The Clouds Above - Silas’ Story

Handsome Silas!

“Being a Batten mom has made me appreciate the small things in life and to live in the moment as much as possible. My love for Silas is unlike any other. He made me a Mama and the second I saw his beautiful brown eyes looking up at me I was a changed person. He gave me a love I never knew existed.”

    ~Melissa Nissen~
Silas’ Mom and Advocate

I began to write because of one little girl who battled. It’s not like I was the only person affected by her story but I was affected greatly. She changed me, and she changed my way of thinking. I had raised a son with special needs who remained with us at the time and the love that I had for him would naturally lend itself to the kids that battle Batten. However, I first had to have my eyes opened to their plight. There were all of the usual distractions in life but many of them would be set to the side once I discovered these children and what they battled. I came to be aware of children with rare and fatal diseases through social media and in a roundabout kind of way. Once I did become aware, I became passionate about the cause of these kids. I began to write through a series of circumstances and I’ve continued to this day. I really wanted to do this as a way to honor these families and especially, these dear children who are so resilient and special. I wondered what it was like to look into their eyes, knowing all that lay in front of them as they battled. What would it be like to walk alongside these kids as they battle the biggest monster possible for a small child (or a growing teen in some cases)? I thought about their plight and it raised so many questions concerning what a family would go through. Why would you bother to take the time to read these stories? To some, they may seem to be all the same. A child has a fatal disease and the families are left with the tragic circumstances that come with this. It’s sad and heart wrenching, the disease is fatal and the outcome is expected to always be the same. 
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Some people won’t give it much thought once they are aware of such a thing. Others will give the subject a little more time but then their thoughts will be consumed by the events of the day. However, there are people who will be moved by the stories of the kids that battle just as I was at the beginning. The science behind how the cells of the human body function is fascinating. However, it is devastating when something like a lysosomal storage disorder is present. Unknown by the parents at the time that their child is born, they experience all of the joy associated with the event. Milestones are reached and great things are anticipated for their child. However, problems in the child’s health and behavior can then begin to show themselves, leaving the parents to wonder. What is it that is taking place in their son or daughter? What follows is so disheartening to anyone that has a heart of compassion. The news brings shock and great dismay and life will never be the same going forward. What ensues is a life filled with a mixture of emotions and a daily struggle to live a normal existence. The emotions are ones that can only be felt by those that are left with this experience. That is the experience of raising such a child as one that battles Batten disease. There is no way that I can adequately describe what the family of a rare child goes through but I can tell you what it is that I see in them. I’m speaking now about the love relationship that is formed as the battle continues. I’ve stated some of what is the battle that is Batten in previous stories and here is some from one of those. 

It goes as follows, “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.” More of what I shared from the same story reads like this, “Undying love in the face of adversity. I understand that I am on the outside looking in and my perspective often reflects that. How difficult is it to move forward each day, knowing the eventual outcome? Deciding that you are going to do your very best in the worst situation, heartbroken and yet, resolute …. The details of the journey can be extremely moving. This, because those details express the outpouring of love towards these young warriors in an extraordinary way.” I was so moved by the details and these are the things that stirred my own emotions. At times, it was because of the way that I chose to place the words together in print. Maybe it was the thoughts behind those words. Either way, I was moved to tears at times. Sometimes, this would take place in the worst places. My passion for writing these stories was continuously fed by this. The bond that I share with my own son that has special needs is so strong and I think about him often while I’m away from him. How much stronger is the bond that develops between parents and their son or daughter with Batten disease. Wherever a family is within their own journey, their story is a love story in the making. 

That love is one that is like none other. That is, even if this is not visible to parents due to the struggles that they face. I see it from a distance and I know that this is true! Most of the stories begin in the same way, two people meet and fall in love. This story is no exception as it also has a charming kind of beginning. Anthony Nissen and Melissa Moore both had goals for their careers. Tony wanted to be an independent contractor and owner of his own business. Melissa was a beautician who also wanted to be an independent business owner. That is what each of them had in mind for their individual futures as both of them were in their early 30s when they met. There are usually a set of circumstances related to two people meeting and that would be the case for Tony and Melissa. The two of them would meet at a 4th of July party in 2015 after Melissa was invited by a friend who was going to the event. The host of the party was Tony’s cousin and Tony would also attend the party. He and Melissa began to talk soon after Tony got to the party and they would spend most of the night together. Tony had been drawn immediately to Melissa’s beauty and Melissa also found Tony to be an attractive person. The party would end and they would say goodbye but things would continue to develop between the two of them. It would be just a few days later that Melissa would hear from Tony again as he contacted her through Facebook Messenger. Neither of them would be in a rush to take things to another level but they did talk on and off for a few months. 

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A Future Together
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Attractive couple for sure!

Melissa said that on one occasion, Tony showed up at her door, asking to take her out to lunch. They had a great time together and the connection between the two of them continued to grow. They both had common goals, hopes, and dreams for the future plus their personalities meshed well together. Melissa also thought about the fact that Tony had a good family background and he was ambitious in regards to his profession as a construction contractor. Tony also made Melissa laugh and they had fun together. On another occasion, in the course of time, Tony had invited Melissa to stop by at his cousin’s wedding that he was attending. Melissa took him up on the invite but her car ended up breaking down on the way. This was on the side of the freeway, no less. Tony had offered to come to Melissa’s rescue but she had AAA on the way as he offered his assistance. She ended up going to that wedding activities later that night and they have never been apart from that time forward. They must have known as time moved forward that spending their lives together was in the making. Both of them were busy working towards their goals vocationally. Tony with his contracting business and Melissa as an independent hair stylist. They were both on their way to reaching their objectives when Tony moved in with Melissa. The love that they had for one another was real and so, they were both excited when Melissa found out that she was going to have a baby. As she put it to me, “they were both excited and scared”.

Tony said that he was told the news over the phone and that he could tell Melissa was excited. This was because she couldn’t stop calling him! The couple would also find out that Melissa was carrying a boy. Tony himself said that the feeling of knowing that he was going to be a father was “amazing”. Even more, he shared about himself, “It was like a ball of energy inside me that wanted to explode out. I was extremely happy to know he was a boy, the same as any man would be.” Tony compared it to the pride shown by the Lion father over his son in the animated movie named the “Lion King”. The days during Melissa’s first pregnancy moved along without complication as life continued. There was anxious expectation over the knowledge that Tony and Melissa would soon be parents, even with normal life taking place all around them. The only issue to occur as she carried their soon to be born son was that Melissa would develop preeclampsia. Also known as Toxemia, the condition includes issues with high blood pressure and swelling in the mom’s legs, hands, and feet. The condition can be quite serious if not dealt with properly and this would be a concern with Melissa’s doctors as the date of baby’s delivery approached. Melissa was due to deliver on December the 16th of 2017 and the big occasion would take place at West Allis Memorial Hospital in Milwaukee, Wisconsin. She would spend about 20 hours in labor without a lot to show for it and the staff at the hospital was concerned about her blood pressure being so high.

It therefore was agreed upon by all that they would bring the baby into the world by doing an emergency C-section (or cesarean delivery). The result would be that Melissa would give birth to a perfectly shaped, beautiful Baby boy. He would make his entrance into the world at 7:19 pm on December the 17th of 2017. Weighing in at 7 pounds and 9 ounces, Tony and Melissa’s newborn son was given the name Silas Anthony! Tony would be the first to hold baby Silas due to Melissa’s need to recover from the C-section along with the complications of the preeclampsia. The staff also had to administer a lot of medication in the course of the delivery. Due to his strong family background, having a son of his own was a big deal to Tony. No wonder he described their meeting like this, “Holding Silas for the first time was the most amazing moment of my life.” Tony would do all of the firsts for his infant son. He held him first and was the one that did Silas’ first diaper change. Melissa would hold her newborn son after she was comfortable enough to do so. Once she did, she couldn’t let go of him. Melissa described the experience so wonderfully, “I was so in love, he was so cute and so aware of everything around him.” Melissa added, “He was our everything. I never knew a love that could happen so quickly and be so strong. We were so happy.” Many have experienced a similar type of love but others can only hope to at some point. Tony and Melissa had found each other in life and now, they had this precious new life to experience together.
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Life was beautiful and Silas would continue to grow as they all  grew in their love for one another. This little man was the source of a lot of pride and joy as his family watched him grow. Silas was meeting all of his milestones and his parents expected nothing but good things for his future development. Just as one would expect, Tony and Silas were growing into best friends as Tony’s little man watched everything that his daddy did. I’m sure that he was a mama's boy too when the situation called for it. I’m just saying…. Tony loved Silas but he also had something else on his mind. That was the other person in his life, that being Melissa. He knew without a doubt that he wanted to spend his whole life with her and that he wanted Melissa to be his wife. A year and a half had passed since the birth of Silas and as a family, they had planned a trip to Panama City beach in Florida. Along with Tony, Melissa, and Silas was Tony’s sister and brother-in-law. Tony wanted to use this setting to ask Melissa to marry him and he had a plan for springing a marriage proposal on her. This would involve little Silas. Melissa shared what took place, “When we got to the beach, I noticed Silas had something in his shorts. I was like what the heck is that? I pulled a ring box out of his shorts and it was empty. I looked at Tony, he was so nervous. (It was so cute) He got down on one knee and asked me to marry him. I, of course, said yes!” Tony did have the ring in his hand as Melissa stood there smiling. She was both shocked and extremely happy and there would be more good things to follow. There would also be future heartache. 

A Family of Four

Adorable!

It would be about a month after Tony’s proposal that Melissa would find out that she was pregnant with their 2nd child. Her next pregnancy would again go well and would take place without a lot of complication. This time, Melissa would give birth to a beautiful baby girl. You could underline the word beautiful because that was truly the right description for the new addition to their family. They would give her the name Summer Raine and the big day would take place on February the 20th of 2020. This was only weeks before the world shut down because of the pandemic. In Melissa’s own words, “Silas gained a new best friend and so did I.” There are different opinions concerning what the perfect size of family is. For two career minded people in their 30s, two children seemed to be the right amount. Tony and Melissa now had both a boy and a girl to call their own. Their attention would be evenly divided between two children and they could afford to give them everything they needed. Kids often spell the word love like this, t-i-m-e. They could give them each the time that they needed while keeping up with life in general. Tony and Melissa both agreed that the decision to hold the number of children to two should be made permanent. And so, it was. Silas continued to grow and achieve each milestone on time as Melissa carried her baby. He was running and jumping and doing normal kid things as a toddler. This was while the bond between him and his parents continued to grow. The only concern that Silas’ parents had concerning their son was that he was late to begin talking. That had been taking place just before baby Summer was born. 

It wasn’t a huge concern for Tony and Melissa because some kids just start to talk later than others. They did want to address the situation and have Silas tested, so Melissa took him to have that done at 18 months of age. At that time, the people running the test said that Silas was fine but that he should be brought back for further evaluation at age two. There really was no need to be concerned though. After all, Silas was strong physically. He had been walking by 9 months of age and running just a month after that. Silas had always been a happy little guy. He was full of laughter and his future continued to look bright. His behavior was also normal for a little boy his age, just needing a little correction here and there. Silas started to receive speech therapy at 2 years of age but it was done virtually because of Covid restrictions. Melissa found that to not be much help and so she began to look at other options. Silas would begin both speech and occupational therapy soon after that. His speech was still the biggest concern in relation to his development. Something like a speech delay is something that can be dealt with but something else would occur when Silas was just beyond 3 years of age. This event occurred one day when Silas was in time out for bad behavior. As Melissa went to talk to Silas about what he had done wrong, she noticed that he was lying in bed, just staring at the wall. It was as if he was ignoring her. Melissa sent Tony to talk with Silas. As he approached his son, he touched his arm and could feel that he was making jerking motions. 

This was alarming as Tony took Silas to the couch where he then started to go into full body jerks. Their son was having a seizure. This would go on for a few minutes and then the seizure would stop. Tony and Melissa took Silas to Children’s Hospital immediately where he would be monitored for a time before being released. While there, they were told only that seizures can happen in young children but that they usually grow out of them. This was not an isolated event and another seizure would take place just four days later. Melissa would take Silas back to Children’s Hospital, traveling so quickly that it felt as if they were there in only a minute. Again, Silas would be monitored for additional seizures. This time while there, Melissa would talk to a neurologist. She was impressed with her immediately as the doctor wanted to run some tests on Silas. They would perform an EEG and an MRI on him and both tests came back as being normal. The neurologist would place Silas on the medication Keppra for control of his seizures and she would also place an order for genetic testing to be performed. Whereas genetic work was once done as almost a last resort, it is now ordered more quickly in many cases. Could Silas’ seizures be related to a genetic disorder? This doctor was on top of the situation and she wanted to know what was happening with Silas. It would only be about a month after this, on March the 11th of 2021, that Melissa would receive a call. The results of the genetic testing were in and the neurologist wanted to discuss the findings with Tony and Melissa.

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It was during this time that Tony, Melissa, and their two children had been living with Tony’s parents. It was a normal kind of work day for the two of them. Tony was working on a renovation project at their family home and Melissa had clients that day but they also had the neurology appointment to attend. Silas had a seizure disorder of some type and it must have been expected that there was a solution to his health issue. However, what they would find out soon that day was quite different from anything that was expected. Melissa said that they both had “walked into the appointment so blind.” They were both blind as to what was about to be revealed to them. What was on their mind at the time they met with the doctor was how the Keppra was affecting Silas’ behavior. Many parents with children on Keppra see behavioral issues associated with its use. However, the doctor had something else on her mind as she quickly asked if Silas was still having seizures. She just stated that he should stay on the medication when Melissa answered “no” to her question. Perplexed over the doctor’s response, Tony and Melissa continued to listen. The doctor continued to say that something had come up on Silas’ genetic testing and as she said this, you could see the tears starting to form in her eyes. She had news that no doctor wants to relay to a child’s parents. The neurologist went on to tell Tony and Melissa that Silas had Batten disease. Melissa said with that, her “heart dropped” and it must have shattered into pieces. There is a part to this story that is yet untold. This wretched disease actually had a history of existing in Tony’s family.
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Never Imagined

Warrior prince!

Melissa had been at Tony’s house when they had first begun dating and had seen a funeral card on his refrigerator. This caught her attention. That card had on it the image of a young boy who was the child of one of Tony’s cousin’s. This cousin, in fact, had lost two sons to Batten disease. Melissa of course, hadn’t heard of the disease at that time and she knew none of the details of it. Melissa said that she was broken-hearted when Tony explained to her what had happened to these two children. There was no way for her to know at that time that Batten could potentially be part of their future. She went numb when given the news that her own son Silas had this disease. For Tony’s part, he never could have imagined that something like this was possible. In his own words, “I never could have imagined or even have had it cross my mind that Silas would have Batten disease.” Tony added, “…. When the doctor told us Silas had Batten disease I was mortified, knowing what my cousin went through with his boy's. It's a feeling of sad loss, as if I have already lost him.” Melissa, sitting there in disbelief, asked Tony if, in fact, that is how his cousin had lost his two sons. She then turned back to the doctor and asked if children do die from this disease and if so, at what age. With that, this very caring doctor explained tearfully that kids do die from Batten disease. She went on to say it usually happens at the middle school age of children. Melissa said that at that point, she just “checked out”. She could hear everything around her but all she could do is stare out the window at the construction taking place outside of the hospital.

From there, Tony continued the conversation with the doctor. She gave him information about another hospital that was doing treatments for CLN2 Batten disease. It was only about an hour and a half hour away from Tony and Melissa’s house. This was enzyme replacement therapy. It could be performed to replace an important enzyme that was missing in Silas’ body. This was what was the cause of the speech issues and more so, the seizures that he was having. There would be more challenges to follow but this therapy, while not a cure, would help give Silas a better quality of life until a cure was found. Both Tony and Melissa got up from the appointment and left. Understandably, they did so as if they were in a foggy mixture of thoughts and emotions. Not knowing what to do, they both went back to work. Melissa handled her first client but then cancelled the rest of her day. She went home to her in-laws house and found that Tony had left work to return home also. Together, they just cried and hugged their little boy who they now knew to be a little Batten warrior. How was this possible? Both Tony and Melissa met each other and fell in love and it all seemed so perfect. They didn’t know, nor would they even consider the fact that each of them could be a carrier of the same mutated gene that causes the CLN2 variant of Batten disease. This is all so difficult for me to wrap my mind around. The disease can’t be a part of a child's genetic make-up unless both parents are carriers. It seems that this part of the story is always the same. Along with the news that your child has a condition that is so horrific, comes shock and great dismay. 

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So resilient!

It’s always the case, it seems that feelings of hopelessness give way to ones of determination. You do your very best for your child who needs you to fight alongside as they battle this disease. There is no other choice and so, you put one foot in front of the other, so to speak. Melissa put it to me succinctly, “Our whole life was changed in an instant!” A life of normal living gives way to what becomes a new normal that most will never know about. Can you imagine the adjustments that would now have to be made along with the sacrifice of time that would be given? They were entering a life that would work around the needs of Silas. A life that is regimented to a large degree due to having a son with Batten disease. Melissa will tell you though, they were fortunate in some ways. First of all, Silas was diagnosed pretty quickly. This happened just two months after his first witnessed seizure. Silas’ neurologist had been quick to order genetic testing and the results came back quickly also. So far, CLN2 is the only variant of Batten disease that can be treated using enzyme replacement therapy and that is the variant that Silas had. He would be able to begin his treatments within a month of being diagnosed. Also fortunate for this family was the fact that the therapy was available at a hospital that was so close to where they lived. That being American Family Children’s Hospital in nearby Madison. They had been doing these treatments for about two years prior to beginning with Silas and they were only about an hour and a half away. It would be easy to get there but there would still be the challenges associated with having a child undergo this treatment every two weeks. First of all, he would have to have the surgery for the placement of a port at the top of his head. 


This can be terrifying for the parents but it has to be done in order for the child to receive enzyme replacement. Silas would be scheduled for the surgery on March the 26th of 2021 and he would receive his first treatment on April the 9th. The entire thing was difficult at the beginning with all the changes but the family adjusted and would figure things out. Melissa explained, “We had some hurdles regarding treatment, Silas being so active terrified me. How am I going to get an active Three-year-old to stay in a hospital room, hooked up to a machine for four plus hours. We figured out the best way to wrap his head and how to keep him calm and distracted from what was going on. It took a while, but we got it done. We had a few bad treatments. Silas pulled his needle out a few times, but we got past it.” Things were all of a sudden very different for this family of four and the events beginning this new life moved quickly. Everything had fallen into place for Silas to begin his treatments. One silver lining in all of this was that Tony and Melissa’s marriage had been planned for what turned out to be an off week for the infusions. They would all pack up and head back to the beach in Panama City, Florida where Tony had proposed to Melissa. There, they would be married, having both Silas and Summer involved in the ceremony. Tony and Melissa would not be deterred by all that had transpired as they married on April the 30th of 2021. No matter what, they were now man and wife and the wedding was beautiful to behold. Melissa said that life was perfect, except for the big cloud that was now hanging over their lives. Their handsome little boy was involved in a battle.

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The New Normal
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Life always moves forward, no matter what, and it would do so for the Nissen family. With Batten families, the diagnosis will bring with it a new normal. With certainty, that normal will always change as the battle continues. All of life’s normal activities continue but life is now different. Both Tony and Melissa would continue in their chosen professions while enjoying time away from work as a family of four. Now however, there was that cloud over everything that would otherwise be perfectly beautiful. Tony and Melissa had found one another in life and they now had these two beautiful children. Just as it seemed that their family was complete, they now had this challenge that most people will never even think about. Both Tony and Melissa loved Silas in the way that would be expected but there was now attached to that love, a type of grief that was anticipatory. The bond with these kids becomes so strong and the love is one that is undying. Melissa says that one of the things she struggled with was the fact that there are things that they just can’t do with Silas. She recalled a time that a situation left her more than disappointed. Melissa shared, “I remember we took him to this event called ‘Touch a Truck.’ They had all the construction trucks, garbage trucks, and fire trucks. All kinds of trucks. I was so excited for him. We got there and there were so many people. Silas wanted nothing to do with it and I was crushed. I cried the whole way home. Things like that are difficult.” There are not too many things that make a parent happier than seeing their child have a fun experience. Melissa was robbed of this because of changes that are due to Batten disease.
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Dad and son at conference.

It’s a process, adjusting to being the parent of a child with special needs. That process is so much more challenging when it involves something like Batten. Melissa went on to say that she has come a long way in dealing with these changes. They will sometimes make arrangements for someone to watch Silas and will take only Summer. Sometimes, one of them will stay with Silas while the other goes out with Summer. This is when it’s not practical for all of them to go out as a family. They are learning to not force things on Silas when he is not interested. No family should have to make these kinds of adjustments for this type of reason but it happens more than one can know. Also challenging is how to handle the situation as to what little sister Summer knows and when the right time is to tell her. Together, Tony and Melissa made the decision to be completely honest with Summer. They don’t want Silas’ condition to be a shock to her one day. For this reason, they give her the information in full even if she can’t always understand everything. Summer is starting to notice things about her big brother as she gets older and this just makes sense. It’s so easy to feel alone when you have a set of circumstances that are unique and which most people can’t understand. There are also so many questions that can’t be answered by people who haven’t experienced what you are going through. That is where the Batten community at large becomes such a valuable resource for people like the Nissen family. Tony and Melissa would, of course, connect with others in the Batten community through social media.

Melissa would find a Facebook page for parents of kids with CLN2 Batten disease and also a page for the Batten Disease Support and Research Association (BDSRA). There would also be connections with individuals with kids that battled CLN2. Friendships are always formed because, all of a sudden you now know others who walk in your shoes, so to speak. In June of 2021, Tony and Melissa would meet a couple named Ryan and Erica. Together, they had a beautiful little girl who they had named Harlowe. Just as Silas had been diagnosed with having CLN2, so had Harlowe and these families were just ten minutes away from each other. Harlowe had been diagnosed just three weeks after Silas and these two groups of people would become knitted together by a common experience. They would become family, even taking family vacations together. Melissa said that she also gained comfort from a mom named Molly Koslowski. She was the mom of a son named Brody who also battled CLN2 Batten. Molly was the first mom that Melissa met when Silas began his infusions at Children’s Hospital in Madison. Sadly, Brody has since lost his battle with Batten. Melissa shared that Molly had been very emotional over the fact that Silas and his family had joined their family in this journey. Molly obviously made an impression on the Nissen family. Also joining in on the support of this family is a name that is very familiar to me. That is Kayla Neveri, the mom of a Batten princess that is really special to me. That being her daughter Breanna, a.k.a., The Breannasaurus. It doesn’t surprise me that Kayla would be listed among the people that Melissa would gain support from. Melissa stated that Kayla answered a lot of the questions that she had in the beginning.

Kayla is now a veteran of this journey with Breanna having battled for some time now. Kayla’s family would be together with the Nissen family as they all attended Brody Koslowski’s funeral. These are gatherings that shouldn’t have to happen but it’s wonderful that these families are there to support each other in times that must be so difficult. As is most often the case, these families also find each other in person while attending a Batten family conference that is put on annually by the BDSRA. It’s a special thing that Silas and Harlowe would be brought to their first conference in Ohio. This was with both sets of parents taking them in a rented van in 2022. This was, no doubt, a special time. They would all also attend the 2023 conference in Texas, this time flying together. I can say first hand that these conferences are special albeit a bit overwhelming at first. Melissa described the first one as being scary. First of all because she didn’t know what to expect. Added to that was the experience of meeting the parents of kids that had finished their battle with Batten disease. No parent should have to anticipate such a thing. I’m glad that these folks do have each other to lean on. They do plan on attending again this July, this time in St. Louis, Missouri. I can’t think of too many things worse. That is then being told that your child has a disease that is fatal and for which there is no cure. Only those with a common experience can fully understand and finding each other makes the journey more bearable.
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Keeping Hope Alive
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No words adequate!

People do call this a journey for a reason and the journey continues for the Nissen family. Melissa shared, “Obviously I was devastated and in shock when I was told my handsome little boy had a fatal disease. I had heard of it before because of Tony’s cousin’s kids and knew it was really bad. I just couldn’t bring myself to believe it was something that would take Silas from me. I still have a hard time believing it.” The feelings are overwhelming at times for Melissa as they come in waves. The tears start to fall when she thinks about her son’s future. This is the type of thing that causes a love so profound. It’s that type of love that once mixed with heartache, it brings with it a type of love that is like none other. It’s a profound type of love and perhaps it’s best seen by those of us that look on from a distance. Melissa does say that she is also stronger now though. This, because it’s the only choice she has. Melissa will tell you that she has been changed by Batten disease. She shares a little more with this, “Being a Batten mom has made me so much more sensitive. It's also made me more aware of other disabilities. I have a special place in my heart for all children or people with disabilities or diseases.” Melissa would now be more apt to handle an emergency situation, like a seizure, while in public. This, because of dealing with her own son. In the past, it may have been a reason for her to panic while keeping her distance but not now. We haven’t mentioned Tony’s feelings as much as Melissa’s but one must realize what a son means to his father. 

Tony would tell me, although I already knew, he and Silas are best friends. There usually is a special bond between a dad and his son and Tony shared that he and Silas the type of bond that would be expected. In his words, “they fit together like peanut butter and Jelly.” It’s no wonder that Tony hates Batten disease with a passion. Tony had seen what  the disease had done in his cousin’s family and it has now invaded his home as well. Simply put, he shares, “I pray a lot that God will cure this disease or remove it from existence.” Life does go on for this family. There are good times together with friends, family, and each other. This is even though there are thoughts and feelings about what the future holds in the back of their minds. There is happiness and a cause for joy but there is also this challenge that not many can know about. Melissa talked about the cloud that is always above them. That is the cloud seen and felt by every family that has a rare child, like one who battles Batten. Melissa waited 34 years to become a mom and she was so excited when she received the news but now, she’s dreading what is yet to come. Think about it for a minute. Silas was diagnosed quickly. He had only two seizures at that point and it was unknown that his speech delay had anything to do with Batten. Once the truth was revealed, Tony and Melissa knew what was before them. Even with that, Melissa can’t fathom what it will be like as Silas loses his abilities. She has already been told that the progression of Batten is taking place in Silas’ eyes. 

The doctors feel this way even though it’s difficult to examine Silas because he doesn’t cooperate well during the examination. He is after all just a young little man! Obviously, This news leaves Tony and Melissa feeling devastated. Who couldn’t understand how they must feel as they anticipate the changes that are expected? Melissa shared, “His smile warms my heart, his laugh makes me smile. It’s the best feeling in the world and I don’t ever want to lose that love.” I wouldn’t want her to lose that love either and I know that Melissa never will. Recently, in August of 2023, Silas started receiving his infusions at Children’s Hospital which, as we mentioned, is located in their hometown. This was a great move and it makes things easier for them. Silas now has a team of doctors and nurses that the Nissens really appreciate. There is always a doctor present during Silas’ infusion and this brings his parents a lot of comfort. There truly are people in the medical and science communities that care greatly about these children. That should be a source of hope for the parents that battle alongside their children. It was in 2019 that we as a family traveled to Denver to attend a Batten family conference? While there, I remember seeing a pamphlet on a table outside a conference room that really caught my attention. It was produced by a company that was working on developing the science for gene replacement therapy for CLN2 Batten disease. This is the science that could eventually lead to a cure. 
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Silas Strong!

On the pamphlet were some of the pictures of children whose journeys I’ve followed and it was hoped that gene replacement would be available for them soon. How wonderful it would be to know that this had become a reality. Really, it couldn’t come soon enough for those who care deeply about these children. Progress would be made and gene therapy would show itself to be successful in one clinical trial outside of the United States. Sadly, about the time this happened, the financial partner for this trial pulled their funding. How could this happen? A child such as Silas would be a prime candidate to receive this type of treatment. His body would hopefully then start to produce, on it’s own, the enzyme that was missing. This is needed in order for the cells of his body to rid themselves of wastes that will eventually do great harm. I asked Tony a question about if he is still hopeful that gene therapy becomes a reality soon. Here is what he told me,  “Not only am I hoping gene therapy is available to Silas ASAP, I also believe it to be a long term cure! I play the lottery hoping I can buy him gene therapy. What's the crazy issue to me, is how can a company develop a donor drug for gene therapy that is proven to work. Tested in a one off trial, in Brazil, in a single human patient and decide after amazing results to halt all advancements because of money. It should be illegal to deny a child life saving treatment because of money.” Who could not understand this father’s frustration over the situation. Multiply this by every other parent that has a child with CLN2. 

Hope had turned to disappointment and frustration over the way things worked because money was involved and the funding had been pulled. This is so wrong and I do hope that a person or group of people step up soon. This, so that these dear children have a chance of being cured of CLN2 Batten disease. Hope is still alive but time is of the essence when you have a child that battles. In the meantime, Silas has a lot of people that love him. There are his parents and extended family, a lot of family friends, and an entire community that knows what it is like to battle. It was important that Silas was diagnosed so quickly and that enzyme replacement was made available to him very soon. We do need a cure for CLN2 and the other variants of Batten disease. This fight can never be forgotten. Never quit because good things still do happen! These children, kids like Silas, are so worth all of the time and energy it takes to rally around their cause. They produce in the people  that care for them a love that is like none other. Special and resilient are each one of them and the feelings they produce in us are profound and undying. All my best to the Nissen family and my thanks to them for allowing me to share their story. I hope that I did it justice. It’s another love story in the making. Thanks for taking the time to read it!

     ~Greg Lopez~
Blogger and Advocate

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