“Looking at Nicole that night when we got home, sitting in her room. Surrounded by her little princess dolls, with fairy lights on and Disney bedtime music softly playing in the background, I cried like I had never cried before. It was a sickening feeling to look at our little sweetheart and to know that she had no idea what she had and how her life was to be. It was absolutely heartbreaking.”
Batten Mam and Advocate
It can happen when I am reading something concerning a child that battles Batten disease, or it can come from a passing thought when I am driving. It might also happen when I am watching a YouTube video in preparation for writing a story. That is what took place today. I get moved by the details. Watching a younger sibling who has Batten disease as she helps her older sister to do something that she is now incapable of doing by herself. This while exhibiting the love that is there while she is doing so. These are the kinds of things that often move me in a big way. Writing is not something that I do full time. I actually work in the aviation field on components for helicopters that fight fires. I work in a shop in which I spend a lot of time with male co-workers and I do so for a lot of hours. I bring my laptop with me, using breaks and lunch to write or get organized to do so. Sometimes, I will have to stop typing as I put my thoughts into words. This is because I can feel myself getting worked up beyond the point where it is safe to continue. Am I somehow exhibiting a pathetic show of emotions as a male individual? Whatever your opinion might be, I can tell you that it is possible to be strong while at the same time, also being sensitive. You only have to allow yourself to become vulnerable. Kids that battle a rare disease like Batten are to me the most special. Others might agree but first, they have to be shown some things.
A person first has to be made aware. Most people won’t even think about kids that battle a rare disease. Many might be reminded that such a thing exists only to shrug their shoulders and move on. After all, the plight of these kids doesn’t concern them. The result could be different for those who take the time and who show some interest. If a person pays attention and takes the time to be involved, they may be changed. They might end up giving a piece of their heart to these kids. As I have said in the past, if that happens, a person may come close to giving these kids all of it. I repeat myself frequently as I write these stories. I do so because I like to place emphasis on things that I have mentioned before. Things that apply to each family that battles alongside their little warriors. Each story is unique because each one shares the details of a different family’s journey. However, there are some things that all of the stories have in common. After all, these are children that we are talking about. The things experienced while battling alongside them can be those which the families share in common. Undying love and a bond that cannot be broken. These are just two examples of what a parent or caregiver of a Batten warrior will come to understand. Battling illness is something that should only be experienced by those who have aged. It is so difficult to watch when a child suffers through something like a rare disease.
One question that I have asked frequently is this - What is it about these kids? Is it the feelings that are produced in us as we consider the plight of these children? Is it just what we are feeling or is it that there is truly something special about them? When something like the loss of a child’s eyesight occurs and they bounce back from the disappointment. When we see them press on in life, finding the joy in living again and showing this through laughter and innocent smiles that can still light up a room. Should we not find that a cause to be amazed? When a child can no longer communicate verbally with us but we can see the love that is there. We can see the love that is being reciprocated as the child’s eyes light up in our presence. There is that smile again. The smile that gives many parents encouragement to move forward and fight for another day. So familiar to me now are the images of the children that battle CLN2 Batten. CLN2 is just one of 13 variants of Batten disease but it is one of the most common forms to affect young children. Oh, how I wish that all of the kids that battle Batten had access to Enzyme Replacement Therapy. Even so, I am thankful that this therapy is available to these beautiful children that battle CLN2. The images that I speak of are the ones that show these kids wrapped in bandages. This as a result of the treatment that they receive every two weeks. What a commitment on the part of the families as they travel to have this procedure performed.
These kids are such troopers. For many of them, the side effects and complications associated with enzyme replacement take their toll. This, each time they undergo the therapy but again they always bounce back. The images of these sweet faces surrounded by bandages will always stay with me. Innocent children enduring more than most any adult will see in a lifetime. The things that they go through will not take away the joy that they have for life. Being in large part, unaware of the enormity of what they face, they simply press on. They do what kids do in every way, for as long as they are able. They battle so hard and they do so, so sweetly. It would be so easy to focus solely on the negative impact that something like Batten will have on a family. They do not call what is experienced by Batten families a “journey” for nothing. There are many struggles along the way and I try my best to detail these in the stories that I write. I do, however, try my best to make the love stories that are produced by these families my focus. There is sadness yes, but a love that is profound and undying. There is a bond that is formed that can never be broken (you wouldn’t want it to be). There I go, repeating myself again. With all of this in mind, I am headed towards telling the story of another family that personifies most everything that I have just talked about. Having sought to be a normal family, finding success as such, they have become much more than average when compared to your other families. Let’s not wait any longer to get started.
Gail Baxter grew up in the city of Carlisle, which is a town that is close to Scotland and in the northern part of England. Growing up, she thought about what her life might be like as she grew into adulthood. Whatever the case may have been in her thinking, she would find success in whatever she endeavored to do. Gail would grow to be a take care of business kind of individual. She would move from Carlisle to the city of Newcastle in her early adulthood and she would begin a career in the banking and mortgage sector. Gail would work her way up to a managerial position by climbing the ladder. She started her ascent serving in positions such as financial advisor and customer service manager for the Northern Rock Building Society. One day, she would begin to manage people, overseeing a team of sixty individuals. Gail was a coach and a person who helped in developing the talent of others. That is something that she thrived in and also, something that she thoroughly enjoyed doing. She had found success in her work life but there was more to life than just working. There was more for Gail to yet experience. The sun was shining on a bright August day. It was actually a Bank holiday in the UK that day and the year was 2006. Gail was out with her sister Emily and they had found their way into a bar which was named Osbornes. This was a well known and well put together establishment in Jesmond (a suburb) within Newcastle.
As the two ladies were enjoying their conversation, in would walk members of the Newcastle Vipers Ice Hockey team. One of the players, who by the way, was brand new to the team was a gentleman named Matthew Rich. Matthew had literally dropped his bags off in the hotel where the team had just arrived and had headed to the bar with his new teammates. Matthew was originally from Glasgow, Scotland and had grown up playing hockey. He had even moved to Canada when he was young to play at the high school level. Matthew had attended school and played hockey for Brock high school in Cannington, Ontario his first year and then headed to Notre Dame High in Wilcox, Saskatchewan to play his second year. Hockey was a huge part of Matthew’s life and Canada was, of course, a great place to further develop his skills. Matthew also sharpened his abilities by playing at the highest level within minor hockey in Canada. This as he played for the Central Ontario Wolves. He had even played for Scotland’s U21 Hockey squad (Under 21 age group) in preparation for a career in professional hockey. Matthew’s career had developed, just as he would want it to and here he was, a member of the Vipers and in Newcastle at Osborne's. This is where Matthew Rich and Gail Baxter would meet. Mathew had walked over to talk with the two sisters and he and Gail would gain an attraction towards one another. Neither of them knew at the time but they had each met their life partner. Before they left each other’s company, Matthew had put his number into Gail’s phone and a friendship would quickly develop.
Texts and phone calls would be exchanged as each one continued on in life with their individual careers. The two of them would soon after begin to date as their relationship continued to develop. Gail was not too familiar with the world of hockey at the time that she and Matthew would meet but she would soon get a crash course in the sport. This, much to her amazement. Gail explains, “I had never seen an ice hockey game before and was soooo impressed!! It is such an amazing sport to watch. I couldn’t even stand up on the ice and to see Matthew flying around the ice with such speed, strength, and skill was just incredible – I loved it!” Gail would have the ability to watch Matthew play in Newcastle for a few months until he was signed by a team named the Manchester Phoenix. This would mean a move for Matthew to a town name Hale, which was about 13 kilometers from Manchester. With this, Matthew and Gail would embark on a long distance relationship. Gail would not let the distance between them get in the way. She began to travel the distance necessary for them to spend time together and to see Matthew play. This would take place most every Friday after she finished at work and the trip would take over two and a half hours one way. She would return to Newcastle in the early morning hours on Mondays, in time to go straight to work. That is commitment but it was well worth the effort as Gail and Matthew continued to grow in their relationship. It is amazing to think about how the two of them would meet and fall in love. They could have missed meeting each other by an hour's time and would have never met at Osbournes but their relationship was one that was meant to be.
Matthew’s career would continue on as he had put in the hard work that it takes to play at a high level. All of the training, practice, and play in competition had really paid off. Hockey had become a way of life for Matthew and Gail was more than willing help by supporting his pursuit of excellence. Sadly though, things would take a drastic turn as the couple took a vacation together in Egypt. It was at that time that Matthew would come down with a severe case of food poisoning. The reaction that took place was one that was extreme and that would land Matthew in the infectious disease ward in Glasgow. This would happen as soon as he and Gail had returned home and he wouldn’t leave that facility for weeks. Matthew couldn’t walk and was bound to a wheelchair upon leaving. He would be diagnosed with Reactive Arthritis and it would take around six months to recover from his illness. Just to walk again, handling his own weight was a struggle but Matthew would overcome and get his health back. Gail would tell me that Matthew’s hockey club was very supportive during the ordeal and they would reinstate him after he returned to full health. Matthew would train and then return to playing hockey when he was able to compete. Being a professional athlete and staying at a competitive level is not easy. Recurring injuries can often take their toll on a player and that is what would take place in Matthew’s case. An issue would arise with his shoulder dislocating. This would take place a few times and it would interfere with his ability to compete. His shoulder was never the same and this would cause him to have to walk away from the sport that he loved.
Hockey was such a huge part of Matthew’s life and leaving was a “massive blow” to his existence. Even so, he accepted what was the reality of the situation. Matthew started to look for work while he and Gail set up home together. Hockey would no longer be the center of the young couples existence but they had each other and they were in love. They now had a life together, although they could not know what that life would entail for them. All throughout this time period, Gail had continued to work in the banking sector and Matthew would find employment working in industry. Life was about work and time spent between the two of them as their new life together continued to take shape. Gail had found success in her work life and had been rewarded by gaining positions of increased responsibility. She had put a lot of time and energy into her work but there was more that she wanted out of life. Before I continue, I should stop to explain that this writer learns something new every day. I am, in fact, learning new things with each story that I write so let me explain. We all know that having children of our own as we grow is a God given desire for most of us. I know there are exceptions. Now being from the United States, I know that most women want to enjoy the experience of being a mom when they grow up. I knew that in certain parts of the world, like down under in Australia and in the United Kingdom, that moms are referred to as mums. However, I did not know that in other places, like Ireland, Wales, and parts of Northern England that mothers are referred to as mams.
More to Life
I think that my readers know where I am going from here. Gail had found success in her work life but there was more that she wanted for herself. She had the desire to be a mam to children of her own and this desire only increased as she met the man that she desired to spend her life with. As a couple, Matthew and Gail wanted children together and that would soon become a reality. Both Matthew and Gail were so pleased when they received the news that Gail was expecting their first child. Just as they would hope for, Gail’s pregnancy would progress without major incident as the time would approach. It was also at that time that things were beginning to change with her work. Gail had been with her company for ten years when as a business her employer began to struggle, and changes began to take place. A reduction in force was coming and this left Gail with some options. What is often called a lay-off here in American is referred to as a redundancy in the UK and Gail would be offered the option of taking part in that. She would take this option instead of going on maternity leave because the timing was perfect for her to begin the next chapter. That of being a mam and raising a family! Gail’s co-workers recommended that she leave work a couple of weeks before she was due to deliver her baby and she agreed that would be a good idea. Gail had planned on having some down time before the day arrived but her baby boy had other plans.
That's right! Their baby, who Matthew and Gail would name Louis, was a boy. He decided that it was time to make his entrance into his parent’s lives a couple of days after Gail left work. Louis arrived on September the 2nd, of 2010. He had all of his fingers and toes and he was the most beautiful thing that his parents had even seen. Babies are by nature beautiful, are they not? Gail explained what she was feeling as she held baby Louis for the first time. I have also included some of what she was feeling after Louis was brought home. Here is what she would say, “... for the first time in my life, I felt I had found my place. I was meant to be a Mam! I remember a few days after bringing him home, we had family around and he was in his crib in the living room. We all sat around looking at him and I burst into tears and said ‘I just don’t want anything to ever happen to him, I want to keep him safe forever.’ It was the most real feeling of protection and I will never forgot that.” Louis was the picture of perfection and purity and he belonged to Matthew and Gail. Was Louis a little hockey player in the making? Well, whatever the case might be, he was adored by his family and still is to this day. His parents loved watching Louis grow and develop into a little man. Watching his childlike ways filled them inside like nothing else could. In all honesty, Louis was not the first baby of the house. That would be Matthew and Gail’s pet Beagle which they name Archie. The couple had researched and found that Beagles were good with children. Archie came to them about three weeks before the couple found out that Gail was pregnant. They, no doubt, loved watching Louis and Archie interact with each other!
Being parents to Louis was an amazing experience for Matthew and Gail. This, just as you would expect it to be. Some people are content with one child and some are intent on keeping things that way. That was not the case with this young couple. They wanted to experience the joy of bringing another little bundle into the world. They again wanted to experience the wonder of seeing those little hands and feet wiggle for the very first time. Gail would receive the news that she was expecting in the summer of 2011 and both she and Matthew were excited about it. They were going to become parents for the 2nd time. Things would go generally well for the first six months as Gail carried baby number two however, a concern would soon arise. It seems that Louis’ brother or sister to be was not growing as a baby would be expected to. For this reason, Gail was in and out of additional appointments because of the concern this created. It would be decided that it was best to deliver the baby by inducing labor three weeks early. Thirty-seven weeks of pregnancy was the goal that was hoped to be reached and that would be accomplished. It was time to bring the new bundle into the world and this would take place at the Royal Victoria Infirmary in Newcastle. It would be on March the 1st of 2012 that Gail would give birth to a tiny, yet beautiful, baby girl. The name given to this precious little person was Nicole Thomasena Rich. Her given middle name was that of Matthew’s Nana. She weighed a slight four pounds and eight ounces but even so, baby Nicole was flawlessly beautiful. She had all of her fingers and toes and she was covered in brand new baby skin!
The infatuation between tiny Nicole and her mam was so strong and it took place the instant she was handed to Gail. There is a special bond between a mother and her newborn child. It begins to develop even before the baby is delivered. The quality of relationship between a mother and her newborn daughter is perhaps greater than can be explained. Here is what Gail had to say about the experience, “That was it, I was instantly and completed besotted!! There is nothing more precious in this world than having your baby placed on your chest for the first time and you feel their tiny heartbeat and know they are yours. You have made this little person. Instant protection, overwhelming love. We could not have been happier.” Nicole would have to stay in the Special Care unit for a couple of nights due to her small size. The medical staff wanted to make sure that she was feeding well. Everything proved to be okay with their precious little newborn and the Rich family was now a family of four. The midwife that had delivered Nicole came to see everyone the very next day. She was so relieved that Nicole was doing well with as small as she was at birth. She was small but very strong and amazing to everyone who set their gaze on her. Gail describes what people were seeing in Nicole as they looked at their newborn daughter, “When we had visitors, they would look at Nicole and say ‘she’s been here before!’ She never had that glazed baby gaze they sometimes have, she was always looking around intently. She fed well, she was such a healthy baby and for as little as she was, she was strong as an Ox! Little did we know this would serve her well in this little life.”
Bringing Nicole home for the first time was, no doubt, a special event. According to Gail, Matthew had described Nicole's homecoming as an “explosion of pink” and I have no doubt that this was absolutely true. Why not celebrate the fact that there was a new little princess in the house? Princess’ and pink just go together. The Mammy of the house had her baby girl and everyone was happy to know this. Gail was now well established as a stay at home mam and this was a role that brought her a lot of fulfillment. Now, the enjoyment that she received as a mam would be multiplied by two. It had to seem so perfect, at that time. She had this beautiful little girl to compliment the son that was so adored by her and Matthew. Just as it had been with Louis, they now had the opportunity to watch little Nicole grow and thrive in the household environment. She would bring so much joy to her family as the days and months passed by. There would be nothing out of the ordinary that would stand out to Gail as she watched Nicole grow. By that I mean in the way that she reached her developmental milestones. It is normal for children to advance in different areas of their development at their own pace. Louis had been quick to reach his milestones and he was a very talkative young fellow but Nicole was coming right along with things as she continued to grow. She crawled and then walked not that long after, advancing to the point of being given the title of “daredevil”. Nicole loved the outdoors and all of the things that went with it. Running, climbing, and going down slides. She was adorable and so fun to watch.
Not Yet Complete
The timing of the reduction in force at the banking institute that Gail had worked for had been perfect! There was no maternity leave to return from, so there was no decision to be made as far as what to do. Gail had been afforded the opportunity to stay at home full time and this was very much welcomed. Especially because there were no grandparents nearby to help with the children. Gail was available to take children to every baby group and play group that there was. She was able to get to know the other mammies from these groups and play areas and this helped her to develop friendships with many of them. This was not to mention the fact that she was able to give her children her own undivided attention while they were young. Matthew and Gail had talked about her going back to work at some point but Gail knew she didn’t want to return to banking. She had a deep desire to work for herself one day and in fact, she had something in mind. I will let Gail explain in her own words, “My dream was to have a coffee shop/tea room and bakery (as I love baking!) and it was going to be called Barndoor Bakery. I have always loved baking and have always made our children’s birthday cakes, made family christening cakes etc. So I thought I might go into cake making – I made our wedding cake and then made two of my friends' wedding cakes too! No pressure there!”
Matthew and Gail knew that Gail would have to return to work someday but the children were young and they wanted to afford them the opportunity to have their mammy around for as long as they could have Gail home. There was something else that would shape their future as a family and that was the feeling that they were not yet complete in the size of family that they wanted. To some, having a girl and a boy seemed so perfect. Why not just call things good and move towards the next stages of family life? Gail explains her feelings in this way, “When we had Louis and then Nicole, a lot of people assumed that was us, one of each, but we didn’t feel like that at all. We were blessed beyond anything we could imagine but I still had a sense deep inside, that I wanted another child. As much as I adored and loved Louis and Nicole more than anything in the world, I just had this feeling that I was not complete.” It was just a fact that for this couple, there was more yet to be experienced. Both Matthew and Gail were people that had shown that they could achieve the things that they had set out to do. They had plans for a life together and the future was still largely in front of them. Things looked bright for them even if their circumstances had changed. Both being intelligent people, they could work together and focus on what their future would look like. Even with this, they were not quite ready to stop increasing the size of their family. Gail explains, “I adore having children, to care and protect them, to feel their love, to have their tiny arms wrapped around your neck. There is nothing more precious or that brings you more joy than the love of your family.” There was more yet to come and much more that would be experienced.
Matthew and Gail’s life would, one day, be redirected by the circumstances that they would find themselves in. At the time, they had no idea what lay in front of them. Most always, we cannot see all that the future holds for us and sometimes, things take place that seem overwhelming at the time. Whatever the case might be, the Matthew and Gail would face life together. They had been joined together in their hearts from the beginning of things and it was always their intent to be committed to one another. Those intentions would really start to take shape as Matthew proposed to Gail in February of 2013. The very sweet thing about the way that things were done was that everything now included the children. The event began to take place as Matthew enlisted the help of his own mam in Glasgow on a secret mission to get the engagement ring resized to fit Gail’s ring finger. There was a bit of clever deception that took place as Matthew claimed that he had left his wallet there in Glasgow after they had visited. He needed to meet his mam halfway in order to get the wallet back (every man needs his wallet) but in actuality, it was the ring that had been resized that he was after. He returned home with it in his pocket, and with Gail being “completely oblivious!” Gail explained what would follow, “He proposed that night and it couldn’t have been more beautiful. It was nearly Nicole’s bedtime so he asked me to get her ready for bed. I started to get her ready and I saw a pink ribbon round Nicole’s tiny tummy with a ring tied to it! I couldn’t believe it! It was a total surprise and such a perfect way to involve our babies. Louis came in with Matthew and got down on one knee and proposed!”
It would be one year later that this mam and dad of two would become man and wife. The date for the big event had been set and it would take place on the 1st of February in 2014 at a place named Matfen Hall. As Gail would put it to me, Matfen Hall is “a stunning and stately home in the beautiful Northumberland countryside.” To look at the pictures would tell everyone that this is, in fact, the truth. The estate is beautiful even from a great distance away and that is where this family affair would happen. Louis was set to be the page boy and the “bestest man” as Matthew’s brother would serve as the official best man alongside Louis. Nicole would serve as the flower girl and you can only imagine that watching Louis and Nicole doing their part was a reason for people to smile. Gail would explain that Louis was dressed in an adorable little kilt and a sporran. The sporran would be filled with his favorite drumstick lollies and other sweets. Little Nicole was dressed beautifully, having a special floral tiara and a wand made just for her. The entire experience would be captured in order to provide precious memories that could be viewed at a later date. Gail talks about the video as she gives some detail about that special time together, “We had contemplated having the wedding videotaped and at the last minute we decided to go ahead. Thank goodness we did, because we now have the most precious, precious memories of that very special day. Nicole running along the front of the guests squealing, excited as we were just about to say our vows! When Louis gave us the wedding ring on a tartan cushion with the ribbon tied around it to hold the ring .… he then says to Daddy ‘look, it’s a present daddy’…. and indeed it was.”
Matthew had used the same ribbon that had originally held the engagement ring onto Nicole’s tummy for a special purpose. It was used to have Gail’s wedding bouquet tied together. What a great symbol of thoughtful intent and commitment that would turn out to be. Everything that was done, was done thoughtfully. This would be an event where the newlyweds would be surrounded by friends and family on what would be an extremely special day. A life spent with each other had really come together. Life as a family of four would continue and it would continue to be a beautiful story in the making. It would only get better as the next big event would take place. This would be the announcement that Gail was expecting child number three. The Rich family would soon be increasing in size as Gail would be expecting once again. There would be several false alarms along the way and the news would come a little later than it had before. Both her and Matthew were ecstatic with the news that Gail was expecting again. She had, in fact, been convinced that they would have another little boy this time around. Gail explains, “I was drawn to boy’s names, and to boy’s clothes. I was playing out scenarios of how Nicole would be protected by two brothers! Ha ha!” Gail would be monitored more closely during her pregnancy than she had been previously. This was because they wanted to ensure that the baby had been growing as expected. All had turned out well with baby Nicole, however, her growth in the womb was not what one would hope for. Things would go well with this pregnancy and the day would arrive.
The Perfect Number
Just like it had been with her big sister (that’s right, the baby was a girl after all!) the new addition to the Rich family would be born at the Royal Victoria Infirmary in Newcastle. The new princess would be born on the 18th of September in 2015 at 5:45 pm. This baby girl came to her family weighing in at six pounds and one and a half ounces. Just like her big sister had been when she was born, this little girl was flawless and she was flawlessly beautiful. Yes, that’s right! She had all of her fingers and toes and she was covered in brand new baby skin. Gail describes the big event and how her life was impacted by that day, “...our darling second daughter came into the world and the second she was placed on my chest I felt complete. There is no other word to describe it. The longing I had felt in my heart for a third child had gone. I had been blessed beyond belief and my life as a mammy of three was a dream come true.” Matthew and Gail had chosen the names Harper or Belle for if the baby was a girl. When they looked at their newborn daughter neither name fit for one reason or another. Their daughter would actually be named “baby no name” upon leaving the hospital because they couldn’t decide on what her name should be. They had thought about the name Jessica at one point in the pregnancy but had forgotten about it. Matthew’s mam would remind them by asking, “What about Jessica?” The young couple looked down at their beautiful little baby and thought, “that’s it, Jessica is perfect!” And so, she was named Jessica Annie Rich. She received her middle name after Gail’s Nana who was named Annie.
Before that would take place however, they would leave the infirmary a family of five, ready to take on what life would bring their way. Still at this time there existed the fact that Matthew was the only one working and this would allow Gail to stay home with the children for an extended period of time. There was no end to a maternity leave that had to be dealt with. She was there for everything like all of the baby groups, school drop-offs and pick-ups. Gail was there to see her kids grow and to reach new milestones and she was there for Matthew when he arrived home from his day. Gail figured that this was time that they would never get back. This was hers and Matthew’s thinking, even with not knowing what lay ahead for them. There were so many life events that they looked forward to as they thought about what the future may hold. There were thoughts of children and their schooling as they graduated and moved onto all of the levels of education. Would these three attend college, or would they work in a vocation of their choosing? Would they work in the family business that Gail had dreamed of having? Perhaps Louis would be an athlete like his father. There could be family gatherings of all kinds and time spent together throughout the years. Christmas’ and weddings, these were some of the possibilities for what could take place. Being a family could also bring hardships. There are often difficulties that can come along as time passes. Part of being a family is dealing with situations as they arise. Scrapes and bruises or a broken bone are the kinds of things that one may expect as children are growing up. Some children are born with special needs and others require greater attention in school.
Going through life together and dealing with life’s difficulties is part of what being a family is all about. There are things that just don’t come to a parent’s mind under normal circumstances. There are so many unknowns that can take us by surprise. That is also part of living on this planet. Something like a rare disease is one of those unknowns that can appear out of nowhere and there was no way that Matthew and Gail could know. There was something in their future as a family that would change life for them forever. Everything had gone so well with young Louis as he progressed and developed. Things would be different for beautiful Nicole during her early development. It would soon be clear to her family that they had a special set of circumstances with her. One thing would be noticed as early as two years of age and that was that Nicole was behind in the development of her speech. It would be at her two year check-up that it was noted, Nicole was not saying any words yet. As a result, language therapists would be called in to intervene. Even with this, Matthew and Gail would not be overly concerned at this point. After all, Nicole was understanding what was being said to her and she was following instructions. They just thought that her language skills would come with time. Little Nicole would receive over one hundred therapy sessions using methods such as visual timetables and pictures cards. It would seem that she would begin to get the hang of things but would lose interest.
Time would continue on and more people would take notice in regards to Nicole’s lack of skills in the area of her speech. Both the teaching staff, as well as her speech therapist, were concerned as Nicole entered the pre-school environment. Nicole was just not progressing, in spite of all of the added attention that she was receiving. It must have been so disheartening for people to look at that darling little face and to know that Nicole was struggling the way that she was. The concern that Matthew and Gail had over Nicole had to be increasing as the days passed by. There had been an additional concern that had been noted while this had all been taking place. That was the fact that Nicole was having an issue with a lack of concentration. Nicole had not yet spoken a word as she turned three years of age and at this point, her situation was alarming. It was time to seek medical attention in order to try to find an answer and the first place to start was with the pediatrician. It is a challenge anytime one of multiple siblings in the house requires extra attention and I know that this would be a challenge as things continued to develop. Little Nicole would undergo a series of cognitive and behavioral tests to try to determine what was taking place. As the testing was completed, a result would be given showing the diagnosis of Global Developmental Delay. GDD is really just a blanket term that is applied to children that show significant delays in their cognitive and physical development.
This would certainly fit with what the family was seeing in Nicole. She was delayed in almost all of the areas of her development. How difficult this must have been at this point. Nicole was, at three years of age, displaying the behaviors and characteristics of a child that is 18-20 months old. She had been first seen as a child that would catch up at her own pace, but now, that was no longer a possibility. The subtle regression of her skills was now, much more obvious. As Gail had stated in a piece that she had written for BDFA of the UK, “We had been watching our baby girl deteriorate in front of our eyes completely oblivious to the reason.” Developmental delay is something that a family can live with in a child. It does take an adjustment in a person's psyche. A parent will learn that they have a special case on their hands and they will come to terms with the fact that their child may need their help over an extended period of time. It could be caused by something that happened at the time that the child was carried in the womb, or it could be something that is out of order genetically. People with special needs have a special place in society and especially, in their families. In Nicoles case, there was more that was taking place. Much more! It would be just days after the diagnosis of developmental delay that Nicole would have her first seizure. There are not many things that are more frightening for a parent to see happen. This little porcelain doll of a child was seen to experience something so terrifying to her parents.
There was no history of epilepsy in the family and Nicole’s parents had no idea what was going on as the initial seizure was taking place. Gail described that first event with the following, “It started with a twitch that traveled up her little body. She then went lifeless, turned blue, and then stopped breathing.” Can you imagine how terrifying that would be for a parent to witness such a thing happening with their child? Matthew and Gail truly thought that they might lose their tiny princess at the time that the seizure was taking place. Like most any set of parents would, the Riches made sure that Nicole was seen at a hospital, and afterwards, an EEG would be performed. The result would be that she was given the diagnosis of focal epilepsy. Focal epilepsy is simply a condition that involves recurring seizures that can affect any one portion of the brain. The doctors would come to believe that there was an underlying syndrome involved in Nicole’s case but they did not know what it was. They were not immediately able to piece together all of the symptoms that they were seeing in Nicole. What would transpire was a search for answers that would take place over a more than two year period. All the while, this sweet little girl continued to regress. The Rich family would experience an attitude of love and concern on behalf of the medical professionals as they tried to help. Gail would use words like “amazing” to describe the care that they would receive but the answers that they sought after would not come quickly. Nicole would, of course, be under the care of a neurologist and the Riches would return there frequently looking for help. This was in addition to visits with Nicole’s pediatrician.
The problem was that her issues were related to a rare condition that was escaping those who were trying to help. You can only imagine that medical professionals would look at someone like Nicole and would want to give her their very best care. She is someone who would melt most anyone’s heart. Just look at her! Gail speaks about what she witnessed during this very confusing time, “They really wanted to help us and bonded very quickly with Nicole. Our pediatrician used to call Nicole ‘poppet’, it was so sweet. Nicole would walk into the room and everyone would instantly connect with her. She was just, and still is, adorable! Because she was so small, she looked even cuter and as she was mischievous, it just added to her infectious and loveable character.” It would be at the time that Nicole’s mobility started to become affected that her parents really became concerned. This had to be more than just simple epilepsy. Nicole would begin to fall frequently, stumbling and never really having control as she walked. Yes, it had to be a syndrome that was yet unknown and there needed to be a conclusion to the search for an answer. Matthew and Gail urged the doctors to continue testing until that answer was found. This family knew that something wasn’t right! People reading this story will already be aware. The Rich family had begun a journey that involved a diagnosis for a rare disease named Batten, known clinically as ceroid lipofuscinosis, neuronal. The problem is that Batten disease is one of 7,000 rare diseases that affect children.
The disease has seen an increase in awareness in recent years but that is not the case in every place throughout the world. More doctors have knowledge concerning Batten disease but not in every place. It is also true that many of its symptoms are shared with other diseases. For the most part, it can only be confirmed through genetic testing and that happens when you have an idea what to look for. The majority of the medical professionals that Matthew and Gail had come in contact with had never heard of Batten disease. Even when it came to Nicole’s neurologist, he had only seen one case of it in twenty years of practicing medicine. The insistence of Riches would eventually pay off even if the result would be completely disheartening. Eventually, doctors would think to have blood drawn to check for a metabolic disorder and to have it sent to the Great Ormond Street Hospital in London for testing. The results of the testing would eventually come back and the Riches would be contacted. The doctor wanted Matthew and Gail to come in to get the results. They wanted them to come in by themselves and without the children. On the way there, the couple thought about what they would possibly encounter with the news. Would their daughter be wheelchair bound for the course of her entire life? Would she remain in her parents' care for many years to come? There were so many things that were unknown to them in that moment and the news would be worse than anything that they could imagine.
Once arriving at the hospital, the Riches would be met by a team that included the pediatrician, a nurse, and a neurologist. Once there in the room with the medical staff, Matthew and Gail would be given the news that their beautiful little girl had CLN2 Batten disease. These are dates that one may never forget although a person may wish that they could. September the 16th of the year 2016 is the day that they first learned that such a thing as Batten disease existed. They would learn the details of what would take place with little Nicole. They would learn that there is not a cure and that the disease is fatal. There usually are not words that can fully describe what a parent is feeling at a time such as this, however, Gail fully explained the experience with these words, “Nothing can ever prepare you to hear those words. It felt like we were looking in on someone else being told. Like it wasn’t real. We kept saying “ You can’t be talking about Nicole, you must be wrong.”…. It was a physical pain, like our hearts were actually breaking. It was the worst moment of our lives.” For a long time, doctors were incapable of doing anything other than to tell the parents of a child with CLN2 Batten …. “Go home and make your child’s life as amazing as possible” Gail would share hers and Matthew’s initial thoughts. Were they just meant to go home and watch their innocent beautiful Nicole deteriorate and die? There was hope for a course of action but in those moments, there was only despair and grief that was unbearable.
So many different things would take place in the weeks and months that followed Nicole’s diagnosis. The Riches would learn much more about the disease and they would connect with the Batten Disease Family Association (BDFA) in the UK. Matthew and Gail would learn that they were far from being alone in this journey. This was very much welcomed, even if knowing this could not take away the pain that they felt. One thing was apparent as they learned that this was a genetic disorder. That was that it was possible for their other children to be affected. Louis was past the normal age of onset and he had shown no symptoms, but what about baby Jessica? The only thing that could make the news concerning Nicole worse than it was, would be if they would learn that Jessica was also affected. They of course, would have Jessica tested genetically and the outcome would not be good. The grief that they now carried would be multiplied by two as their hearts were broken for a 2nd time. This would also take place in 2016, three months later in December. Jessica’s test results had shown that she also had CLN2 Batten disease. The news couldn’t have come at a worse time. The Riches were beginning their travels with Nicole to a Starlight Lapland wish trip that is hosted by the Starlight Children’s Foundation. Jessica, who was only fifteen months old at the time, was staying with Matthew’s parents in Glasgow.
It was difficult but necessary to leave Jessica as this time was meant for focusing their attention on Nicole while also making memories. They had “cuddled'' Jessica and said that they would see her in a few days. Matthew and Gail would leave, not knowing the utter despair and pain that would follow. How could this be happening to both of these innocent little faces? Being a mam to these children had brought Gail to the place that she had longed to be. Matthew had to have been beaming with pride over this beautiful family that was his responsibility. Now however, he was met with concern that was overwhelming. Again, what was being felt would be more than most could describe, however Gail paints the picture perfectly with these words, “I still don’t know how we were able to get through and how we managed to hold it together as well as we did. I remember wailing and screaming, I felt like the life had been sucked from me, I have never felt so helpless or so weak. It was definitely a physical pain, but my mind was in pure turmoil.” It would be early after Nicole’s diagnosis that the Riches would learn about a life changing treatment that had recently become available. Through BDFA they would learn about Enzyme Replacement Therapy. A child that has CLN2 Batten disease is missing an important enzyme that is responsible for cleaning wastes from cells in the body. In particular, this has a big impact on the cells in a child’s brain as they are not able to rid themselves of the waste. Enzyme Replacement is a pioneering procedure that uses a solution that takes the place of the missing enzyme. The solution is administered directly through a device which sits inside the skull. Nicole would gain entrance into the program on a compassionate use basis and she would begin therapy in January of 2017 at the Great Ormond Street Hospital for children.
How difficult would that have been to deal with the realization, back on that day in December. Jessica also had Batten disease. She was only a baby. A time meant to honor Nicole and to further process their feelings had turned into an even larger nightmare. Gail explains the feelings of desperation and the resolve that would follow, “I remember pacing the corridor outside our hotel room, hysterically phoning our social worker, asking her ‘what are we going to do?’. We spoke to Harriet at the BDFA from the airport, as I was desperate for someone to start looking at how we could get Jessica on treatment like Nicole. Through the devastation, we jumped into practical mode and wanted as many people looking into it as we could.” Little Jessica was so young that it was well before the time that she would start showing symptoms of the disease. In a very real sense, Nicole would become a hero to her baby sister, even if they were both unaware of that fact. Why would I say something like this? Two things were in Jessica’s favor. One was that she would be diagnosed before symptoms appeared. The other was that Jessica could potentially begin to receive enzyme replacement before any damage could be done. The goal would be to get Jessica started as soon as possible. It had taken over two years to determine the cause of Nicole’s symptoms and there had been a progression into the disease. Nicole had paved the way for early intervention towards her little sister.
Beautiful Nicole would be given one of the final places within the compassionate use program for enzyme replacement. Little Jessica was another story all together. A child had to be at least 3 years of age to begin the trial and Jessica was only 15 months old. Besides this, all of the places in the program that Nicole was in had been taken. There was no place that Jessica would fit into but this would not stop her parents from working on getting her what she needed. They would begin a desperate search to get Jessica accepted into a program. In the course of time, Matthew and Gail would learn that there were sibling programs in Ohio of the USA and in Hamburg, Germany. Both of these options would each present a different challenge. That is if they could even get Jessica into one of these programs. There are however, not too many challenges that these families will not accept on behalf of their children. Even with this in mind, an exception would have to be made for Jessica to be accepted into a program. The best option would, of course, be the trial that was taking place in Hamburg. This even though the travel time by air, all total, would be around eight hours. The Riches began calling the BDFA and BioMarin, which is the company responsible for the clinical trial. They also called the doctors in Hamburg that were involved with the program. It would not be easy. Nicole was already getting her treatments done in London every two weeks at GOSH. That, in itself, would be enough with traveling there from Newcastle every other week.
It didn’t matter how difficult the circumstances though, the Riches pressed on because they had no other choice. Gail explains the outcome with the following, “It was a difficult time for us because we knew that time was of the essence and Jessica didn’t fit anywhere. So, after many emails and phone calls, Biomarin agreed to amend the protocol and reduce the age limit to allow Jessica to join the trial….”. Jessica would begin to receive Enzyme Replacement Therapy at only 21 months of age. This would take place before any symptoms whatsoever began to appear and that was so important. Jessica would “pave the way” for other children to receive enzyme replacement safely at a younger age. Just like her big sister had, Jessica would need to undergo the surgery. This was to implant the port that is needed to accept the fluid for enzyme replacement. There on the operating table would be this tiny child who had shown no symptoms of Batten disease. Gail stated that for Matthew, signing the consent was the hardest thing that he would ever have to do. She was still a baby and still perfectly healthy but it had to be done. It was the best thing for her. Something that was even more challenging was the fact that the Riches would have had two children receiving enzyme replacement in two different countries. Can you imagine having to travel a distance with one child that is three hours one way and then, also having to travel to Germany with the other child? Of course, the hope would be that Jessica could one day have her treatments done at the same location as her sister.
That would not take place right away or even soon however. Jessica would travel with one or both of her parents to Hamburg for ten months before a sibling program would open up at GOSH in London. At first, both Matthew and Gail would travel with Jessica but that would prove to be too much. Germany one week and London the next. We haven’t mentioned Louis too much yet. We haven’t talked about the effect all of this would have on him. So much time would be taken to deal with the issues that surrounded his two sisters. There is always a challenge for the siblings that are unaffected and making sure that they are not neglected is important. For this reason, Matthew and Gail would make the decision that only one of them would fly to Germany with Jessica. That responsibility would be taken up by Matthew as he traveled with Jessica every other week to her infusions. This would ensure that Louis had more time with his mam and less time of being left in someone else's care. They would also make sure that both girls were receiving their treatments in the same week so that they had more time together at home as a family. Gail stated that at the beginning, the girls were receiving the same treatment on the same day, only in different countries. Matthew, in looking at the number of flights that had been taken in that ten month period, placed it at around 300 flights. There were no direct flights available from Newcastle to Hamburg and the locations of the connecting flights are too numerous to list here. You do get the idea though. Life was very hectic and this family would have to warrior on.
A Family Together
The commitment on the part of Matthew should make it obvious to everyone that he is an outstanding father who loves his family! Obviously, I have personally, never been to the Great Ormond Street Hospital in London. I have read about it and I have read comments made by people that have been there. It is a very outstanding hospital with a rich history of caring for children. It is a very special place and they would open up a siblings program, just as was stated earlier. Ten months after Jessica began her treatments, she would begin to get her infusions at the same time that her big sister would receive them. At the same hospital! There were no more flights to jump on every other week and less separation time as a family. Gail said that Jessica “transferred smoothly and without any apprehension”. She would also refer to her as being “one adaptable little lady” and I might add a darling little lady as well. So often, I use words like “special” and “amazing” to describe these children who endure more in their young lives than most adults will see in a lifetime. Their innocent little ways remain, even as they go through everything that they encounter. There truly is something special about them. Gail describes what she sees in both of her little girls with this, “Both girls are so tolerant, so resilient and super brave. We are in awe of everything they endure, always with a smile on their faces. We get our strength from them.” Gail said that they are currently talking to their local hospital, hoping that the girls will soon be able to receive their treatment there.
What a difference that would make as the travel time would be reduced to a mere fifteen minutes. That would be a great reward for all that they have had to endure. From Germany, to London, and then to treatments at home in Newcastle. This would be best for everyone and that includes Louis who has had to hang in there. He has been such a trooper! So often, the siblings of children that have special needs have to take a back seat to the kids that battle. The attention that is required often robs them of the attention that is due to them. Both Matthew and Gail are intelligent people who understand that this can be a real issue. They take the necessary measures to make sure that Louis knows how special he is. Here is just a little of what Gail had to say about her son, “Louis is 10 years old now, and we cannot put into words how much we love him and how proud we are of him. He is a sensitive, thoughtful boy, who has had his struggles watching his sisters go through what they have. Surgery, testing, regular hospital visits, we always make sure we make time just for Louis.” The Riches make it a priority to spend time with Louis as the regimen of a Batten family’s life continues on. It began four years ago and continues to this day. With all of the feedings for Nicole and all of the special medical needs, they make sure that Louis is not forgotten about. Louis loves playing chess with his father and going on bike rides with his mam and dad. He is a rugby player on a local team and is involved in the Cubs. He also enjoys time spent with his mam and dad during family movie night. Louis loves going to school and being with his classmates.
Matthew and Gail get the affirmation that they are doing a good job with Louis through the comments that they receive from others. Louis is considered to be polite, well mannered, and considerate. This is the kind of thing that takes place as parents take the time to show a child that he or she is loved. Louis, of course, knows what Batten disease is and he knows what it does to children. He has watched the effects of the disease take their course with Nicole. Louis has watched as his sister has gone from running and playing to losing her skills and becoming dependent on others. Gail stated that it is difficult and heartbreaking to see how he is affected by this. She went on to say that Louis would often remark about how he would send fighter jets into Nicole’s brain if he could. This, to blast the disease with something that it hates in order to destroy it. In his youthful way of thinking, he tries to think of ways to battle Batten disease. Louis is inquisitive and asks a lot of questions. Matthew and Gail have always done their best to answer his questions in an age appropriate manner. What do you do when a child asks you if his little sister is going to die? How do you deal with something like that? This is just another kind of challenge that is faced by Batten families. Gail said that hearing a question like this was like an arrow had been placed in their hearts. Louis has grown in his understanding of Batten disease over time and the Riches have helped him in that way. He is a young man that most any parent would be proud to have for a son. Obviously, he is a special big brother as well.
As Matthew and Gail began this journey, they had found a lot of support within the medical community for what they were going through. People wanted to help in every place from Nicole’s school to the various doctor’s offices. There was however, not a lot of knowledge when it came to Batten disease. Being only one of thousands of rare diseases that affect children made a difference. The disease does not always come to mind when doctors are looking at symptoms. What they see is most often misdiagnosed as being something else as the search for answers continues. The BDFA (Batten Disease Family Association) is trying to change this. For the Riches part, they formed a non-profit foundation named the Nicole and Jessica Rich Foundation in January of 2017. They wanted the work that they do to be a legacy on behalf of their two beautiful daughters as they attempt to have a positive impact. This, on the lives of those who battle Batten as they raise awareness and funding. They want to do things to support research, the BDFA, and to help with their daughters' needs. Like with many of the other non-profits there in the UK, the Rich family, through their foundation, has organized some impressive events to raise funds. From holding the world’s largest half marathon to having a climb to the top of the highest peak in the UK. From something as difficult as an event involving an assault course to something as simple as a Christmas coffee morning, they do a few fundraising events each year. Like it has been this year for every non-profit, their’s has been impacted by Covid-19.
This has obviously not dampened Gail’s spirit. You can see that as she talks about her favorite event and the impact that the events have on them as a family. In her words, “The most special event to us is our fundraising ball which we have hosted for the past three years, ‘The Sweetheart Ball’ named after our sweethearts! 2020 would have been our fourth event, but due to Covid-19, it couldn’t go ahead so we are committed to make the 2021 Sweetheart Ball the best yet!!! We put our hearts and souls into organising and planning the event so it means the world to us to see the amount of support there is for our little girls and our family on that night, it is truly humbling.” They are proud of what the foundation has been able to do and so appreciative of all of the wonderful support they have received. I can only imagine that people look at those two little faces, and knowing the plight of these girls, they only want to help. Gail continues with these words about the support that they are receiving, “We have been blown away by the level of support we have been shown, people are always thinking of us and how they can help. It really makes you feel like you are not alone and there are so many people who want to help you make a difference. We are so thankful for every penny.” The funds that have been raised has assisted in the Riches being able to afford specialized equipment. Things like a bath seat and a car seat for Nicole.
In addition to lending support monetarily to the BDFA, they donated a sum of money to a research team at UCL London in 2018. This funding went to a gene therapy research project meant for saving children’s eyesight. The Nicole and Jessica Rich Foundation will continue to give to projects that appear to be worthwhile as they are able, and will continue to aid families that have children that battle Batten. Fundraising and raising awareness are two things that they will never stop doing as the foundation seeks to make a difference. We never know what life will bring our way and the Riches are making the best of what life has brought to them. They have fought battles for kids that battle Batten alongside other families within the UK. The most notable battle was the fight to secure the funding of Brineuera (enzyme replacement) through the NHS (National Health Service). The fight to have this treatment funded was exhausting for all of the families that were involved. The reasons why this was so important should be obvious. That being the little faces with trusting eyes that look up to their parents for help. This pursuit was worth every bit of energy that it took on behalf of the Rich family as they joined with others in this cause. Those that battle Batten alongside their children are a special bunch of people. Only they can truly know what it is like to go through what is referred to as a journey through Batten disease. I’ve been told that in many ways, the road that these families travel is a lonely one. The loneliness that is sometimes felt is lessened by knowing that there are others that know what it is like and who are willing to come alongside. It also helps to know that there are people who try their best to be empathetic and helpful.
Important to the Rich family are the people that they have connected with through this hardship. Matthew and Gail are two people that just wanted to have a family together. They have been asked, however, to deal with something that most will never know about. I liked what Gail had to say as she shared her thoughts. Here it is in her own words, “Ours is a difficult road but we are never alone. We have each other, we have our friends and family. We also have the wonderful Batten Disease community, who have become like family. There are few people in the world who know what it is like, then even less who know how it feels to have the diagnosis twice. Through devastating circumstances, we have connected with some truly wonderful, caring and inspiring people, who we would never have known had it not been for our daughters illness.” Like most of the families, the Riches had never heard of Batten disease before they were given the news concerning Nicole. Their grief was then multiplied by two. There is no telling what their lives would have been like if Batten had not reared it’s monstrous self into their existence. They were at first, “carefree and spontaneous” but now, their lives are regimented. It revolves around feedings and the giving of medications, this not to mention the trips for the infusions. It is not what they thought that it would be but again, they are making the most of the situation that they have been given. There are reasons to rejoice and embrace the life that they have, but daily, there are also struggles.
Matthew and Gail would be the first to tell you that they have their good days and their bad days. You will never know how strong you can be until you have no other choice! Gail shared the most difficult part of their journey to date with this, “The hardest part of our lives has been to watch our beautiful Nicole lose her abilities. Then see her little sister doing all of the things she once did. We are in the heart breaking situation of being able to compare our daughters who are both at very different stages of the disease. This is why we feel so passionately about early diagnosis because we see the difference it makes to our daughters every single day. It is hard on our emotions because we are rejoicing for Jessica and all she is achieving, but we feel heartbroken for Nicole that those moments were taken away from her because of Batten Disease.” Gail would go on to talk about the fact that in spite of what the disease has taken from Nicole, she remains bright and beautiful. That is so easy to see, even from this great distance away. She is still, very much so a porcelain doll with her big brown eyes that speak love to those who come in contact with her. As I alluded to at the beginning of this story, there has, in a certain way, been a reversal of roles in the relationship between the two sisters. You will frequently see Jessica hovering around her big sister, ready to help her in whatever way she can. The bond between these two sisters is strong and very special. For Nicole’s part, she is the heroine to her little sister. This is because she led the way and allowed Jessica to enjoy a quality of life that she herself has missed out on.
Every time I have chatted with Gail, or have listened to her voice messages, I see or hear a person who is trying her very best to keep things on the positive. Life is not what they would have expected it to be but they are making every day count through teamwork and an emphasis on family. I have found her to be a remarkable person who has made the most of a difficult situation. Matthew and Gail are truly a team. With the following, she explains a little bit more about the adjustment they have had to make, “Getting a diagnosis like Batten Disease quickly makes you re-evaluate your priorities and you soon know what is important in life and what is not. It is not the life we planned or envisioned when we had a family, but as we had to learn, you are forced to accept your life will never be the same. You try your hardest not to look back and instead, try your very best to make every day count. The happiness of our children is all that matters. We can’t control the disease, but we control how we respond.” As a general rule, Batten families do not look too far into the future. Rather, they make each day count. Some deal with the challenges by pouring themselves into working as advocates for the community that they are a part of. Gail puts it like this, “We live each day with hope in our hearts and we will never stop doing everything we can to find a cure for this horrendous disease.” The Riches feel like it is a waste of energy to worry about what might happen. They stay focused and handle things in the most practical way that they can.
It all began as a simple outing in a public place that brought two people together. They had likes and interests in common and they fell in love. Finding a life together, they both loved the idea of having children and being a family. Wow! It is sometimes staggering to think about how quickly our lives can change. A chain of events can be set off by a single occurrence and then, we are changed forever. Gail had found her place. Being a mam to three beautiful children had brought her more fulfillment than anything else in life could. There was much that they would look forward to but more would come their way than Matthew and Gail could have ever expected. The love that they have for these three children is stronger than words can describe. Again, Gail does an able job of sharing her feelings with us with the following, “My gorgeous girls, my world….along with their big brother. One of my favourite sayings is ‘the most precious jewels you can have around your neck are the arms of your children’, and this is so true. I cherish every day, every smile, every cuddle, every bedtime, every single part of being a mother is a blessing. Hearing the words ‘Love you Mammy’ is the best thing in the world.” Even though Nicole has never been able to say those words, you can just see the love that she has for her family in her eyes. I can see it too, even from such a great distance away. She is just so beautiful.
The Riches are grateful for every day that they are given with these three beautiful little souls. Watching the bond that exists between the three of them brings so much joy and strength for another day. There is nothing that this couple will not do for their children and they will be there for them, every step of the way. One moment at a time, taking each day as it comes their way. Life for all of us is so short and we can't waste time worrying about what we cannot change. Undying love and a bond that cannot be broken. There I go again. These kids do change people. I know that telling these stories has changed me. There is sadness along the way. There are difficulties and very trying circumstances associated with having children that battle a rare disease like Batten. It would be so easy to make that the entire focus of these stories, however doing so would not paint a complete picture. Beautiful are the details that reveal the depth of feeling that surrounds these children. The depth of love that exists between the kids that battle Batten and those that care for them is something that is beautiful. The life of both Nicole and Jessica is a beautiful story. This is because both of their lives embody all of the beautiful details that we have talked about.
Life is not what Matthew and Gail expected that it would be. They, along with their children, are on a journey that they once were unaware of. The circumstances that they find themselves in has, in part, changed who they are as people and the direction that they are going in. Never, could they have thought about being rare disease advocates. In all likelihood, the thought had never entered their minds. Who knows what number of people they will affect by sharing the details of their journey. How will their lives be affected by being involved in the lives of others? Life is a lot better when you look beyond yourself and try to have a positive impact on other people. The Rich family is doing exactly that, even in the midst of some very trying circumstances. I am thankful for the researchers that have brought about the treatment that Nicole and Jessica receive. It is making lives better, one family at a time but we do need a cure so badly. For the families, that can’t come soon enough. Speaking of families, this is a very special one in yet, another special place. I am grateful for every opportunity I am given to be involved in a community that I care deeply about. Thanks for taking the time to read this story. Thanks Gail for helping me to tell it. All my best to you, Matthew, and those three very special children of yours!
Blogger and Advocate
Is it them or is it us? Is it the feelings that they produce in us or is it that there is something truly special about them? I think that the answer has to be all of the above. It is them, but they also do something to us. Once you give a little piece of your heart to these kids and their families, they come close to taking all of it. Let it happen!
I would like to welcome to my blog Sanfilippo families, as well as, families in other MPS communities. I am grateful for every opportunity to write about those who battle an MPS disorder. This, of course, alongside their special children. This is a labor of love that is extended to each one of you! Scroll down to see MPS family titles.
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...
Take the titles listed below and go to month and year listed in the archives.
January 2017 -
*The Story Between The Lines
February 2017 -
*I Know Just Where You Are
*My Kind Of Royalty
March 2017 -
*The Caring Type
*Face to face: A Fairy-tale
April 2017 -
*No Ordinary Love
*A Rare Friendship
May 2017 -
*Tyrell and The Rare Love Story
June 2017 -
*Seth and The Healing
July 2017 -
*Little John and The Reason
*Sands and The Scottish Princess
August 2017 -
*A Rare Kind of Devotion
*The Giggle Box
September 2017 -
*Sweet Montanna and the Status
October 2017 -
*The Blog About My Blog
November 2017 -
*Lydia, I Love You!
*Kayden And The Change In Plans
December 2017 -
*Those That Are Like Them
January 2018 -
*Nora Skye - I Know Just Where You Are
*Brock and The Priceless Memories
*Jamesy Boy and The Treatment
*The Priceless Princess From Beyond
*Forever Royal - Part One
*Forever Royal - Part Two
*That Kind of Love - Conner's Story
*What I See (She is a Butterfly)
*No Longer Alone - Kristiina's Story
*Things We've Talked About - Oscar's Story
*The Reason Why - Hannah's Story
*Kayla's Perfect Princess - Breanna's Story
*Where Do I Go From Here? - An information only blog
*Warriors Unaware - Mia and Kaleb
*Her Little Story
*Someone Like Raelynn
*Forever Royal - The Final Version
*Awfully Beautiful - The Life of Noah and Laine
*A Lifetime of Love - Tegen's Destiny
*Forever Beautiful - Gabi's Story
*The Miracle Baby - Amelia's Story
*Dylan's Life - A Small Town Story
*Princess Grace and the Challenges
*Anything for Tessa - Her Story
*Haley Bug's Battle - A Family Story
*Journey Unexpected - The Rich Family
*My Life, My Everything - Lydia Rose
*A Love That Hurts - Shashka's Story
Sanfilippo Syndrome and other MPS family titles -
*The Beautiful Light of Haidyn Grace
*Any Distance Traveled - Reagan's Story
*The Missing Someone - Kiernan's Story
Archives - Use title listed above with the applicable month and year.
Highlights from the beginning of gregster60.com -
Benjamin's Daddy - March and June of 2016 - Revised May 2017. Titled Benjamin's Daddy Revised.
My Fearless Adventure - April and May of 2016
A Different Type of Warrior - January 2017
Larry - February 2017