but also added to that the pursuit of another degree from the university. This one in Care and Education. She, along with Mike, was driven towards achieving their goals. They had three little boys and yet Lucy continued with her education. It was her third and last year of her degree. She said that she spent that last year juggling all her responsibilities. This included night feeds along with assessments for school. This not to mention potty training and all the other things that mums do for their kids. Just amazing! That is enough to wear anyone out but as Lucy began to get tired she knew something was up. It came as a complete surprise this time but she again received the news that she was pregnant. Lucy said that her and Mike felt a little nervous this time. After all, their boys were still so young. Soon, their nervousness turned to excitement over the fact that they were going to have another baby in the house. They loved kids and couldn’t wait. Lucy said that they were well aware of the need to keep a close eye on this pregnancy, given her prior history. They had a lot of extra scans done of this baby and the bond with this little one was intensified by having them done. Lucy put their feeling in this way, “We had lots of extra scans and bonded with our little baby even before it was born. We watched this little wiggle on the screen get bigger and bigger each week, waving its arms and kicking its feet! It was an amazing experience.”
Twenty-Four weeks into Lucy’s pregnancy they received the news that their next baby would be a girl. With that, the family’s aspirations for raising up their own little football team were gone forever. There would be no team but they were having a GIRL and they were so very excited!! The excitement spread to all three boys and as a family, they were “over the moon!” A daughter and a little sister. Everyone was so happy! The joy, however, was short lived as only three weeks later complications set in. It was at that time that Lucy started feeling unwell again. This happened while out shopping and panic hit everyone. Lucy said that Mike, being the person that he is, was absolutely amazing. He kept the boys calm while getting in contact with Lucy’s mum who took her to the hospital. It was confirmed while there that Lucy was yet again in premature labor. Oh my gosh! This time it was 13 weeks before their little girl was supposed to be delivered. As Mike was rushing back to the hospital, Lucy received some very unsettling information. She was informed that if she was to deliver their baby at this stage, the baby would most likely die. She was again offered the drug that had been used twice now on her. She of course said yes! This time, however, there was an addition complication.
Things went drastically wrong. Once given the drug Lucy was sent into anaphylactic shock. That being a life threatening allergic reaction. The hospital room was filled with medical staff who worked to save Lucy’s life. As Mike arrived, he stepped into a hospital room that looked like a scene from a movie. Lucy said that, like always, Mike was her rock as he brought comfort and helped her through her fear! The doctors and nurses managed to stabilize Lucy and her yet to be born girl managed to stay put. Over the next few days Lucy was ordered to bedrest. The doctors warned her that her baby could be born at any time and said that she needed to stay in rest. Lucy said that the days would turn into weeks and their unborn daughter stayed put. It was then that Lucy was allowed to return home with instructions to not move.
Lucy stated that her ability to remain still enough with three little boys running around was nothing short of a miracle. Their baby girl hung on for thirty-eight weeks and it was then that she made her own little dramatic entrance into this world. It was an extremely difficult birth and their daughter had to be resuscitated. This was terrifying to her. Lucy's sister had accompanied her into the labor room this time. She still remembers the look of terror that was on her sister’s face and said that she will for the rest of her life. Lucy was not allowed to hold her baby girl as she had been so used to with her sons. Instead, she was taken away. They took her from her mum for an hour and while that doesn’t sound like a long time, to Lucy “it felt like forever”. She was in the theatre (labor room) and had not known where her baby girl was. All she knew was that Mike had taken off after her as their daughter was taken from the room by hospital staff. The baby princess would of course be returned to her mum and amazingly, the doctors informed Mike and Lucy that their daughter was perfect! I had quoted Lucy in the first blog that I did about this family concerning what she was feeling as she held her daughter for the first time. Here is what she had said, “Breathing in that amazing newborn smell, feeling that warm breath on your skin, counting them tiny little fingers and toes. We had just been handed our beautiful baby girl, after a complicated pregnancy, and nearly losing her during labor.” They were now a family of six as the final piece was delivered in the form of this most perfect little package. She was absolutely beautiful. Lucy had this to say about their daughter after her chaotic entrance into this world, “She lay there looking up at me and Mike completely oblivious to the drama which she had created. Little did we know that this would just be how our little girl would roll.”
They had known in advance what they would name each one of their boys before they were born. Things were different with their new princess whom they had not yet picked a name for. Instead they spent hours looking through names trying to decide what would be a fitting name for her. She was to them so perfect in appearance! Finally, the name that was as perfect as their little girl was chosen. Amelia Faith Jasmine. She had been born on the 4th of March in the year 2013. She weighed 6 pounds and 13 ounces. Lucy said that they had never seen a more beautiful baby. This was confirmed by the fact that medical staff was stopping in to see her. They knew about her dramatic entrance into the world and were amazed by how utterly perfect she was. It was at this point that Lucy felt that their life as a family was complete and she looked forward to all of the precious memories that would be formed. So much had taken place during all of her pregnancies but they had all made it through.
Lucy would tell me that the boys fell in love with their new little sister the moment that they laid eyes on her. In fact, they promised that they would look after her no matter what. There was no jealousy, just brotherly love and kindness. She went on to say that her and Mike felt blessed, thinking that they had the perfect little family. The running joke that they would one day have a complete football team had been broken up by the appearance of little Amelia. However, there were still three boys that most likely would pick up their father’s passion for the sport. As they continued to grow, it became apparent that football would be the king sport at the Carroll home. Even though it was early, so many memories were already being formed. The possibilities for what the future might hold were endless. A family formed by two hard working people, three charming little men, and a little princess.
Lucy described the atmosphere in the home so perfectly as the children continued to grow, “Our house was always full of laughter, with children running around and playing. You couldn't walk a foot without tripping over toys. So many things got broken as the boys kicked their footballs around. Danny would spend hours training Ollie up to be just as good at football as he was. To this day I remember his shouting "Ollie heads, Ollie kick, Heads again Ollie. Yes that's it, yay, you have scored!" The cheers and the excitement that warmed our hearts.”
Their life together was a beautiful story in the making. In fact, it was already beautiful. Every family faces problems along the way. That is always to be expected. Mike and Lucy had faced some significant challenges but had made it through. They had their family in place and the future looked nothing but bright. They had many a conversation about what the future might look like and they talked. They talked about all that they would look forward to as parents. It was the type of atmosphere that dreams are made of. In the back of their mind, and in the forefront of their conversations, were all the life events that one would think of for all of their children. Neither of them had any idea what the future would really hold, or what additional challenges that would lay ahead. These were sweet times for them and their days were filled with joy and laughter. Lucy would say as much using the following words, “Without wishing the time again we often spoke about what they would be like as they grew older. Three brothers and a little sister. We laughed wondering if Amelia would love or hate having her brothers looking out for her, making sure no boys could get near.”
With only a couple of exceptions, the families whose stories I have shared had no idea that a wretched disease such as Battens even existed. Without any warning, life at the Carroll residence would soon change forever. What one would normally expect, was not to be. Lucy conveyed that truth with the following words, “Little did we know that the future which we dreamed of would never be.....”
I will share the rest of their story in my next blog.
Forever Royal. To be continued…..
“Our hearts dropped but at this stage we knew we could deal with this and still give her a beautiful life. We went home still blessed we could stabilize this.”
Before the correct diagnosis
How would you feel if you were me and you were provided the details of all of these stories? How would it change your way of thinking? Would it change the way that you looked at life? Would it cause you to look more at the circumstances of others instead of just your own? Would it increase your sensitivity towards children, especially those that are battling an incurable disease?
This blog has become so important to me. Telling the stories of those that face Batten Disease has become my passion. I feel a connection through my writing and it will always be felt that I am privileged to be able to do so. I know that I always sound like a little bit of a broken record when I say that I am moved by the details of your journeys. It has happened on every story that I have done. My mind will wonder and there will be a certain detail that causes me to well up with emotion. Sometimes it happens in the most inconvenient places. Is there a more important use of my time? I really do not think so. It is so much better when we choose not to live our lives in a vacuum. It is much better to care than to not. I know that the families that battle Battens are so appreciative of those that follow their journeys. Some would otherwise feel very alone.
It was not my original thought but part of my goal in writing is to help spread awareness. This has turned out to be, perhaps, my own unique way of doing so. I am happy to be able to do my part. The other reason for why this blog continues to exist is to let Batten families know why I feel that they are so very special. Although there are other journeys experienced by children and their families, there are not many like the one traveled by a Batten warrior. The constant care and the sacrifices demonstrate an unordinary type of love on the part of the caregiver. The heartache is pushed off to the side as determination to do the very best for the child takes over. It may sound like I am being overly dramatic but I don’t think that I am. The way that you press on each day is worth noting. We all love our kids. I know that I love all three of mine, however the needs of my special son Benjamin have caused the bond between the two of us to be off the chart. It makes a difference when somebody really needs you. For the parent of a child with Battens, the feelings go even deeper as the little warrior becomes the center of attention while they battle.
Being involved with Batten families has most definitely heightened my sensitivity towards children. I have talked about this before. When you think about a child’s appearance you may realize that it hasn’t been that long since they were covered in that brand new baby skin. Their little faces are free of any of the lines that age and life’s difficulties bring. Their actions, although sometimes a little naughty, are so adorable and fun to watch. Their childlike innocence should charm the hardest of individuals and it most often does. Although there are lessons to learn, a child’s existence should be free of stresses that are brought on by childhood cancer or rare diseases. Sometimes that is not the case though. A lot of people know my friend and fellow advocate, Sandy Garrett. She gets completely despondent when she loses a child whose journey she has been following closely. Sometimes we chat about all our health issues and how, in spite of them, we are still here. Then we talk about how unfair it seems that one of these kids will have their little lives cut short by something like Batten Disease.
I know that disease is part of the human condition but it really does seem unfair at times. Kids are supposed to be able to run and play. They are supposed to be able to enjoy their innocent fun as they learn life’s lessons. Being a warrior of this type should be left to people other than children. Anyhow, where I would like to go with all this is that I never thought about these types of things before I started following the journeys of kids with Batten Disease. Now, these kids are sometimes all I think about. The value of their lives cannot be measured in terms of dollars and cents. They are priceless to me and I know that I am far from alone in believing that! I can’t say it enough. The opportunity to write these stories is perhaps the best gift that I have ever been given. I don’t think that it is just a coincidence that I have opportunity at this time to blog about some of the families beyond the Atlantic Ocean and in the United Kingdom. Everybody in the Batten community is special to me. I hope that you know that. There are many that I admire and there are some that I have connected with in a big way. Some are my favorite characters and one of those characters is Claire Bradshaw from St. Helens, England. She is a Batten mum to one of the most adorable little faces in the UK. Her daughter Kaycee has CLN2 Battens and she is receiving the enzyme replacement therapy that is now available in a clinical trial. Little Kaycee is yet more evidence that the therapy works. The following is a little bit of hers and her mum’s story and I am so happy to tell it with Claire’s help.
There are many wonderful qualities that make Claire Bradshaw the person that she is. My favorite is the fact that she is oh so funny. It is my belief that laughter in life is so very important and Claire feels the exact same way. Don’t you Claire? She is funny and she makes me giggle. One of the other things that I love is the fact that she loves kids. When it came to having children, Claire was busy. She had 5 children already but there was room for one more. Four of her kids are not really kids anymore. Bryan is 27 and his younger sister Natasha is 25. Callum is 22 and his younger brother Tyler is 16 years old. Claire, please don’t tell anyone this but my oldest son Daniel will be 34 this month. Oh wow! I’m getting old. If her hands were not full enough, she would have another. She brought little Tamzin into the world and she is now 9 years old. So cute she is and makes her mum so happy! Each and every one of her children are so special to Claire and they help to make this family just that, a family. There would then come one who is royal beyond compare. A princess of a most rare kind. That would be little Kaycee Louise Bradshaw who was born on the 26th of April in the year 2012. Claire would tell me that she herself is a type I diabetic and that was perhaps responsible for Kaycee showing up two weeks early. There was no reason to wait. Right Princess? Kaycee was born so healthy and a pretty healthy size at 6 pounds and 14 ounces. It didn’t matter to Claire that this was her sixth time around. The whole experience brought a lot of joy to her as she welcomed this tiny princess into her family. Kaycee was so perfect in appearance. She had all her fingers and toes and you just know that she was covered in brand new baby skin. So beautiful. She was the newest citizen of St. Helens, which had been her mum’s home for all of her life. Of course our kiddos don’t come about without a daddy and there would be no exceptions with Claire’s children. As life would often have it though, relationships don’t always flourish. Claire would spend some of her time raising her children as a single mum and that is commendable. I know how much her kids love their mum and it is easy to see why. Good things would come for Claire in the future!
As Kaycee would continue to grow, her families adoration for her would grow right along with her. She constantly had a smile on her adorable little face and she caused everyone around her to smile. One thing would become apparent and that is, that she is Claire’s mini-me. Looking at that smile it was so easy to see that she has her mum’s personality. Little Kaycee was up and running in no time at all, and she would reach all of her milestones on time. When it came time to start visiting everyone’s favorite room, wink wink, she was right on target. That right there would make every family happy! The only thing that was of concern to Claire was that Kaycee’s speech was not developing as soon as she expected it to. Claire had plenty of experience at this point with observing developing children and she had her eyes and ears wide open when it came to her tiniest princess.
The pace with which her speech was developing was enough of a concern that Kaycee was scheduled to have a hearing test. This, after attending a routine medical check at two and a half years of age. Kaycee passed her little hearing test with flying colors and Claire was told that her princess was just a little slower. Could this, however, all have been a sign of other things to come? Of course, time would move on and Claire, along with Kaycee and her siblings, would forge ahead. As a family, they had no idea what they had in store for the future. They knew nothing about the challenges that would be out of the ordinary. The only thing they knew is that Kaycee was so cute and her smile brightened the entire room on the dreariest of days. She was endeared by the entire family and her charming little self was so loved. If you could hear her laugh, you would know just why! As the summer of the year 2014 approached, Claire and the family began to notice more things that would be symptomatic of a bigger problem. They noticed that Kaycee’s eyes would flicker. This darling little girl was a livewire and she would run like the wind, but things began to change. That would be most disheartening to all that knew her. Kaycee’s legs were beginning to collapse when standing, and Claire said that she had to be picked up more and more throughout the day. Claire was told that Kaycee could have a form of epilepsy, and an appointment to see a specialist was set up. There was no way that anybody could be sure of what was going on before more tests were performed.
How traumatic it must be for any parent when problems with their child arise. Each and every little person is a gift and a treasure. It is difficult to watch a child struggle as you hope for the best but prepare for the worst. The search for answers can come sometimes comes with ease, however sometimes the answers come with greater difficulty. False answers can bring optimism that doesn’t belong. A lack of answers can bring increased tension and worry. In doing the stories that I have so far, one thing is repeated over and over. That is that parents do not give up until they know what is happening with their child. In general, they do not quit. Their determination is always worth noting. For little Kaycee the testing would include brain scans, blood tests, and and a lumbar puncture. Poor sweet thing! The test results came back and it was determined that little Kaycee had Eyelid Myoclonia. This is a form of epilepsy that was thought to be causing the flickering of Kaycee’s eyes. It is often accompanied with brief absences. Perhaps this was what was causing her little legs to collapse as well. It was on September the 14th of 2014 that this news was delivered. Was it a correct diagnosis though? Claire’s reaction, in her own words, was that their “hearts dropped” with the news. Her little girl was special in a different way. She would bring an additional challenge to the family, but one in which they could handle the situation. They could deal with Kaycee having a form of epilepsy. She could be stabilized and perhaps have a close to normal life.
As anyone might guess, and as I have already spoken about it, a situation like this could be very stressful on a family. It could also be stressful on a new relationship. In fact, that is where Claire would find herself. It was six to eight weeks before all the circumstances concerning darling Kaycee hit, that Claire would meet her future husband, Greg Riley. They clicked right away and would begin to spend time together. A lot of men would see something developing as it was with Kaycee, and decide that the situation was not for them. This was not to be the case for Greg. He saw the situation as one in which he could help and that is what he did! I have no doubt that the love that had developed between him and Claire, along with her kids, left him with no other choice. How could you walk away from little Kaycee in this time of need? In fact Claire said that she told Greg on many occasions that her situation was not his problem. Claire stated that her Greg in response, just looked after her and her daughters all the more. Outstanding. What a stand up gentleman! Oh, and did I mention that his name is Greg? Why yes I did. He is the other Greg. Or, maybe I am.
As suggested before, early diagnoses are not always correct. It was only a few weeks later that more changes started taking place in Kaycee. Ones that would heighten everyone’s concern. This darling little girl started falling harder and harder. This would occur anywhere from 8 to 10 times a day and things were getting worse by the week. Can you imagine the increasing anxiety as the situation grew worse? There was no way that this mum was going to give up until she had an answer. Doctors would continue to give the family false hope and wrong answers concerning Kaycee’s plight. Claire knew that there was more to what she was seeing than just epilepsy. I am certain that she was receiving strength from the fact that Greg and her kids were standing alongside of her, but little Kaycee was in the forefront of her mind. She was insistent that the doctors dig deeper. The answer would eventually be found, and here is how Claire would describe receiving the news, “Almost a year to the day of Kaycee getting worse. Of watching her mobility go from zooming around everywhere to relying on mummy and daddy carrying her everywhere. Receiving enormous amounts of tests on her little tiny body, we were blown away by the worst news ever.”
That news brought with it the knowledge that little Kaycee had Batten Disease, the variant CLN2 or Late Infantile Battens. Just like any parent would wonder, Claire and Greg would ask. What is this disease? What will happen? How do we cure this? Claire said that their heads dropped along with the nurses that were in attendance. Their lives were changed all of a sudden by news concerning a disease that they had never even heard of. They went from having hopes of being able to control Kaycee’s symptoms to being completely hopeless. Darling Kaycee’s life expectancy was all of a sudden anywhere from 6 to 12 years of age. Their priceless princess had a terminal disease. Claire put it like this, “Our life's stopped there and then. On that Friday, August 31st, 2016. Not even our nurses knew what Batten Disease was as Kaycee was the first child they have ever seen with it.”
Can you imagine the shock of being told that your child has a rare disease for which there is no cure? Like with all parents, Claire’s reaction shows the devastation that this kind of news brings, “Our world was shattered. I couldn't walk, couldn't move, just tears and waiting for them to tell me it was a joke. We went home knowing we had 5 other children as well as family to give this devastating news to. How were we gonna tell all the other children?”
What could they do but return home and try to make sense of things? The family was sent home with information about BDFA (Batten Disease Family Association) and were encouraged to contact them. Perhaps Claire and family could receive the support that they would need from the workers there and other families that were going through the same thing as they were. This is exactly what would happen. All of sudden, they were part of a community that they knew nothing about. They would soon discover that they were not alone in this. There was help out there from a very caring circle of support. People that understood. One could only imagine the reaction of family members, especially Kaycee’s siblings, as the news was given. The princess of the family. The one that they all adored was in trouble. After a couple of weeks, they regained their focus and contacted BDFA. Can you imagine all the things that were going through Claire’s mind at that time.
It never ceases to amaze me, although it shouldn’t. The support and understanding within the Batten community is so remarkable. Only the families themselves and those that work on their behalf can truly understand what it is like to be given this news. Claire received a call back almost immediately after she contacted BDFA. The result would continue to bring about a whirlwind of events but also some hope for a course of action. The family was informed about a form of treatment that was available. It would replace the enzyme that was missing in little Kaycee’s brain, and it was part of a clinical trial that was showing a lot of promise. This was not a cure but it would improve Kaycee’s longevity and quality of life greatly. This was something that was so important. Granting her time in hopes that a cure could be found. This of course brought up some questions. Things like, how does it work and will this princess do well with it? You would have to think that Claire’s head was spinning with all this new information. Can you imagine beng Greg and having this on your mind as you try to function at your job. As for Kaycee herself, she was, of course, unaware of all that was going on around her. There was only innocent laughter and continued smiles as this was taking place. Claire was informed that there were five spots open under the compassionate use program and her princess would be one of those five. Most any parent faced with this situation would jump at the opportunity to help their child. This is how Claire would describe her reaction, “I couldn't take and hold it all in. But one thing I kept ahold of was them saying it's been proven to be able to prolong our baby's life. With holding onto those words, I knew we were going to London for the compassionate use of this drug.”
Yes, they would go to London and not to just any place. The Great Ormond Street Hospital is part of the leading edge of the medical community when it comes to children’s health. In fact the hospital is quite famous. It was founded originally in 1852 and at the time it had only ten beds. It quickly grew into something much larger. According to Wikipedia, it is the largest centre for childhood heart surgery in the UK but it doesn’t end there. The list of things that have been accomplished at GOSH is immense and their commitment to children includes those who have Batten Disease. To that, their contribution is vast. Families that have children in a number of clinical trials travel to GOSH on a regular basis at specific intervals. I am told that the families that go there become extended family to one another and Claire and Kaycee would soon find that to be true.
So it was at the beginning of December of 2016 that little Kaycee would have brain surgery to implant a shunt. The shunt would be used for delivering the chemical that replaces the missing enzyme. It was later in the month of December that princess Kaycee would begin receiving her treatments. This required that Claire and others travel on the train with their darling to London every two weeks. The level of dedication that is shown by her and all the parents is amazing. Feelings run deep, I am sure, as one might expect. I have seen so many pictures of these darling faces with bandages wrapped around their heads. This after each child peacefully receives the enzyme replacement therapy. Claire and family lovingly refer to it as the “Miracle juice”. This because it works! It cleanses the brain of wastes that would otherwise be impossible to get rid of. It has been proven to be safe and it is very effective. To me it seems that any rational parent in the same situation would do what it takes to get their child this therapy. I, however, completely respect each family that has made the commitment to help their children. As I am writing this article, it has been 14 months since Claire and other family members started accompanying princess Kaycee to London. She is such a strong little girl and did I already mention her smile? You should hear her laugh, just priceless. According to Claire, the Great Ormond Street Hospital has become their second home and I can believe that. We need a cure to CLN2 and all the other variants of Batten Disease. In the meantime, there is this wonderful treatment available. What wouldn’t you do for your own child? Claire can’t be certain what the future holds, but she wouldn’t do anything different at this point. She shared that her purpose right now is to keep Kaycee alive and pain free for as long as possible. Wouldn’t you do the same thing?
It would be wonderful if the only thing that Greg and Claire had to be concerned about was getting Kaycee to her treatments every two weeks. There is, however, a very major concern. That is whether or not the cost of the treatment will be covered in the future. Yes, that’s right. The treatment that is now being received by Kaycee and other children with CLN2 Battens could be taken away. In fact, there are children that could benefit from the treatment that are not receiving it. This because of a recent decision made by two agencies in the UK. I reached out to my friend, Lucy Faith, in an attempt to make sure that I had all my facts straight. Most people that follow children with Batten Disease know who Lucy is. For those that don’t I will tell you that Lucy and her husband Mike Carroll are parents to two of the most adorable Batten warriors that you will find (I actually adore all of these little warriors). They are actively involved in work as advocates on behalf of all the kids with Battens in the UK and abroad. She explained that the two organizations are the National Health Service or NHS and the National Institute for Health and Care Excellence or NICE. NICE is responsible for giving recommendations to the NHS on whether or not to fund new drugs and treatments. Even though there is clear evidence that this treatment works NICE recommended that it NOT be funded by NHS. This according to Lucy was based on the fact that they felt it was not a good use of NHS resources. They also felt that there was a lack of evidence, long term. Ollie’s Army (the Carroll family) along with others in the Batten community are now involved in a Battle that is seperate from the fight against the disease. They are not giving up and are joining forces to get this decision overturned. I think that this entire issue points to the fact that government agencies can be so very impersonal in their decision process. Lucy talked about the process of evaluating the evidence and I will let her share it herself,
“The pharmaceutical company, doctors, BDFA and the parent representative (us) have put in all our evidence to NICE. We then attended the meeting in January where we were met with a panel of 18 professionals from NICE. We were all questioned on our opinions, and all the evidence and proof from the trial were shown.”
In spite of clearly showing that the treatment works, a vote to recommend funding was denied. I wonder what it is like to have to live with yourself after making that kind of decision. I personally wouldn’t sleep well after that. These kids are priceless and it is the right thing to do in making sure that this treatment is available to them. A petition has been started and at this time it has approximately 82,000 signatures. They would like to have at least 100k. If you are reading this and have not signed the petition, I would ask that you follow the link provided and PLEASE sign. It only takes a few minutes.
Kaycee is one of only five children in the UK that are on the compassionate use program for this treatment. There are, however, others that receive the therapy, and there are also many here in the United States. As I mentioned earlier, there are kids that should be receiving this treatment but who are not because of the decision that was made only a couple of weeks ago. Can you imagine what it would be like to be a parent and have to face this type of thing? Claire is not sure where they stand, and princess Kaycee could be taken off of the treatment at any time. Of course kids like Kaycee are completely unaware of the circumstances that surround them. It would be our hope and prayer that this decision would be reconsidered. It needs to be.
My last blog was about James Toohey and his mum Kate. Like Claire and Kaycee they travel to London every two weeks for the treatment. Kate talked about what it is like to go and spend time with the other families while their children receive this life changing therapy. One of the things that she included was how amazing these kids are. She brought up the fact that these little people haven’t a clue as to what an influence they are having on the world. More and more, people are learning about kids with Battens and other rare diseases through social media and increased awareness. To the caring of heart, these stories captivate the individual. They are stories of undying love and sacrificial giving. (BROKEN RECORD ALERT). Yes, and the indomitable human spirit that is displayed by these kids along with the smile that each one possesses. These children are something that you cannot put a price on. Not only that, but there is something else, in my humble opinion. When it comes to human life, you do whatever it takes to preserve it. Especially when we are talking about the most precious of children. The fact that there is no long term evidence concerning the success of this treatment means one thing. You need to create it or it may never exist. Just look at these kids and you will see that it is working! IT WORKS!
I’ve said it over and over again but will continue to make my point. Every little bit helps when it comes to raising awareness, and I have my little part in that. My real reason for writing though, has always been to honor these families in telling their stories and what they face. They move forward each day under difficult circumstances. This, while demonstrating an extraordinary kind of love. There is something about these children that I find so very special. It is difficult to put my finger on exactly what it is, but they have a big piece of my heart. The words often escape me, but it is there. Maybe it, in part, is what they teach us about ourselves and how they change us. Claire told me that Greg and her children are her rocks. They steady her. It is difficult for her to be everything all at once. That is a mum, nanny, nurse, and partner. Claire keeps her sense of humor because she feels that is the best way to deal with things, and I like that about her. Greg has the challenge of holding down his job and supporting his family with all of the distractions that take place. I know that he gladly accepts the challenge. Claire says that he is a great daddy to her two younger girls and has stayed close them throughout. He is the family’s guardian angel. As a family, they will do what is necessary in support of princess Kaycee. They are so blessed to have her in their house, and she is loved by everyone that knows her, including me. That, even though I live an ocean and a continent away.
As I was drawing close to the completion of this story, I was chatting with Claire and gathering more details. It was her bedtime there in England and I wished her a restful sleep. She said thank you and told me that she would rest well all snuggled up next to Kaycee. I wish her a ton load of days and many years with Kaycee in their midst. She is so very special (as is her entire family). In fact, she is the Priceless Princess from beyond the Atlantic Ocean. As for me, I am a blogger of a different sort, and a friend to the families that make up the Batten community. This means more to me than you could know. Thanks for having me!
Blogger and Advocate
“He is by far defeating the odds and we are extremely proud of him. Please ask me anything else as I would like the world to see that James is the walking talking living proof that this treatment works. He needs to carry on with receiving it.”
What is Batten Disease? Just ask the parents and they will tell you. This because each one is forced to become an expert on the disease once their child is diagnosed with it. Previous to that point, most had never heard of Battens. While searching for answers, many will see several physicians and their child may be misdiagnosed more than once. In fact they most often are. Once the truth is found the diagnosis brings with it the devastating news that their child has a fatal disease for which there is no cure. Almost without exception, each parent starts to research the disease in hopes that knowledge of Battens will make a difference. It is also true, that they just want to know more about that which they previously didn’t even know exists. As a family, Batten parents were enjoying everything that comes with being the recipients of a young child’s love. Not just any child but the one that they had dreamed of having in their presence. Then, almost all at once, their lives were changed. Some go on exhausting searches in the beginning, while others find answers more quickly.
Like with most anything else, the sooner the answer is found, the better it is. In the case of a child with Batten Disease, the benefits of knowing the truth early can make a big difference in their health and in knowing what care to provide. As I have stated previously, it is the love demonstrated by Batten families that has given me a desire to tell their stories. The amount of sacrifice that takes place is so noteworthy. Most often it involves a financial burden that requires private fund raising. It always involves time and an extensive amount of caregiving. Often times, there are procedures that have to be performed at home by the parents and there is usually several medications that have to be given. Many days are often spent away from home when medical care is required in a hospital. As a family, one or both parents do whatever is necessary to help their little warriors press on. Regardless of how the disease has progressed or what they are facing at the time, Batten families do whatever it takes to serve the best interest of their child. It is the details of this type of love that often brings me to a level of emotion that I otherwise would have never experienced. I want to empathize with Batten families as best that I can. Mostly, I want to honor the sacrifices and talk about them as a community. All of you, as families, experience the difficult circumstances and you feel the emotions personally as a result. For me, and the others that follow your journeys, we see the sweet details of your love towards your child. I think that I represent those of us that follow the Batten community well when I say that you are some of the best examples of sacrificial giving that one could offer. Yes, it is true that you are just being parents but you have been asked to do so much more than most will ever be asked to. It is my goal and my desire to tell as many people as I can about what you all go through.
So again, what is Batten Disease? I am learning as I go. The disease involves things like seizures, blindness, and also progressive neurological impairment. I know that there are different variants of the disease and that it involves genetic material that is inherited from both parents. It is rare because both parents have to be carriers of the same defective gene and that rarely happens. I have read the statistic that says 2 to 4 cases occur in every 100,000 births in the U.S.A. While that does sound rare, the prevalence of the disease seems to be greater. The disease does not seem to be that rare if you follow these families and you personally know about the number of children with Battens. There is a severe lack of funding for research because of the fact that the disease is considered to be rare but some great progress has been made in spite of that. This because of the work being done by some concerned individuals both in the medical community and in the private sector. Gene Replacement Therapy offers hope for finding a cure for some of the variants but it carries with it a hefty price tag. I hope that one day the clinical trials that are being performed bring about real change. We need a cure!
One of the most common forms of Battens is Late Infantile Batten Disease or the variant CLN2. It happens to approximately 20 precious children in the United States alone each year. Add in the rest of the world and the numbers are much higher. Although parents of these children are still looking for a cure, there is a form of therapy that has been made available to them in a clinical trial. It uses a solution that has been produced that serves as a replacement for a missing enzyme within the child. It is injected directly into the brain through a port, much like they would use in oncology. Many of the families that I follow, here in the U.S. and abroad, have children that are recipients of this therapy. It is working wonders. It is generally administered to the child every two weeks at a facility that is equipped for the procedure. It often times requires travel on a regular basis. Sometimes, it requires moving to a different area within the United States and some families have even moved to this country to have their child treated. Similar things happen in other places. Sacrifice! Every family’s story contains a mixture of difficult times and sweet details of tender care. As such, it is an absolute joy to see kids that are thriving as a result of this therapy. There are some stories that are exceptional and I would like to share one with you.
My new friend Kate Toohey, of Liverpool England, contacted me late last year because she wanted her son’s story told. His name is James but he is referred to affectionately as Jamesy Boy. Now when the city of Liverpool comes to mind, it makes me think of a famous group of musicians. You may have heard of the Beatles, right? Maybe I am dating myself but I think that everyone knows who they are. Maybe you are a sports fan and you love Soccer. Um, it is actually called Football. Well two of the Premier League Football teams reside in or nearby Liverpool. That would be the Football clubs of Liverpool and Manchester. I could share more about Liverpool but I would rather talk about this little guy named James and the impact that he is having on his family. Let’s get to it. Kate has shared several videos of James running and playing as any child would. That is because she wants to place emphasis on the fact that her son is thriving and doing so well. This in spite of the fact that he has the CLN2 variant of Batten Disease. Things weren’t that way earlier on but after a quick whirlwind of events, Jamesy Boy is turning some heads and putting smiles on a lot of people’s faces. And Kate? Well, she has discovered a community that has had a tremendous impact on hers and James’ circumstances. I will do my best to give some of the wonderful details as I tell the next story. The following story belongs to James, his dear mother Kate, and their family. It also belongs to those caring individuals that work within the Batten community to affect change.
James John Toohey was the most perfect little baby as he made his appearance into this world. This happened on a Saturday, the 7th of May 2011, at 12:46 pm. As life would have it, Kate would start parenting as a single mum. That doesn’t mean that she was all alone though. With her at the time of James’ birth was her own mum Margie and her close friend and sister-in-law Natalie. All together, they were elated over the presence of Kate’s little prince. He came to them with all his fingers and toes and yes, he was covered in brand new beautiful baby skin. So perfect! Kate had expected James to be a big baby, however he was delivered to her weighing in at 5 pounds and 13 ounces. His smallish size did not matter one bit except that he was swimming in the clothes that Kate had purchased for him. When the staff brought James back to Kate, he had a tiny white hat on his sweet little head. It was tipped slightly over one of his eyes and this made him look all the more adorable. She still remembers what she was thinking as she held her new little man in her arms, “So adorable, so innocent, and I was responsible for him…” Although she was a little intimidated by the new responsibility, Kate anticipated, with excitement, all that was ahead for her and James. Having baby James in Kate’s life fulfilled a desire that she had held for a long time and being a single mom didn’t bother her one bit. In fact, she welcomed the opportunity. In her own words, “I always wanted to be a mum. I was the one to go for the baby of the family at parties. I loved being a single mum to be honest, having him all to myself was amazing. He wasn't spoiled and slept in his own little room. We had a great routine.” According to Kate, James was a happy little guy who didn’t cry too much. He was a welcomed part of a good sized family. Kate would tell me that she has amazing parents. Married to her mum Margie is her wonderful father, Jim. she has a sister named Laura and two big brothers named John and then David. The marriages of her siblings brought nieces and nephews to this growing group of people. In other words, they were a family.
As James continued to grow, he would add much joy to this growing family. Especially to his mum. He was such a happy little guy and and he was a pleasure to be around. I am sure that Kate had many thoughts and dreams about James’ future. Who would he grow up to be? What would he become? As part of the family James would of course have cousins to grow up with. In fact, Kate’s sister Laura had delivered a little boy just six weeks before James was born. His name was Thomas and he would become known affectionately as Tom Tom. The presence of Thomas would of course create an opportunity to draw a comparison between his and James’ development. It was at about ten months that Kate noticed that James was slower at reaching his milestones than Tom Tom was. Thomas was up and and trying to walk and he was eating a more varied amount of things than James was. James was up and walking at 20 months. It was also at that time that he had only begun to use a few words such as hiya, mummy, oh dear, and no. James and Thomas spent a lot of time together as both of their mums were quite close. The differences between the speed of their development was obvious. Being a small baby at birth, and having some minor issues early on, the family felt that James was just “catching up”. While some kids do develop their skills slower than others Kate had questions about what she was seeing. As she would put it, “I think deep down I knew there was a obvious difference.” She remembered speaking with her uncle concerning James and the differences that they were seeing,
“I remember a conversation with my Uncle Peter and he didn't want to bring the subject up about James, thinking maybe he was slightly autistic. I was glad he did as he helped me admit it and at an early age. I think I knew, James was different and was his own unique person.”
Perhaps at this point Kate was thinking that she had some special challenges ahead of her with James. Whatever the case might be, she would handle it with the support of a loving family. She had no idea that the differences that she was seeing were a precursor to a condition that she had never heard of. The future challenges were greater than one would expect. This little guy was more special than Kate could know at the time and his future impact on family and friends was yet unseen. This, not to mention the fact that James was just so adorable. His story would begin to complicate, but it would also bring with it details that are sweet and so very special.
It was three months before James’ third Birthday that a major event would take place. He had a seizure while he and Kate were at her mum’s house, along with Laura and Tom Tom. The seizure was approximately 20 minutes in length. Kate was not sure what was going on at the time. She just knew that things were not right with her Jamesy Boy. At the time of the seizure, James was was lying on the floor and was stiff as a board. He had a strange smile on his little face and his eyes were locked to one side. Besides all that, he had a small groan coming out of him. Kate had picked James up as her sister jumped to the phone to call emergency services and she remained calm while holding him close. They were told by emergency services to lay him down as he could be having a seizure. Kate said that as she did that James’ little body started moving erratically with his arms flailing and foam coming down the side of his face. She would tell me that it felt like forever until help arrived. As the ambulance got there, James was blue lighted to Children’s hospital in Liverpool. How scary this entire event must have been for the entire family. The differences in James’ development and then the advent of the first seizure must have left Kate wondering what the future would hold. As James was seen at the hospital, the family was told that he had a high temperature and an ear infection. The seizure was attributed to that. This is all sounding too familiar. James was prescribed antibiotics and the family was sent home. Not knowing what was ahead of them, they were comfortable with what they were told and Kate was happy to leave the hospital. As a family, they carried on with life but Kate had a question stored in the back of her mind. That is, what if it were to happen again? She continued to keep her eye on James and checked his temperature routinely.
James would have another seizure a week or so later. This while walking from the house to have a picnic with her sister Laura and Kate’s niece and nephew, Evie and Tom Tom. At the time that it happened, James’ head tipped back in his buggy and Kate knew right away that he was having a seizure. This was difficult as the two other little ones, Evie and Thomas, were also with Kate and Laura. They needed to stay calm for the children’s sake. They quickly returned home and James was seen by paramedics after which he returned to normal. Kate said that she knew that this occurrence was not having to do with just an infection and high temperature. A trip to james’ doctor was scheduled. I am positive that the level of concern that Kate had for her son was growing by the day. Obviously, there was something more going on. James had an appointment to see a doctor for a different issue at a local children’s hospital. While playing with another child in the waiting room, James would have yet another seizure. It was after this that doctors had James sent for an MRI. They also requested blood tests. After waiting for the results, Kate and family were informed that James had a form of epilepsy. They also wanted to look into autism. The neurologists who viewed the results of the the MRI scan had seen something that he didn’t like and he wasn’t sure what form of epilepsy he might have. This also resulted in more tests for James. It included more blood work being done and this time it would include Kate.
Kate said that it was at this time that she was informed that James could have something called Batten Disease. She had never heard of it and had no idea what it was. Her mum, however, did. Margie had recently seen a television program that talked about a little girl named Amber that had Battens. (sweet Amber would eventually lose her battle to the disease). Margie, therefore, knew what the disease was and she was terrified over the possibility, however, she kept things to herself. I am certain that she kept it to herself in order to not upset her daughter and in hopes that the testing for Battens would come back negative. During this time, they were introduced to the neurologist. His name name was Dr. Iyer. According to Kate, Dr. Iyer was a very positive and pleasant individual. He informed Kate that there was a 50/50 chance that James had the disease. His testing was was first to include Battens in order to eliminate it as a possibility. The family had to wait 4 weeks or 28 days to receive the results. In one comment, Kate referred to this period of time as “28 days of hell”. Who could argue that it wouldn’t feel that way to any young mother. Kate was told to not research the disease but she knew that it wasn’t something that carried with it a good prognosis.
It was time to learn of the results. Kate had planned on being accompanied by her mum, sister Laura, and her sister-in-law to receive the answer. Kate took the bus to meet her family at the hospital. She still recalls her feelings on that bus ride, “I remember the bus ride and not wanting to get to the stop”. Who could blame her for that? She would go on to say something that signifies what must have been felt at the time by all of them, “no words were said when I saw my family. We just walked together towards the hospital.” Their silence actually spoke volumes as to the tension that was being felt. Once there, Kate’s sister-in-law was asked to watch James in the reception area while the others talked to Dr. Iyer about the results. On this day, the normally upbeat doctor was not smiling. The doctor had news that no one would want to deliver to a young mum. Doctor Iyer never really told Kate that James had Batten Disease. When kate first saw the doctor she said, " he's got it, hasn't he?" With a tear running down his face, this very caring physician simply nodded his head, confirming the fact that James had Batten Disease.
Kate said that with that her sister Laura fell to the floor and her mum screamed. As for Kate’s response, she simply stated over and over,
"my baby's gonna die". She told me that she wanted to know how and when the end would come. Would she wake up one day and find James in his bed, gone to eternity? Kate describes what her thoughts were after receiving the news, “I always said that it felt like a soap opera where you see something tragic happen. The family is told and someone falls to the floor. It looks so dramatic. This time it wasn’t a soap opera. It was my life.” The diagnosis took place on 28th of July in 2014.
I have done a number of these stories now. In the vast majority of them, the diagnosis of Batten Disease comes within a varying length of time and never quickly. In some cases, the truth is found out in a few months but there have been some that take a lot longer. When that happens it involves false diagnoses and a lot of tension and heartache for the parents. It seems that in those cases, the news gets worse as time moves forward. Think about the difference it makes when you receive a quick diagnosis. Even though Battens is fatal, you can deal with the symptoms more quickly in a more effective way when you have the answer sooner. There are stories out there that are just nightmarish. Long searches for answers. As stated at the beginning, James was diagnosed with CLN2 or Late Infantile Batten Disease. In a span of only four months from the time of his first seizure, the family had their answer and they were given some hope concerning a treatment that was available. It showed real promise. Not a cure, but something that would greatly improve longevity in hopes of a cure. Having a medical community that is well informed and is knowledgeable makes all the difference in the world. Doesn’t it? Kate would describe perfectly what transpired after receiving the diagnosis,
“Straight away, Dr. Iyer wanted to inform me of a chance of hope, the trial. He asked me to return in a few days time and we could talk bout it.
A few days later, myself and my oldest brother John along with James, returned to see the doctor. He informed us of the trial and that he had already spoken to the doctor leading it at Great Ormond Street Hospital.
He had only just heard of the trial and wanted to make sure James had a chance of making the trial as he was the perfect candidate. To be part of the trial the child needs to be able to speak a certain amount of words and be able to walk unaided. As Dr. Iyer knew, James could do all this and more. In a way it was a blessing that he was tested for it first as it gave him the best chance with this treatment.”
So bracing for the worst of what might happen, Kate had the hope of this clinical trial that was just starting. Thanks to a physician that cared greatly and progress that had been made, a treatment was available. The enzyme that was not being produced in Jamesy Boy’s body would be replaced by a material injected into his brain. Due to the quick diagnosis and the perfect timing, he would get started at just the right time. Would all this make a difference? You just know that the answer is yes! So Kate, with the support of her wonderful family, made plans to move to London. They would begin the trial in December of 2014. This would include surgery to implant the device needed for the infusions. It was right after the diagnosis that Kate was informed about an organization that could offer her assistance with what her and James were facing. The organization is BDFA or the Batten Disease Family Association. Concerning their mission, they state the following, “to enable everyone who is affected by Batten Disease to live life to the full and to secure the care and support they need until a cure is found.” Kate was soon to find out just how very caring and supportive the staff at BDFA was. In the process, she would meet a very special person. Kate would also discover a community full of compassion towards what she was going through. There is also no doubt the tender care of a medical community at Great Ormond Street would become a blessing to her and James.
Everything changed for Kate in an instant as James was diagnosed with Batten disease. In her own words, “From then on life was different, everyone was different. Family and friends organized events and raised awareness and money for James and the BDFA. Then it was time to move to London.” They sent James off by first having a fancy dress party for him as they had no idea how long they were going to be living in London. Kate said that it turned out to be an amazing day for James and all the family. Initially, her sister Laura and her mum came to stay with kate and James. Her brother David drove them to London with all of their belongings. Packing everything up and moving to the other side of the country was a difficult task for her, however she really had no choice. Kate got down to business and got things done. She was, up to that point, always with family and friends but was soon to be alone with James in a city that was strange to her. This is how she would put it, “Always having another child or a mate with me, it was so hard moving so far away. At first it was like being on a different planet. Being from a pretty big city, I thought we would adapt but we couldn't. We had to get on with it though, we had no choice.”
When I was talking to Kate about doing hers and James’ story, I had some ideas on who should be included. She thought things through and talked to family and she wanted to make sure that one individual in particular was included. She said that the name of the person that she wanted to include was a lady named Mel Hall. My eyes lit up when I saw this in my chat window. This because I was a facebook friend of Mel’s but I had never got to know her all that well. I knew that she was a Batten mum but that was about it. To Kate, Mel was a very important person and I wanted to know why. In fact, she referred to Mel as “her rock”. I learned why that was after reading what she had sent me on Mel.
Kate said that she was numb from all that was going on at the time that she was told about BDFA. It was at that time that her sister Laura had decided to come and stay with Kate and James for a while in order to help. Laura had felt at a loss, watching Kate being so vulnerable and James just being James. Kate felt that she couldn’t face explaining things to the BDFA representative so Laura stepped in and explained their situation. Once they had gotten settled into London, Kate was asked if it was okay if she met with a BDFA support worker named Mel Hall. She was worried as she didn’t want to speak with a stranger. After all, how could she possibly understand what Kate was feeling at that time? She reluctantly scheduled a time for her and Mel to meet at their apartment in central London. Mel was having a difficult time finding the apartment so she rang Kate’s phone. This fortunately broke the ice, and once she arrived the conversation began. She wasn’t sure what it was but Mel had a presence about her and she took to James right away. She would later grow to adore James and I think there is a big reason for that. They talked over coffee as they began to chat (Mel loves coffee). Kate said that she can’t remember the exact words with which the conversation started with but she said that it went something like this, Mel said “How do you think me being here will help'” and Kate, in response said something like, “You can’t know what I’m feeling if you’re not going through it.” Mel, in response simply said “I do know”. In fact, Mel did know exactly what Kate was feeling!
Mel had experienced much as she was a grieving mum that had lost her precious son Matthew at age 7. She had dealt with Battens in a much greater degree at the time her and Kate met. Matthew’s battle took place in a time when there was much less awareness in the UK and in other places. Like many other families, Mel had to go on a search for answers. She faced the agony of not knowing what was taking place with her son and then the devastation with the news. Matthew had Batten Disease. The clinical trial that was available to James at the very beginning was never available to handsome Matthew. When Mel expressed to Kate that she knew what she was going through, she really did and then some! All of that experience was available to Kate. This because Mel had taken the tragic circumstances that she had experienced and turned them into an opportunity to help others. She was there for Kate in a big way. This is the way that Kate explained it, “She didn't want to tell me but I did ask, was she a Battens mum. She then told me about her battle and the story of beautiful Matthew. She never pushed it, she let me ask questions. Things that were probably the worst things in her life to experience. She shared them with me. She didn't shy away from the truth and that's what I needed. She knew what type of person I was, and even as a young single mum. she was extremely shocked at how well I was dealing with a huge life change.”
Kate would tell me that Mel became her go to person and she would become very active in Kate and James’ life. She helped to secure James’ first disability buggie and she got an Epilepsy alarm for if he had a seizure during sleeping hours. She arranged for them to all visit a beautiful hospice facility together and it turned out to be a wonderful day for Kate and James. Kate said that Mel arranged for James to receive an ipad from an awesome charity. James unfortunately lost it on a train so Mel arranged for him to receive another. James still has a pair of pink sunglasses that he took from Mel’s purse. Kate continues her sentiment about Mel with the following words, “She is and always has been the person I get my strength from. She is able to come and support, comfort, explain where it hurts. She also shows you a determination to not give up.” Mel exemplifies the character that I see in so many people within the Batten community. What impresses me about Mel is how she has taken her own experience and grief and has used it as motivation for a career that benefits others. She is truly special. Mel now works at a children's hospice full time. In fact, it is the hospice that helped her son Matthew while he was battling Battens. Kate goes on,
“I miss her and really want her to see James and how fab he is now. I know he would remember her. She is a woman of strength and passion and she does that for Matthew. She has stuck to that promise she made to him to fight always and he would be extremely proud of her. I honestly love this lady with all my heart. I haven't seen her for a very long time but I know she's always there. She came into my life at a really hard time and she still doesn't understand how much she helped and still does. I knows she’s there all the time no matter what time and I’m always in awe of her.” You know Mel? I am a little in awe of you as well. Everything that Mel does is for her Matthew and the other children. Just amazing! Oh, and by the way Mel, I love coffee too.
There would be other hurdles to jump over after moving to London. The biggest one was the surgery to implant the device. The night before was a sleepless one and there was much anxiety. Kate had questions. How bad would James be scarred? How much pain would he be in? How was all of this going to affect his life in general? Kate knew that these were questions that she couldn’t answer. When it came time for the surgery, there were only so many people that could go near the operating room. Accompanying Kate and James was Kate’s dear father, Jim. As they were leaving sister Laura and Kate’s mum behind, James shouted “Bye Laula”. According to Kate, this caused her sister, and no doubt mum, to hit the floor. She was so upset and who could blame her? After all, this is a family. Their fright was turned to pure joy though as Jamesy Boy’s surgery was a complete success. Kate would look on as the doctors worked on James. She described what she witnessed in this way, “That wasn't a nice experience. Watching doctors with all the medical gowns on. Seeing the tiny needle with the machine and wires. I was terrified.”
The doctor returned to the family having a huge smile on his face. Everything went perfectly. Great news! James surprised everyone with the speed of his recovery. His family thought that perhaps he would be in bed for days after the procedure. That was not to be the case as he was up and running the next day! After that began the regiment of treatments which would take place every two weeks. It was time to settle into a routine and to live life away from home as best that they could. Kate’s family stayed involved long distance and I know that they would drop in as much as they were able. It was always her desire to return home and from there they would make the trip to receive James’ treatment every two weeks. They had figured on Kate and James needing to live in London for at least a year and it actually took almost two years before they could move back to Liverpool. One of the big obstacles is that they had to find a suitable dwelling place. One that met the needs of this little guy that loves to run and play. Kate had a desire to get James home to Liverpool and have him enrolled in school. She also joked that at that time, James was losing his Scouse Liverpool accent. She needed to get him home for that reason as well. Wink wink. This would all come in time.
This is not to say that they did not enjoy their time in London. Family would visit and they would see the sights together. What was amazing to Kate is that which I already suspected was true. That is the relationships that were built with the hospital staff and the other families. Kate talked about what took place after they were moved to the research facility at the Great Ormond Street hospital, “After about eight months we were moved to the research facility at Great Ormond Street. No more intensive care. Being in the actual research part of the hospital we were all a big family. All the parents know each other and we’re all on first name terms with nurses and doctors. Families get to know each other from different trials but we’re all in it together. James has many friends at the hospital and it’s so adorable. To see all the amazing children who haven't got a clue what an influence they are having on the world.” You know what? I live in this world and yes, I have been heavily influenced by what I see in these children. Even from an ocean and a continent away.
Everything would come together and the family would find the perfect place for meeting their needs. So after almost two years they would make the move back to Liverpool. James was enrolled in a special needs school and Kate says that it has worked wonders. They would not say goodbye completely to London though. Jamesy Boy is back at Great Ormond every two weeks. He puts smiles on everyone’s faces as they see him running down the halls of the hospital. If it wasn’t for the treatment that he is receiving this would not be possible. A big part of why this article has been written is to help tell the world that this treatment works. It also tells people about the importance of being diagnosed early. Awareness is key! Kate is so proud of her guy and all that he can do. Here is how she puts it, “James has been on the treatment coming up to 3 years now and we can actually say it's working. James can still see perfectly. He can walk unaided and runs extremely fast. He can speak over 80 words and has small conversations with understanding. He can sing and dance, and he can count to 15. He can also eat everything orally.”
More and more, Kate and her family are amazed by the strength of Jamesy Boy. The kids, in general, that are are being treated under this clinical trial are achieving amazing results. James’ memory is off the chart when it comes to kids with Battens. He constantly repeats the things that he hears his mum say and he remembers who people are. One example is his nurse Jodie. She had gone away for a maternity leave for over 12 months. James recognized her the instant that she returned. He inspires everyone that he meets and he is such a charming young fellow. Can you tell? People who know what Batten Disease is and who meet him for the first time are amazed. He is very inspiring!
The most important people that James inspires is his own family. Everything that he achieves, every day that he amazes, is another day of very special memories. Memories that would otherwise be replaced by different (although still very special) ones if this treatment was no longer available. We still need a cure to CLN2 and the other variants of Batten Disease. Until then, the clinical trial that James is undergoing is doing wonderful things. Each one of these kids is an individual treasure and they need to be treated as such. Yes, it is a fact that these kids have no idea how much they inspire the people that follow them. I know that I am greatly inspired, from an ocean and a continent away. In fact, this blog has become dedicated to telling their stories. It will continue to be that way for as long as I am allowed to do so. That is a heavy commitment but this is where my heart is. This is all because of kids like Jamesy Boy and parents like Kate. Thanks for taking the time,
Blogger and Advocate
“My life was completed that day. We had two girls and finally, my boy. My life was at the highest. Grade School hottie as my wife. He was gonna be my fishing, hunting and farming partner.”
A Warrior’s Daddy
When it comes to this blog and it’s content, what you have read is genuine when it comes to expressing my feelings. If you know me the way that I would like to be known, you will feel that my heart is in the right place when it comes to the families in the Batten community. I think that for the most part, the feedback has been positive and most people appreciate what has been done so far. Those that know me can see that I love my special needs son Benjamin to the moon and back. Yes, I guess it is true that perhaps I talk about him a bit much. He is, however, the center of our world at this house. Benjamin had genetic tests and an evaluation done on him when he was much younger. The doctor could not give him a diagnoses at the time but she felt that there was evidence that his disorder was degenerative. As he has gotten older, things like his mobility have been affected. He now needs help getting around for even the shortest distances. There are other issues that cause concern. Where I am going with this? My point is that he needs us in a big way for his daily care. I don’t ever want to think of him as a burden. He can be just a pure joy to be around and the bond between us is so very strong. He will be with us for as long as we are able and it may be a race to the finish line. Having Benjamin has increased my sensitivity to people with special needs and I think that lends itself to my sensitivity to those with Batten Disease. That, of course, came after I was first made aware.
My heart goes out to Batten families, and actually, you have a big piece of it. The journey that you take is one that is unique. I repeat details about how I feel concerning the Batten community routinely. It is normal to repeat things when you want to place greater emphasis on them. Is it not? So when I repeat my feelings about the course of events that take place in your journey, I do so because I feel they are noteworthy. The undying love that is shown. The initial trama of receiving the diagnosis and how it gives way to determination. Determination to do your very best for your child. It is easy for me to over simplify things because I have not been there. I can, however, make observations. You were told that your child has a fatal disease and what do you do? I know that initially you may have been totally despondent over what seems to be a hopeless situation. Your heart breaks but it is your deepest desire to do the very best that you can in providing the richest experiences for your young warrior. You provide everything that is needed to the best of your ability. I have done enough of these stories now and each one has turned out to be an amazing account of sacrificial love and care. It is not because of my words.
All I have done is to use the details that I was given in order to put them into words that tell the story. The story is yours, not mine. Awareness? Yes, but my greater purpose has been simply to share the details of what is faced and how the love that I often speak about is displayed. My foremost purpose has also been to honor the little warriors that face this battle so bravely. In their innocence, they press on while displaying a smile until they are no longer able to. They are just so resilient and I am amazed at how they are able to teach us about the indomitable human spirit. I am truly moved by each one’s story and I was truly blessed, over the course of last year, by all those who had placed their trust in me.
It is true that you can’t please everyone, all the time. It is also true that this blog is not for everyone. Several families have said “No thanks.” For others, the timing wasn’t appropriate and I completely understand. For others, they have been more than willing to be included and they have been very appreciative at the time of their story’s completion. Batten parents will express the gratitude before going back to the challenges that lie before them. Finally, there are those that have offered me a great deal of encouragement and support. This has happened through various means and sometimes it has just come through subtle comments and kind words. One such case is comments received from the Scarpetta family via Brad Scarpetta, the dad. His kind words have not gone unnoticed. He has had several nice things to say about what I do with the blog and so I thought that I would ask if he would allow me to tell his family’s story. He said yes! As I write this I am thinking, duh Greg! Maybe you should have asked sooner. I hope that my readers will have a sense of humor in regards to some of what is to follow. Sometimes laughter is the best medicine.
This story could have taken place in any town in America, but this particular one is taking place in a town within the state of Illinois. In a small town named Shirland to be exact. At the time of the last census that was taken the town had less than a thousand people. This sounds like a great place to hide. Maybe get married and raise a family. I am sure that there is space enough to enjoy all kinds of outdoor fun with the family that you dreamed about having as you made plans. As the years pass and time moves on, the memories that are made together stay with you for a lifetime. There are so many things that can be bought but the memories that come from being a family are priceless. These memories carry more value than anything money can buy.
Brad Scarpetta and Christina Jobson grew up in this little town and both attended Shirland Elementary all the way through the 8th grade. According to Brad Christina was one of the most popular kids, while he was not. She had Brad’s attention at an early age but it sounds like poor Brad went unnoticed by Christina. I feel your pain Brad! I wasn’t one of the popular ones either. As it is in most small towns, the kids follow each other from grade school to the local high school. Brad was one year behind Christina and so he literally followed her to Hononegah High School. I am sure that Brad still had his eye on Christina as according to him, she was “the hottest chic in their hometown.” Christina did everything in school activity wise. She played sports like basketball, volleyball, and she was a gymnast as well. Brad would tell me that she continued most of these into high school. Christina went on to attend Junior College and she earned her Associate's degree before going to full time employment. Brad, on the other hand, began work life right out of high school but he would later attend technical college for heating and air-conditioning plus refrigeration. This turned out to be an excellent move as he now works full time servicing equipment for large retail stores in the area. Christina would work for a single company for 14 years before taking a job as a school bus driver part-time. This so that she could have more time at home with her kids. She would later decide to return to full-time employment and she now works for the postal service. She has been there for three years and loves it. Oh, did I leave something out. I am getting ahead of myself here.
It was while they were in their mid-twenties that Brad and Christina started to date. Finally, that girl that caught Brad’s eye back in grammar school started to become interested in him. Brad told me that it was at the Boone County Fair that they dated for the first time. Gee, I wonder if it was Brad’s Harley Davidson that attracted Christina at first. Hmmm, maybe! It was in 2004 that Brad and Christina started to date on a regular basis. They both had a love for horseback riding and would frequently ride the trails together on horseback. Their fun in the country together had to include conversations about memories of their time as kids growing up. Having grown up in the same town and attending the same schools gave them much to remember and discuss. Their relationship continued to grow and a wedding date was set. They would get married in November of 2005. Memories of the past would soon be connected to new memories made in a life spent together. Memories, and yes, challenges unseen as they began their life together. Christina would bring her daughter Karly into the marriage and Brad would adopt Karly in 2006. As his relationship with Christina has continued to grow, so has the one that he has with Karly, his adopted daughter. That is special! Karly is now twenty years old!
Brad said that June the 1st of 2005 was an extremely happy day for him. That is the day that their daughter Cloe was born. She was so perfect and perfectly beautiful. The Scarpetta family was starting to really look just like that, a family! Brad would tell me that he had just taken a severance package from a company at the time that Cloe was born. This allowed him to spend everyday with baby Cloe for the first six months of her life. For him, it was an amazing experience. Brad had been working a lot of hours when he was younger but it was during this time that he attended school for refrigeration. Going to school and settling into a stable career would give him more time with his growing family. Christina’s own steady employment added to the family’s standard of living. They had two beautiful daughters and life was pretty good!
Sometimes, one gender runs predominant in a family. My wife’s sister Laurie and her husband Randy had three girls as they completed their family. I can remember Randy saying that he would have given anything to have had a son. He went on to say, that is except for one of his girls.
In our case, we had three boys as Rosie gave birth to Benjamin. I have said in a joking fashion before and I will say it again. We were trying for a girl but we got a Benjamin instead. Life has never been the same for us and it was soon to change again for the Scarpetta family as Brad again heard those famous words, “I am pregnant”. Brad told me that they decided as a couple that they would not seek to know the baby’s gender but would wait to be surprised. Surprised they would be! That was on April the 9th of 2007. That was the day that baby Brock was born. Just like his sister Cloe when she was born, he was perfect. He had all his fingers and toes and you guessed it! He was delivered brand new to the showroom floor complete with that new baby smell! Was this a big deal to Brad? Oh, you betcha it was. Here is how he would put it to me, “I’ll never forget the joy! When the doctor said it was a boy. I was overwhelmed! I love my two girls, but a little partner to get into mischief with was my dream come true!”
As I have been preparing to do this story, it has become apparent to me that Brad is a character. He loves to laugh and make other people laugh. I like that! Having little Brock in his life would allow him to mold his son into his image. That image included being a character and that would soon be the case! As Brock continued to grow he would bring mom and dad much joy. He would develop and reach his milestones on time. This would provide memories for their future together. Memories that are priceless and wonderful to remember. As Brock grew, he began to show off his charismatic personality to all that he was in contact with. Brad said that Brock developed a fondness for tormenting his sisters. Go figure! In concluding every sentence after a fight with the sisterhood, he would use the word duh! He used that word generously! You know Brock-man, as I was thinking about this, the question came to mind. What else are sisters good for other than tormenting? After all, they don’t like the things that us guys like and they don’t think like us. They are made of sugar and spice and all that stuff. You can have all of it girls! Yes, you torment them in the most endearing fashion that you can. Then as you grow up and sit and enjoy the holidays with family, you share in the memories of the time that you spent together. Those memories would include those of tormenting your sisters with much love and affection! Brock made sure to let people know that he was the smartest person in the house. Brad said that Brock loved making faces at people. No doubt, he loved using this tactic on his sisters. More memories.
In all seriousness, little Brock loves his family so much and that would include his sisters. As Brock continued to grow, he enjoyed time spent outdoors with his family. Brad said that he would take the kids fishing together all the time and the first fish that Brock caught was a Largemouth Bass. Brad would go on to say that the Bass was over half as big as Brock. That is no fish tale! Brock loved hunting for mushrooms, being on the the family tractor, and helping with chores. Just like their parents, both Cloe and Brock love horses. Growing up, they both had ponies. Cloe’s pony was named Princess and the name of Brock’s pony was Star. The family has always had livestock of some type. That would include horses and donkeys. Brad and Christina made sure that there were plenty of activities to help increase the bond as a family. It was also just plain fun! Brad said that they would travel to Wisconsin, when Brock was five years old, to attend Little Britches Rodeos. Brock rode Bare Bronc Ponies and according to his dad, he was pretty good at it. Perhaps we are talking about a future Rodeo star in Brock. This was taking place in 2012. According to Brock’s dad, there was one white pony that Brock would draw frequently. Brock got bucked off of that pony three different times, after which he referred to her as a mean pony. Sounds pretty mean to me too Brock-man! You can tell from all of this that this couple sought to do everything that they could do to give their children outlets for growth and learning. They were doing their best to make sure that the kids were experiencing life to the fullest.
With living in the same area that they were raised in, time together included time spent with their extended families. You remember that Brad talked about having someone to get into mischief with. You just know that Brock was going to pick up some of his dad’s character traits and it sounds like he was developing some of his own. According to Brad, Brock would have him rolling on the floor laughing at an early age. He had this to add, “He just did funny things all the time. He was gonna be the class clown and give me lots of gray hairs, Lol.”
Brad recalled one incident in particular that happened when they were driving with Brad’s dad and uncle. Brock was in the middle of the two of them in his car seat and he had control of the radio. Brad would go on to say that Brock loves country music. By my own admission, so do I! Anyhow, what took place is that Brock turned up the radio and when he did it happened really fast and it was turned up extremely loud. Brad reached for the radio quickly and turned it down as fast as he could. As this happened little Brock looked at Brad’s uncle Dennis and uttered a phrase starting with the word “Oh”. It ended with the choice word that starts with the letter “S”. Get it?
(Now this whole scene reminds me of a country song and this would not be the first time that talking to Brad would do that for me. He he!)
Brad said that he would have disciplined Brock for the use of that word but all the adults in the car were too caught up in their laughter to do anything about it!
Every family has their struggles but you have the feeling that up to this point, things were pretty good at the Scarpetta house. They had a solid work ethic and were receiving the benefits of that. Their emphasis on family was where everyone's should be. Life wasn’t perfect but it was really good. Looking forward, one would only expect that Brock’s life would hold a ton of promise for the future. All the time spent together only served to increase the bond that was felt, and this also is the way things should be. Nobody would have suspected the challenge that would lie ahead for this family. Things can sometimes change in an instant, and for the Scarpetta's they did. Brad remembers that it was on May the 1st of 2014 that Brock would have his first seizure. The seizure would be called a Febrile seizure which can be brought on by a change in body temperature. This assumption was due to the fact that Brock had a cold and a fever at the time. This, in fact, happened the night before Brad’s graduation from technical college at two o’clock in morning. Brock had a Grand-Mal seizure while sleeping with Brad and Christina. Brad had told me that this was extremely scary and who would argue that it would be. The family was at the hospital until 7am and no testing had been performed. Brad said that at the time, they had bought the false diagnoses “hook line and sinker”. No one can blame them for that. Why would they suspect anything different at that point? There was no way of knowing that they were entering the world of those that deal with a rare disease in their child. A disease that they had, most likely, never heard of.
As one would expect, the next seizure came and it happened on June the 26th of 2014 while Brock was watching cartoons with his mom. He was transported, again, by ambulance after having back to back Grand-Mal seizures. Brad said that they were persistent this time, believing that Brock’s body was trying to tell them something. This was taking place at Rockford Memorial Hospital in the city of Rockford. It was there that Brock would be kept for two days while the staff performed EEGs, brain scans, and various other tests. As might be expected, he was prescribed the medication Keppra for seizures but the pediatric Neurologist was too busy to see Brock right away. Instead, he was given an appointment for 3 months later. Brad’s response to this shows the type of father he is and it also shows the love that he has for Brock, “I flipped out. Nope not my child. Not 3 months. We gathered all the information from the hospital that Friday while still there. I called the UW of Madison Wisconsin. I cried and begged them to help my child. They complied and got him in the following Monday.”
Some of what happened next sounds all too familiar as I have written other stories. One in particular about a beautiful warrior princess named Montanna. She lives in Wisconsin, of course, with her very wonderful family. Anyhow, it was on Brock’s very first appointment at the university that the family conferred with a Pediatric Neurologist named Dr. Hsu. They were also seen by a Geneticist named Dr, Rice. They had both reviewed Brock’s file previous to the appointment and had questions and things that they wanted to test for. Batten Disease was mentioned but, at the time, the family didn’t think that was possible. By the end of 2014, a blood test for Batten Disease had come back negative. Doctor Rice wanted to have a skin biopsy performed just to make sure. Perhaps he had seen blood tests come back before with a negative reading, only to be proven wrong at a later date. One thing that is frustrating for many people in this country is having to deal with insurance companies who refuse to pay for testing that is needed. As Brad would put it, they had to play the “insurance game”. Do you think that the people order this type of testing for the fun of it? I wish this kind of thing didn’t happen. Brad and Christina had something happen in relationship to this. I’ll let you read what he said,
“In the meantime we visited Mayo Clinic. I’ll never forget this. It was a Wednesday night and I was taking Cloe to softball practice. It was February of 2015. At 7:30pm, I get a call from a Wisconsin area code. It startled me as I knew I wasn’t on call for work. It was Dr. Rice. He stated that his secretary had never submitted Brock’s paperwork because she “knew” insurance would deny it. I lost it on him. We needed them to deny it so we could appeal it. He asked this question and I’ll never ever forget the feeling. He asked me if I wanted to fire her?”
Initially, Brad wanted that to happen. After he had cooled down and thought through the situation, he felt different. People make mistakes and he felt that since she probably had a family to feed, he said no to her firing. In the end, they just got the whole process moving again. It took Unitedhealthcare a long time to approve as the testing was denied twice. That is so wrong! I can only imagine what the Scarpetta family was feeling as they had to wait during this period of time. It was in June of 2015 that the testing was finally performed. In July of the same year, they received the awful diagnosis that must have brought with it devastation. Brock in fact, had Batten Disease. He was at the beginning of his battle with the CLN8 variant of Battens. He is a warrior and a courageous champion. Not to mention, a darling of a young man!
The family was told that the only thing that would help is Gene Replacement Therapy. They have done fundraising for the therapy but it brings with it a price tag of 3.5 million dollars. I think that for the average family, this would seem like an unattainable amount of money and the treatment is not covered by private insurance. Most parents would do everything that they possibly can for their child no matter how big the challenge is. Some challenges are so big that all you can do is to do the very best that you can do and know for yourself that you did. No matter what, for now, Brock’s care is the most critical thing and this family is giving it their all. Christina is Brock’s main caretaker and she does this very lovingly, just like you would expect from a mom.
I love knowing how involved Brad is with his children. He is a great dad! Why would he not be though. This is what he longed for. This and the memories that come with raising a family. The priceless ones. In regards to moms, you have heard me say that there is no love like a mother’s love. I have also said that momma feelings are the best feelings. By that I mean, in part, that moms are often the best at putting their feelings into words. Christina started her comments to me like this, “It’s definitely been a roller coaster of emotions, from the day he was diagnosed in 2015 until now. The first few months were horrible. Who do we call? What do we do? How can we help Brock?”
The effects of Battens on Brock were gradual at first. According to Christina most of 2016 was pretty good and Brock was seizure free. However, by the end of 2016 Brock’s balance and walking began to decline. As time goes along, he is needing more and more help getting around so that he doesn’t fall. Brock’s mom says that there are days that he will take steps without assistance and that is considered to be an extremely good day. Christina said that his eyesight is gradually getting worse. First signs were that he was having problems seeing when the lights were dim or it was dark outside. Lately, as of 2017, his eyesight in the light and in the daytime is declining. They have their ups and downs with the disease and Brock’s appetite is hit or miss. Some days he eats non-stop and others he will just pick at his food. Mom now feeds Brock and he drinks using a straw. Christina bathes and dresses Brock and they now use pull-ups under his clothing. Concerning the seizures associated with Battens, she said that at one point they had gone two years without any seizure activity. Then, all of a sudden, there were four seizures in a week and a half. Christina recounted one experience with that and I will leave in her own words, “I remember two seizures when I was home alone with Brock. It was the worst feeling I’ve ever had, Helpless.”
Getting enough rest was difficult for this heartbroken momma. As she stated, “I didn’t sleep very well, often crying myself to sleep.” Brock battles like a warrior but the family has seen some big declines with him. Like with any Batten family, medicine changes take place almost routinely for Brock.
As I stated earlier, one of the continuing themes that I have seen in doing these stories has to do with the determination that is shown in the midst of very trying circumstances. Christina would confirm this in her comments. She stated how difficult that it is to stay strong while dealing with the heartache associated with Brock’s circumstances. She is her son’s primary caretaker. Like any child with special needs, the Batten warrior requires special care. I see what my special needs son requires in the way of help but for the Batten parent, I feel that the demands are greater in many respects. Christina takes Brock to all of his doctor and therapy appointments. I have no doubt that there are many that they attend. Brock is a battler! Christina said that Brock will decline to the point that the therapist will not know what to try next. Then, after a change in medication, he will bounce back a few days later. He will be his “spunky self”. You go Brock-man!
The level of devotion displayed by the Scarpetta’s, and others within the Batten community, to me is without equal. Yes, I know that there are other rare diseases that affect children. There is pediatric cancers for which more needs to be done. I believe that the parents of Batten warriors represent those in the other communities quite well. Moving past the heartache and just getting on with each day. Yes, I’ll say it again, the undying love that is shown. I know that Christina cherishes every single moment that she spends as Brock’s caretaker. Each little victory or achievement on any given day brings with it pure joy. As she put it to me, “I shed tears on the littlest things he does or a word he spits out.” This, I am sure, is true even when the fatigue sets in. It has to be tiring at times but I don’t think that she would want to give that responsibility to anyone else. She shares some of the experience in this way,
“I sing the star spangled banner to him every night. That was one of his favorite songs he loved to sing. If he’s having a rough morning, we sing and dance in the kitchen. I do anything to make him laugh and smile. There are nights where I just watch him sleep and kiss his forehead until I can’t keep my eyes open any longer. He was up all night one time and wouldn’t stop crying. I drove him around the countryside and we listened to music to calm him down and make him smile. I take him everywhere I go. Shopping movies, etc. Some trips are better than others.”
Christina stated that she doesn’t like it when people stare or make comments. We get plenty of stares with Benjamin when out in public. You can take a quick glance but staring shows that you are more handicapped than you may be aware of. Christina is not shy about voicing her opinion when people exhibit this type of behavior. She is, after all, the mother of a warrior and a warrior mom! She said that she tries not to think about the toll that the disease is taking on her son’s health. Like all warrior moms, she places one foot in front of the other each day and she gives it her all. I know from following this family that Brad can’t wait to arrive home each day to spend time with the son he dreamed of having. His little warrior and champion. He lives to hear the laughter that comes out of Brock as they horseplay together in the evening. He roughes his boy up as much as he can get away with in the most adoring fashion. This family rocks the house!
Moving forward there will be big challenges and some more heartbreaks. The Scarpetta family will do absolutely everything that can be done to help their son have maximum success and the greatest longevity that can be found. Just like with any family, there will be bunches of sweet memories. Ones that are priceless. Would it not be priceless to know that Batten Disease was easily treatable? That the science is there and clinical trials are over and successful? That people were well educated on it and a diagnosis was always determined quickly? Insurance companies paid the bills, and on and on? I know that I am a bit of a dreamer.
The message in this video is priceless to me. Because of the challenges that are faced, Brock’s story is even more priceless. No matter what the future holds, Brock’s life will impact the lives of his family members like no one else’s. These kids teach us things about ourselves that we never would have known and they change our perspective on many counts. Their stories have changed me! Brock-man you are a warrior of the most adorable kind. Please battle on and kick Batten Disease square in the teeth to the best of your ability!
Scarpetta family, thank you so much for letting me tell your story which is among the best because that is what you are!
Like father like son as the saying goes. I am a firm believer that people need to laugh a little or life often gets to them. In my case, I like to laugh a lot and Brad is just the guy to help me with that. He gave me a few funny things that have happened with Brock while in his presence. I’ll close with one of the real cute ones. As told by his dad, this happened when Brock was five years old:
“We would be in Walmart. I’d put him in the cart and talk to him. I used to ask him about hot chics at school and wherever he was that day. As we are headed to the checkout here comes, well, hot chics. He hollers, ‘Dad look at those hot chics.’ Pretty sure I power walked looking at the ground while they giggled and laughed, talking about how cute Brock was lol”
Brad then went on to finish the story by acknowledging a fact. That is that there are things that he and Brock have missed out on because of the disease. “Greg he was a lot of fun. I miss them days. I miss seeing what else he was gonna come up with.” There are some memories that will not get made because of Batten Disease and there are others that will never be taken away. There will be other memories made and you can bet on one thing. Those are the ones that will be priceless!
Families like this are why I am doing what I do! That is why my time and energy away from my job is spent here and it will continue to be, for as long as you will have me. Thanks so much!
Blogger and Advocate
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:
The Giggle Box
Is it that face or is it the catchy title? It is one of the more widely viewed stories in this blog. Little Samantha represents well, those who battle Batten disease so sweetly. Always remembered because she would be impossible to forget!
How Did it all begin?
People do read it and I am glad that they do! How did I get started with all of this and why do I do what I do? I have done some slight upgrades and so here it is!
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
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