“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
Concerning kids who battle Batten disease (neuronal ceroid lipofuscinoses or NCLs), “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.” That is what happened to me!
Hello Everyone. These kids changed my life to a large degree as I was taken over the plight of one little girl. Through these kids, I have learned that it is important to look beyond ourselves and take notice. I've stated that Kids that battle are the most special and the most amazing and I believe that this is true. They are strong and resilient, even if unaware of the plight that they are involved with. I first became aware of kids that battle Batten disease as I learned of a little girl who fought the disorder. She really caught my attention and I began to write through a series of events …. never looking back. Hidden in between the titles of all of the stories is my own. These kids taught me how much I love to write. I’ll never win an award but the kids that battle taught me that I have a passion for sharing their stories with others. They are stories that are full of heartache and grief. From a distance though, you can observe a love that is profound and undying.
I have now branched out a bit to include some of the families whose lives have been changed by having a child who battles MPS (mucopolysaccharidoses).
The science behind how the cells of the human body work is fascinating. That being said, the result of what takes place when those cells don’t function correctly is devastating. Lysosmal Storage Disorders are my area of focus and these two groups of diseases are both LSDs. Perhaps I will add another lysosomal storage disorder community as time continues. Whatever the case may be, this all started because I was made aware of the plight of one little girl who changed me. I hope that you will take the time to look within. Thanks for doing so.
Blogger and Advocate
HOW THIS BLOG WORKS -
Take the titles listed below and go to month and year listed in the archives below (way below).
January 2017 -
*The Story Between The Lines
February 2017 -
*My Kind Of Royalty - CLN2
March 2017 -
*The Caring Type - CLN2
*Face to face: A Fairy-tale -CLN3
April 2017 -
*No Ordinary Love - CLN2
*A Rare Friendship
May 2017 -
*Tyrell and The Rare Love Story -CLN2
June 2017 -
*Seth and The Healing - CLN2
July 2017 -
*Little John and The Reason - CLN2
*Sands and The Scottish Princess - CLN3
August 2017 -
*A Rare Kind of Devotion -CLN2
*The Giggle Box - CLN2
September 2017 -
*Sweet Montanna and the Status - CLN3
October 2017 -
*The Blog about my Blog
*Sebastian's Story - CLN8
November 2017 -
*Kayden And The Change In Plans - CLN3
December 2017 -
*Those That Are Like Them
January 2018 -
*Nora Skye - I Know Just Where You Are - CLN2
*Brock and The Priceless Memories - CLN8
*Jamesy Boy and The Treatment - CLN2
*The Priceless Princess From Beyond - CLN2
*That Kind of Love - Conner's Story - CLN2
*What I See (She is a Butterfly) - CLN1
*No Longer Alone - Kristiina's Story - CLN2
*Things We've Talked About - Oscar's Story - CLN7
*The Reason Why - Hannah's Story - CLN3
*Kayla's Perfect Princess - Breanna's Story - CLN2
*Warriors Unaware - Mia and Kaleb - CLN2
*Her Little Story - CLN1
*Someone Like Raelynn
*Forever Royal - The Final Version - CLN2
*Awfully Beautiful - The Life of Noah and Laine - CLN2
*A Lifetime of Love - Tegen's Destiny CLN1
*Forever Beautiful - Gabi's Story - CLN7
*The Miracle Baby - Amelia's Story - CLN1
*Dylan's Life - A Small Town Story - CLN2
*Princess Grace and the Challenges - CLN2
*Anything for Tessa - Her Story - CLN2
*Haley Bug's Battle - A Family Story - CLN1
*Journey Unexpected - The Rich Family - CLN2
*My Life, My Everything - Lydia Rose - CLN2
*A Love That Hurts - Shashka's Story - CLN2
*The Things Looked Forward to - Addy’s Story - CLN3
*Love Complicated - Bailey Rae's Story - CLN3
*So Many Questions - Evelyn's Story - CLN2
*The Feelings They Produce - Violet's Story - CLN2
*Because of Love - Frankie's Story - CLN2
*The Need to Breathe - Scarlett's Story - CLN2
*The Beautiful Light of Haidyn Grace - MPS3
*Any Distance Traveled - Reagan's Story - MPS3
*The Missing Someone - Kiernan's Story - MPS3
*The Transformation - Victoria's Story - MPS3
*Not by Accident - The Charlie Grace Story - MPS1
*The Difference They Make - Harvy's Story - MPS6
*Love Profound - Veda Rae's Story - MPS3
Highlights from the beginning. When the blog was named 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