“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
My faraway friend, Jessica Montville, became a member of the Batten community when her beautiful little daughter Nora Skye was diagnosed with the disease. This happened after she began experiencing symptoms. Jessica’s search for answers was not as long and exhausting as many of the others were although the answer that she received was nonetheless very distressing. I love her daughter’s name! Nora Skye is a name that could have easily been taken by an actress who wanted to be known as someone more than of the ordinary. It is the type of name that would endure through time and would be associated with someone who possessed great beauty of a rare kind. The name will always be remembered because of who it belongs to!
There have been several blog stories that I have done in which I have drawn a comparison between my own experience as a father of a special needs child and one who has a child with Batten Disease. In the truest sense, there is no comparison. The knowledge of the fact that a child has a disease for which there is no cure brings with it circumstances that stand alone. The only reason that I have written in this way is to try to relate my own experience as best that I can in an attempt to try and understand what those in the Batten Community go through. I know that my attempt falls short of this.There are of course, other types of little warriors out there that deserve to have their stories told. My son Benjamin has a seizure disorder that is, for the most part, controlled by medication. We suspect that he still suffers from absence seizures in times when things are out of order. His mobility, among other things, has become an issue. I have mentioned it in brief before that there was a time that Benjamin experienced a bout with aspiration pneumonia.
Benjamin came down with the stomach flu or a virus when he was seven years old. Without giving too many details I will tell you that he aspirated into his lungs and not just a little. Whereas this was a one time event for Benjamin it was a very serious one. I know that young children with Batten Disease often experience aspiration pneumonia and it is always a very serious thing. When this happened to Benjamin, we called 911 and our son was taken by ambulance to the nearest hospital. He was almost immediately Life Flighted to a hospital that had the type of equipment needed to treat him. This consisted mainly of a special type of ventilator. Within a few days, he was transferred to a to another facility and put on a less aggressive machine. After he had settled into the first hospital, we consulted with the physician that was treating him. The doctor said that Benjamin couldn’t be any sicker than he was at that time. Of course, he would recover and I am so glad that he did. Where am I headed with all of this?
During the time that we were at that first hospital, we received a pastoral visit. He stopped by to offer support and to check on us and Benjamin. It has been a long time now but I can remember how he had commented about how upset or alarmed we must have been by what was going on. It was a fact that, prior to this time, we had already spent a lot of time with our son at the doctors and at hospitals for a variety of reasons. So our response had apparently taken him by surprise as we might have appeared over confident in our own ability to handle the situation on an emotional level. Perhaps our attitude seemed a little flippant or out of character. It was like if we were saying, “don’t worry, we’ve got this.” I am sure that if things had become worse and we were to have lost Benjamin, we would have been devastated. I think in hindsight, that perhaps we were a little bit naive. For the child with Batten Disease, if aspiration happens, it comes in a time when the disease has progressed into a later stage and the damage can be irreversible. Something like aspiration comes at a time when the family has been through the earlier stages of the disease and all the devastating circumstances that go with it. There is no way that a Batten parent can posses a flippant or careless attitude when this happens, and they never do.
I have often talked about the bond that has developed between me and Benjamin as time has gone by. It has been mentioned before that I actually have three sons and that Benjamin is the youngest. Even if I haven’t always demonstrated it by my actions, I would like to think that I love all three of my son’s equally. In the case of Benjamin, things are a little bit different though. Both his brothers are on their own and are self sufficient, while Benjamin has been under our care his entire life. All the time that we have spent together has, of course, created a special bond. How much more intense is the bond between a Batten warrior and the family? The bond that is created as a result of giving everything that is required for a Batten warrior’s care cannot be broken. This is my observation. Such is the case with Jessica and Nora. The following is a brief account of their story and some of it comes from a previous blog that I did. I felt that Nora deserved a little better than I had previously done for her in February of last year. I wanted to give a little more detail and make it a little bit sweeter because she more than deserves that from me. Here goes,
Baby Nora must have been something to behold as her parents held her in their arms. They were so happy to see her after welcoming her into this world. She was born at Heywood Hospital in Gardner Massachusetts on April 21, 2005. There was only one thing keeping her parents from looking ahead to all that she could accomplish in her life. That was the fact that they were mesmerized by her tiny and most perfect appearance. She was flawless and beautiful. Nora Skye had all her tiny fingers and toes and she was covered in brand new baby skin. It was no small thing that this baby girl had made her entrance into the world as her mother, Jessica, was having problems conceiving. After consulting with doctors and after much time and difficulty the purest of perfection was delivered.
There is no bond that exists like that which is between a mother and her child. Especially between a mother and her daughter. As little Nora Skye continued to grow she captivated her family by all the the very cute and precious things that they observed in her. They had no reason to believe anything other than that she would fulfill all of the dreams that they had for her. Certainly she would make her own decision one day for her future but her family thought nothing other than that her future would be bright. There was no way that Jessica could know what would lie ahead for them. I have now heard the testimonies and comments made by many parents that are either facing the daily struggle of caring for a child that has a rare disease or one who has lost a child to such. The absolute heartache that has been told to me by the now many is heartbreaking to the reader if it is allowed to sink in. I feel that I am personally drawn into these families stories as I write and I do my best to empathize with what they face. My emotions often get the best of me as I think about each one.
Jessica had to be treasuring every moment of little Nora's life as she watched her grow. I am sure that the strong bond between the two of them continued to develop as Nora reached all the normal milestones that each parent looks forward to seeing their child achieve. Each new day would bring another memory to be treasured as the entire family continued to look forward to the future. It wasn’t until the age of three that a sign of what was to come appeared. Nora told her mommy that she could not see. This would be alarming to any parent. It should also be noted here that Nora as a baby experienced tremors and that Jessica was told that Nora was merely vitamin deficient at that time. She did not know that what her baby was experiencing is a piece of a puzzle that no parent would want to have to put together.
The truth, however, would soon be found out as changes started to take place. Nora was taken to her primary care doctor who felt she was fine. It was while she was at a neurology appointment that Jessica was advised that she should have Nora’s eyes checked and that was not all. The most notable, and certainly the most alarming, concern was that Nora began to have seizures. Not just one or two, but more than could be counted accurately. This would lead to an early diagnosis of Uncontrolled Seizures. Like a lot of parents that go through such an experience, the exact day becomes etched in a person's memory. Jessica remembers that day which she will never forget. June the 25th, 2009. You’ve heard the stories before about how a parent will go on an exhausting search for answers, however for Jessica, that would not be the case. The Neurologists that were consulted would be quick to determine what was going on with precious Nora Skye. The early diagnoses would eventually give way to the news that Nora had Late Infantile Batten Disease (CLN2). Jessica was informed that her daughter has a disease that is fatal and for which there is no cure. She was all of a sudden faced with the knowledge that her child could be gone as early as age 6. The variant of Batten Disease that Nora had would bring with it a life expectancy of no more than twelve years at the time she was diagnosed.
What would you do if this was your child? How would you respond? Jessica was seized by emotion after receiving the diagnosis. Nora was her firstborn child and one that she had dreamed about having. She came into this world through a fair amount of difficulty and she was Jessica’s darling Nora Skye. Jessica would tell me that she spent the first day overwhelmed and in tears after receiving the news. Those emotions, however, would give way to determination. That determination would drive her to do whatever she had to do to save her daughter. Jessica would tell me that her thinking at that moment in time was that she would save Nora, even if it killed her while doing so.
As somebody that doesn't know Jessica and her family really well, I can only use the examples of others whose stories I have heard in understanding what the news they received did to them at that time. How devastated they must have been to know that their most perfect little person would go through all the stages of a most dreadful childhood disease such as Battens. My heart aches for any parent that receives this kind of news. Anybody that is a parent of a Special Needs child understands that strength of the bond that develops as a result of their child's dependency on them. However, for the parent of a child that has a rare and incurable disease it becomes even stronger. I am thinking that the constant care for the child's very survival on a daily basis and the time spent by their side only serves to increase the bond exponentially. We are not at all members of the Batten Disease community, but I love my special needs son Benjamin so much! I have to tell all of you that are parents of a rare child how much I respect you. The love and respect increases with each story that I hear or read about. To know that there is a clock that is ticking and that your time is limited. To know the eventual outcome and to make every moment together memorable. The determination to fight for your child in every way possible. There is nothing to compare it with. When it comes to Nora and Jessica, it needs to be said that Jessica battled for her daughter in every way that she could. If you were a doctor or a nurse, a teacher or a bus driver, you better have had your act together when it came to your care for Nora. Professionals of all kinds needed to have their facts straight or this momma bear would see through everything.
It was apparent to those who followed the journey of Nora Skye that emotions often ran high. This was due to all of the frustrating circumstance that surrounded mother and child as they sought to fight off the monster that is Batten Disease. Nora Skye often led a lonely existence as other children and adults avoided her out of pure ignorance. These people do not understand what a tremendous gift that each one of these children are to us. Each carries with them the title Prince or Princess. They are rock stars, each and everyone. To stand in service to them is indeed a great privilege that should never be taken for granted. They are little warriors in the truest sense. Nora Skye was and forever will be beautiful to those who followed her journey. I know that she is to this guy. She would understand the love that those who knew her best had for her.
There was never any doubt! Nora would go through all of the decline that one would expect with Late Infantile Battens. This included loss of most all of her abilities, seizures, and lung congestion. But you know what? She battled hard and she did so with a smile on her sweet face for most of her life. Jessica tells me that she battled through the later stage of Battens for many years. She was a fighter and Jessica was a warrior mom because she loved the daughter of her dreams. Jessica did everything possible to help Nora fight. This would include the use of Cannabis derivatives for the control of seizures. To this day, Jessica is an advocate for their use and she is an expert on the subject. Even with the physical decline, Nora’s mind stayed sharp for a very long time. Jessica would tell me that everytime Nora was tested, her mental capacity was way above average.
Sometimes I think about what it would be like to no longer have Benjamin in our house. Benjamin always has his little space or room no matter where we live. His room is cluttered with all his favorite things. At the center of it is his computer which he uses to play his John Madden football game. He doesn't play it like a normal 27 year old person would but he does so to his satisfaction and pleasure. He is surrounded by all his favorite stuff. Magazines and books with pictures. Toy cars and school buses. New England Patriot signs, football cards, and stuffed animals he has received over the years. What would it be like if he was missing and his room was still filled with this stuff. More days than not, I walk into his room after returning from a long day at work. I will wrap both arms around him and put my face right up next to his. I tell him things like what an awesome person he is and how lucky I am to be his dad. I like to kiss him on the bridge of his nose and then back away slightly as I continue to talk to him. Most of the time he will push his face close to mine as if to tell me to give him another. What if he was no longer there? What if I couldn't wrap my arms around him anymore?
What would it be like to be the parent of a child with a rare incurable disease. Your days are filled with the constant care of your child. As the disease progresses the level of care needed increases. The trips to the doctors and the stays at the hospital increase. The level of home care and procedures increases but you do it because of your love for your child. Simple things like bathing become increasingly more difficult. The number of procedures required on a daily basis and the medications needed for the child's very survival go up. Your house may become more cluttered with medical equipment and perhaps the gifts from friends and well wishers fill your child's room. As a result, the bond gets stronger as you battle through each day. Your child is a little warrior and you warrior on as well. And then....some have had to say goodbye, while others have precious warriors that continue to battle.
I am so glad that progress is being made but much more needs to be done. Many families that are dealing with CLN2 are benefitting from Enzyme Replacement Therapy. I am so happy that this became available to them. These kids are just amazing to me!
Nora Skye Montville completed her journey on February the 2nd of 2017.
It was well over three thousand miles away from Jessica’s home that one of my favorite stories took place. This in Plymouth of the United Kingdom. It is the story of Elsie Clark and her grandson Marshall. Elsie was very lovingly given the care of Marshall by his awesome mum, Lara Clark, because of the special bond between Marshall and Elsie. This and also because of the qualifications that Elsie had as nurse by profession. She left her job in order to care for Marshall full time. The entire story, called No Ordinary Love, is so incredibly sweet and it will always be an out of the ordinary kind of beautiful to me. What is most special is how Elsie spoke about what Marshall had waiting for him as he completed his journey. She spoke of the freedom and pure enjoyment that he would experience once he had finished his battle. The words that Elsie spoke about Marshall as he was soon to gain his reward will always stay with me. I would like to think that her mention of things like strawberries and playground slides are just the tip of the iceberg for these kids. Both Elsie and Jessica exemplify the love relationship that exists between Batten warriors and their closest caregivers. This is especially true when we are talking about their closest of family members. That is a special bond that cannot be equaled and it is one that never fades away. There is no love like that which exists between a mother and her child. And yes, this same kind of love can exist between a grandmum and her most suave little grandson. Nora Skye, I know just where you are.
As I was preparing to post this story, I received word from a friend that little Brianna Skriver had gained her wings. Her family has a page for her on Facebook called Friends of Brianna. As I looked at my news feeds I saw many people reacting to the news of her loss. I also saw that some of the other Batten mom’s had the opportunity to meet Brianna and her mom. No doubt, it was at a Batten conference. How beautiful is Brianna! One very special video was taken of Dixie Bergeron’s Jacoby as he met Brianna. Oh my gosh! Another very special pic is one of Jessica with Brianna and her mom Tammy. The love that these families have for each other is evident. Only they can truly understand what they go through. It is true, however, that this outsider does the best that he can to understand. I hate Batten Disease but I love the families that battle it. Fly high Brianna!
It is the natural outcome that this blog would spread awareness. Anytime you put something on the world wide web, people will see it. That, however, was never my original thought. My original intent was to tell the stories of love and sacrifice that come as a result of caring for these kids. Their lives are truly special to me. They change people. They will have the biggest effect on those around them even when their lives are cut short. To me, they have the awe factor like none other. The love that exists between Nora and her momma will never fade away. Jessica would go on to have four additional children within Nora’s lifetime. Wow! There is no doubt in my mind that each one is special to Jessica, each in their own little way. The bond between her and Nora is different though. The strength of it cannot be compared to any other. Nor should it be. Batten families may think that they are just regular people that are trying to survive the day. You may have been pretty regular as the journey started, however, you became anything but that along the way. At least you became that to me. You are extraordinary!
I hope that I did good for you this time baby girl!. Nora Skye, you ARE beautiful and I know just where you are!
Blogger, Advocate, and
Highlighting a previous blog that might interest you:
Journey Unexpected - The Rich Family
One child in a family with a rare disease is a challenge. What if there is two? Heartbreak multiplied you pick up the pieces and will yourself to do your very best. A love that is profound and a bond that is stronger than any other is formed. Two little beauties in one family is two, too many but you seek to make a difference! A journey unexpected...