“Is it them (Children with Batten) 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!”
Blogger and Advocate
It has been a while since the time that I had last written a story about a child who battles. This has caused me to reflect upon the reasons why I first started to get involved in this way. I have often stated that while greater awareness is important, I really started writing these stories to honor the families who battle alongside their little warriors. I felt honored that people would entrust me with the details of their lives in order to put their stories in writing. The stories were simple in the beginning but they grew in complexity over time. They were also very moving emotionally for me to write. I can remember very clearly, causing myself to tear up because of the words that I chose to include as I was writing. Just thinking about a certain child and what their family was going through would bring me to tears. Sometimes, it would be in a most inconvenient place. Like at a stoplight or pulling into a gas station. Being at work in the shop was maybe the worst place of all. It is easy at times, as you do one story after another, for the details to become just a matter of fact. At a certain point, those types of feelings would not be present as much as I continued to write. That is perhaps a good reason why taking a break for a while was a good thing. I so missed writing about the kids that battle after a while. These children and their resilient ways do amaze me. The way that they adapt to life as they face the changes that take place.
Their bodies go through changes as they battle a rare disease like Batten. Yes, they go through very frustrating circumstances but they do bounce back so often from the adversities that they face. These kids always find a reason to maintain the smile that one would expect in a child. This is in spite of going through things that would cause most adults to lose their joy. These kids are resilient. I had never heard of Batten disease but had been made aware through the life of one little girl. I followed her journey like so many others as she had a large following, both locally and through social media. So many were made aware through her journey. It would be through a series of events that I would learn about her and her family. These events actually started for me as I was following the legacy of a United States Navy SEAL named Adam Brown. A connection was made through social media to a little girl named Ali (sounds like alley). She was from the same small town as Adam was and she was battling CLN2 Batten disease. I had written these words about her for a previous story, “The image is etched in my memory and it will forever be there. I had followed my Batten princess in her journey and I knew from what I was seeing that she had taken a turn for the worst. The post on social media told the story. This created in me a sense of urgency which caused me to develop a plan. I had to meet her and it needed to be soon.…”
We did develop a plan that was very “outside of the box” so to speak. We would make the trip of over 2,000 miles from our state of Oregon to the state of Arkansas. This was in large part to meet Ali and her family. Very sadly, Ali would gain her wings just a short time later. That trip was magical and the entire experience of following this one little girl’s journey changed my life. My eyes would be opened to kids who battle rare diseases. I am a father to a son who has special needs. I’ve stated that his life factors into the love that I have for these other children. What I’ve said in the past is this, “The love that I have for my own son with special needs has lent itself to a love that I have for the kids that battle.” This statement is absolutely true. Something has changed since my last story and that is that my son Benjamin has received a diagnosis for his disorder. This took place through Whole Exome genetic testing. Benjamin was born with a mutation of a recently discovered gene that has been designated as PACS2. Benjamin has PACS2 Syndrome. Developmental delay, facial dysmorphism, and a seizure disorder are things that had set him apart from other children. Now we know and we have a small group from around the world that we are a part of. As it turns out, our Benjamin is one of the oldest known cases. I have a strong appreciation for those in the rare disease community that encouraged me in seeking to have Benjamin tested genetically.
We have more knowledge now concerning our son’s condition and it has made a difference. I feel a connection between our family and all of yours. We have had our challenges along the way and have made sacrifices to keep Benjamin with us all of these years but having him in our lives has been a wonderful experience. Let’s be clear about something. Anything that we have gone through, pales in comparison with the things that a rare disease family will face in the course of their journey. They experience a depth of love for a child not felt by many and will know a level of heartache not known by most of us. There are struggles that a Batten family will go through as they journey with their young warriors. Some of these things are seen in people that are much older. I’m speaking of the degenerative aspects of the disease. My 90 year old father has been in decline and is experiencing some of this type of thing right now. He has, however, lived a good long life. Although these things are difficult to see take place, it’s understood that it’s normal that they do happen at his age. This is a normal part of what we know to be the cycle of life and what we expect can happen with the elderly. I’ve often said that this type of thing should not happen to children. Instead, they should be left to live a carefree life, experiencing childlike fun as they learn life’s lessons. A lysosomal storage disorder like Batten disease can bring about symptoms that should be left to people that are aged.
Childhood is a time of life that is treasured by every family and it is tragic when something like a rare and fatal disease is revealed. In the course of time, love mixed with grief over someone who still remains with you will produce a love that is profound and undying. This creates a bond that is so strong that it can never be broken and memories that can never be taken away. These kids…. their lives are priceless and every effort towards new therapies and a cure should be made. Their fight should be our fight. I know that is the case when it comes to their families. This is also true for those in the research and medical field that are working for their care and a cure. Some good things have taken place in regards to help for kids that battle the CLN2 variant of Batten. There is more that is hoped for and more to be accomplished. Okay, it’s time to get to our story. Like I’ve stated already, my interest in the kids that battle all began with a little girl named Ali. It easily could have started with another little warrior. A warrior princess like Violet. This is a story about her and her family.
Two People Meet
Like it is with almost every story that I’ve written, this one begins with two people that meet and fall in love. That was the case for Peter Jones and Michelle Curran even though it was not love at first sight for them. They were both 17 years old when the two of them would meet. This was at a club named The Grafton in Liverpool England. Their meeting took place in March of 2001, just before Easter. Infamous may be too strong of a word but The Grafton definitely had a reputation of being a rough kind of place. Peter and Michelle were just out to socialize with others and to have a good time. They had no idea that they would one day start a life together, nor what being together would entail. They had spoken a few times at The Grafton and Peter found Michelle to be fit, or attractive. They had kissed one night but things wouldn’t go any further for months. For Michelle’s part, while she thought that Peter was a handsome fellow, she also thought that he was a little bit full of himself. That is opposite of the type of person that Michelle was looking for in life. Her opinion of him would soon change however, as his true character would soon be revealed to her. One day, he would be the father of her children and her best friend in the world. As it would happen, Peter’s best friend lived by Michelle and he would drop her off at her home after the club closed. One night, Peter asked for Michelle’s number as they were leaving and he would soon call. He just knew after that first conversation that she was the one for him. Michelle also felt that one day, they would be man and wife. That would indeed take place in due time.
Peter acknowledges that, in certain respects, they are opposites in their personalities but they would just click as a couple. Peter and Michelle’s love for one another would grow by the day and each of them would become the love of each other’s life. As their relationship progressed, Peter and Michelle would move in together. This was just before they each turned 21 years of age. Life together was good but it wasn’t complete without children. They both had wanted a family and so it would happen that Michelle would be expecting their first child soon after their move. Everything would go well with her first pregnancy and the time would arrive for them to welcome their first child. They would name her Millie. That’s right, Michelle would give birth to a baby girl on the day of October 16th, 2006. Millie was a big baby due to the fact that she was born two weeks beyond her due date, arriving at 9 pounds, and 11 ounces. She was flawless in every way. Although Michelle’s first pregnancy took place without complication, the delivery was a bit more challenging because of Millie’s size at birth and Michelle’s slim frame. Millie’s mum and dad were so happy to see her and she was born a perfect and beautiful baby. Together, they would settle in as a family of three. Life was good. Peter and Michelle enjoyed life together with Millie, spending time with friends and extended family. Holidays together were more special for them now that they had little Millie in their life.
Everything was just better and they hoped to increase the size of their family in the near future. Life was busy with work and play but Peter and Michelle had high hopes for what the future held for them as a family. All of the wonderful things that normally take place were anticipated. Michelle had hoped to have another child before turning 30 and that would happen one day. It would take a couple of years but both her and Peter would be given the news that Michelle was pregnant again. They were both elated to know that they would soon be a family of four. What would they experience over time as a family? Only time would tell but this news was met with a lot of excitement. Again, Michelle would do well with her 2nd pregnancy and one would expect that their next baby would be healthy and whole. Life continued in anticipation and the time would arrive for the next member of their family to be welcomed into this world. The big event would take place on July the 25th of 2015 at Liverpool Women’s Hospital. Guess what? Michelle would give birth to another baby girl and her parents would name her Violet. Just like with her sister Millie, Violet was born with all of her fingers and toes. She also was a flawless and a beautiful little baby girl. Both of Violet’s parents looked at her and thought that she was perfect in every way. She had a perfect complexion and dark hair. To her mum, she looked like a real size little doll. Violet’s dad noticed that she looked just like Millie, only smaller.
As Peter looked at his newborn daughter, he thought that his family was complete at that moment. In fact, four people would be the perfect size family to many. The possibilities for the good things that could take place in their future were many. More time spent together, experiencing all of the milestones that take place in a family home. From the first day of schooling to the graduation from college in some cases. Parents look on with pride as their children grow and achieve their goals. Marriage brings grandchildren and the feelings that surround being together intensify with each addition to the family. These are normal life events that one could expect. They will normally come to pass unless they are interrupted by problems that may yet not be seen. I may be stating the obvious here. Bumps and bruises and even broken bones happen when you raise a child but those are the things that a person might expect to come their way. Life in itself can be a journey but most parents will look forward to the future with joy and optimism when their children are young. Why would it be any different when you had just brought a little girl like Violet into the world? She was just beautiful and also an amazing baby according to her mum. Michelle stated that Violet slept well and she was easy to feed also. Violet’s little temperament was amazing. She was a happy little girl and very loving towards others. She was a little princess from the beginning and she was loved immediately by anyone who came in contact with her.
Life Less Perfect
Early in her development, Violet would thrive in the ways that one would expect a little girl to. She was learning life’s lesson while having fun and looking at the world in wonder. The only issue with her total health was that she had a speech delay. This is a common issue in preschoolers with it being present in as many as 10% of children. Michelle explained that Violet could speak in one word answers using terms like mamma and daddy. She then went on to use simple phrases like “there you go” and “oh mamma, where is green?” She wasn’t where she was expected to be with her speech but Violet was making progress. Something so simple wouldn’t necessarily cause a lot of concern and Michelle thought that they were turning a corner on Violet’s speech delay. But then, an event would take place that would shake them as a family. This would happen on February the 16th of 2019. That is a day that Peter and Michelle will never forget. It was not known at that time but this day would change everyone’s life forever. Life would never be the same. That day was a Tuesday and Michelle had just arrived home at her usual time of 5:30 in the evening. Walking through the door, Michelle walked into the kitchen. Once there, her neighbor came running into the house screaming that little Violet wasn’t breathing. She immediately ran to find her daughter in Peter’s arms. In Michelle’s own words, “I remember running next door where Peter sat with Violet in his arms. She looked so peaceful, I thought we'd already lost her.” Can you imagine the thought of such a thing racing through Michelle’s mind at that time?
Not knowing it right then, this was the beginning of much more that would follow. Everything was not right with this little princess that everyone adored. As it turned out, Violet had vomited and turned blue while her dad was running her a bath after coming home from nursery. Thinking that she was choking, Peter had run out into the street looking for help from neighbors. So many people tried to help that night and it was something that Michelle would never forget. What caused this to happen? Eventually, an ambulance would arrive and Violet would be taken to Alder Hey Children’s Hospital in West Derby, Liverpool. What was revealed is that Violet had a seizure and a pattern started to develop over the next few weeks. Violet would fall asleep and then wake up within the first hour while vomiting. Her eyes would go to the left or right and then she would seem to go back to her normal self. Can you imagine how terrifying this would be as a parent….the heartache over seeing this little girl going through such a thing? The trips to Alder Hey would become a regular occurrence for this family for the near future. All of a sudden, there was an abrupt end to what one would consider to be a normal type of family existence. Peter and Michelle were always on high alert after that first incident and tension increased. There had to be the fear that they might lose Violet to whatever had a grip on her. Violet would eventually be diagnosed with epilepsy and was prescribed a medication called Keppra. Was that all there was to this little girl’s condition? As Violet’s mum, Michelle had her doubts. Her intuition as Violet’s mum told her that there was more to the situation.
Even at that early stage, she thought that there may be more going on with her daughter than just epilepsy. Violet’s initial care was being provided through the NHS, or National Health Service, and Michelle had her doubts about the diagnosis. Wanting to use every resource available, Michelle would seek the help of a private medical provider as she had health insurance through her work. Unfortunately, Michelle felt that this turned out to be just a waste of time. This was because none of the doctors through private medicine had a clue as to what was behind Violet’s symptoms. To make matters worse, they weren’t taking Michelle’s concerns seriously. It is outrageous to think about how she was treated by one doctor in particular. She explains, “I had a doctor laugh in my face when I told him something was off with her, I felt in my gut something wasn't right. He actually laughed at me and said "look at her, nothing’s wrong."’ Along the way, Peter and Michelle were advised to have an MRI performed for Violet as a precaution. This advice was given even though they were assured that there was nothing to worry about. What would seem to be alarming to most of us is the amount of time that it took to have the test performed and also, to receive the results. I have often heard of families, already in distress over their circumstances, having to deal with red tape and denials within a medical system. Between gaining the approval for the MRI and waiting for the results, it would take this family months to get the results of the MRI.
Michelle explained, “The NHS made us wait for weeks before refusing to do the MRI. We then had to go through the longer process of waiting, being referred by an NHS doctor. The man waited 12 weeks for her results.” Weeks turn into months and the level of frustration increases by the day. Michelle would call weekly for some kind of an update but it was to no avail. On one occasion, Michelle would call and be told the following by a receptionist, “if there was anything to worry about you would have heard from us by now.” The MRI would be done in July of 2019 and the results wouldn’t come back until the following September. Peter and Michelle would finally be called to the hospital where Violet was seen by a neurologist and they would be told about the results. Michelle remembers the appointment like it was yesterday. After all of this they were only told that there were some abnormalities found. That is all. Genetic testing would be ordered the same month and that would of course, create another waiting period. At this point, other symptoms had started to creep in for Violet. The seizures increased and Violet was shouting things like the word mamma. One day the shouting sounded off and it was slurred. Violet also started wetting herself and she was stumbling over her own feet. Michelle had just thought it to be a phase that Violet was going through at that time. Looking back now, she knows that it was much more than that.
The Painful Truth
Michelle remembers, “When I look back, I could scream at myself for not clicking on the fact that something more sinister was happening, but at the time everything happened so subtly. Is it crazy to say I can’t pinpoint a moment where I knew it was really, really bad?” In fact, Michelle had been convinced initially that Violet’s symptoms were related to a B12 vitamin deficiency. She would continue to google the symptoms over the time that her and Peter were waiting for the results of the genetic testing. This would take months and that would leave her a lot of time to wonder while she continued her research. One of the things that she ran across as she googled information was a genetic disease named Batten. Both her and Peter would have to be carriers of the same defective gene that causes Batten in a child. That makes Batten disease rare, affecting 2 to 4 children out of 100,000 here in the United States. She mentioned the disease to Peter and they discussed it. They both felt that the odds of Violet having such a thing were more that a million to one but Michelle knew that it was a possibility. The wait for the results of the genetic testing wouldn’t become available until the middle of January in 2020 and that would leave Peter and Michelle with time to wonder. Could Violet really have a genetic disorder? They would have no choice but to press on and put one foot in front of the other but then the day would arrive. Michelle tells us, “January 15th, 2020 will forever be embedded in my brain for all the wrong reasons.”
Michelle’s baby girl, her mini-me, had CLN2 Batten disease. Her thoughts before that were conflicted. She perhaps had already known before the diagnosis that her daughter had Batten. However, she didn’t want to believe it to be possible. Michelle shared, “When I talk to people, I talk about life before that date and life after, and life will never be the same ever again.” Devastation has to be the word for any parent receiving this kind of news. That would certainly be the case for Peter and Michelle. No matter what would take place in the days to follow, it would take time to recover from the news that their little girl had Batten disease. Michelle had already done the research and her and Peter knew what this meant. Their little girl who had become the family princess had a disease for which there was no cure. It was fatal! There had been a groundbreaking treatment developed but they were told only vaguely about it. Peter and Michelle were just too overwhelmed with the news and the doctor didn’t know much about the treatment. The family was given a leaflet about it to read at home but didn’t think much about it at that moment. Michelle explained to me, “….I just thought we were given this devastating news by the hospital and just left to get on with it.” The information that was given to them was very important but it was lost in the grief that they were experiencing at that time. A family can feel so alone and confused at a time like this but help would come soon after the Joneses returned home.
Michelle’s intuition combined with her need for help no doubt caused her to get on the internet as soon as they arrived home. She googled Batten disease and would find the help that she needed almost right away. She would actually find another mum dealing with Batten right there in Liverpool and in addition, would find a page for a couple of other young girls who lived in the UK as well. Their names were Nicole and Jessica Rich and they both also had CLN2 Batten disease. Their parents, Matthew and Gail Rich had started a non-profit foundation in order to help their little girls and to help others at the same time. Gail and Michelle were able to connect over social media and then Gail messaged her phone number to Michelle. Knowing Gail, it’s no wonder that she was a great resource and a fast friend to Michelle. She explains, “Gail was my rock when Violet was first diagnosed, I will always remember calling her the day after Violet’s appointment when we got the news. She was just so positive and made me dust myself off and get off the floor to fight.” And that is how it often happens. People come alongside you and you get picked up. Sometimes, you do so on your own accord but you do what is necessary in order to help your own child. In this case, Michelle had all of Gail’s experience and insight to draw from. Both of her daughters, Nicole and Jessica, had been receiving the new treatment for CLN2 Batten disease. Gail was certain to have had a wealth of experience to learn from.
The therapy for CLN2 involves replacing an enzyme that is missing from the affected child with a solution named Brineura. This solution is infused into the brain through a shunt that is surgically implanted into the child’s head. Can you imagine what it is like for the parents as all of this is taking place. Knowing others that you can draw from as you experience all of this has to be so helpful but life after the diagnosis is still a struggle. Michelle stated, “The days and weeks after her diagnosis were a blur. I remember feeling numb and not being able to process the reality of what was happening. One minute you’re a family of four, you have the nice house, nice car, you go on two holidays a year, the kids have the best clothes and all of a sudden you are traveling to London every two weeks to keep your child alive.” Can any of us that are not in Peter and Michelle’s situation fathom such a thing? How life would change for this family. Both Michelle and Peter were working successfully in good careers. They had a good home and a good family life. Their hard work had brought this family of four the things that people would expect to come their way. All of a sudden, that was interrupted with the news that their daughter had CLN2. Now, Violet would have to have surgery to have the shunt implanted and there would be the infusions every two weeks. These would be done at the Great Ormond Street Hospital in London and that would require a 5 hour trip one way.
There is also the financial cost of dealing with something like this. Michelle explains, “The biggest challenge to us after her diagnosis was cost. Not only were we going through hell after Violet's diagnosis, but as we both worked, we were not entitled to claim our travel expenses. We were paying ridiculous amounts of money to travel across the UK and stay in hotels every two weeks.” To make matters worse, Covid-19 had hit the UK a few weeks into Violet’s diagnosis and as a result, overnight stays had to be self- funded as resources became stretched. Michelle added, “It's something I look back on now and wonder how we did it. We would never of been able to afford it without the support from family and friends.” If you know anything about the staff at the hospital affectionately known as GOSH, you know that they are a great source of help and encouragement. However, the trip to and from had to have been grueling and difficult for a working family. As it is with many families that rely on Brineura treatments for their little warriors, it takes a while for a more local hospital to come on line with the treatment. That would eventually happen for the Joneses as the treatments became available at Manchester Children’s Hospital. After two and a half years of traveling back and forth to London they would then be able to have the treatments done just 45 minutes away. Michelle refers to this as being “life changing”. It is easy to imagine how that would be the case. Michelle finds the staff at Manchester to be amazing stating that “nothing is too much trouble for them”.
Like all families that have a son or daughter with CLN2 Batten, the Jones family has settled into a new normal. Certainly, there are good times together and sweet memories are being made but life is still difficult. There is much involved in caring for a child that has CLN2, or any variant of Batten disease for that matter. All of a sudden, without notice, you become a family with a child that has special needs and those needs are constantly changing. There are infusions every two weeks and a host of other appointments. Medications for the Batten warrior can change constantly in attempts to fend off seizures or to help with a different issue. Changing needs regarding medical equipment are present also. That one person in the family affects all others living in the same home because so much attention is required. That’s what life is like for the family who has a child with special needs. Michelle stated, “Life since diagnosis has been hard. Am I wrong to say I was so bitter?” One needs to try to understand how much the life of a family is interrupted when something like Batten comes along. Michelle shared that it was difficult to be around other families that have normal functioning children. Especially when the children are at play. Michelle shared, “I couldn't stand it. I was so jealous. I probably still am, of people and their seemingly perfect lives.” An event would take place that would help Michelle in this way. That would be the birth of their little boy in May of 2021. He was a darling little man right out of the gate and they would name him Luca.
Baby Luca has been a ray of sunshine during some very dark days for this family. Michelle struggled with being around family and friends with children of the same age but Luca has helped to ease the hurt that she felt. The hurt will never completely go away but having Luca in their lives has helped. Michelle shared that the only negative may be that as Luca develops new skills, it will serve as a reminder of the things that they had hoped to see in Violet. Michelle says that she does have a group of mums that she speaks to most days. These other Batten mums understand exactly what she is going through. As Michelle shared, it’s nice to know others that get what you’re going through and that can relate perfectly to your struggles. Michelle adds to that, “….to be honest, I wouldn’t of got through the past three years without them.” Michelle and the other mums will even spend time together at a hotel from time to time and those times together are priceless. For Peter’s part, he goes about things differently. He’s more quiet about his struggle over Batten disease but it is a struggle nonetheless. He doesn’t spend a lot of time speaking with the other Batten dads. He just gets on with what life has brought his way while doing his absolute best for his family. His relationship with Violet is a special one and one might say that Violet has him wrapped around her little finger. This writer loves his son with special needs. I had wanted a daughter at the time my wife was expecting our Benjamin and I understand why Peter’s relationship with Violet is special.
Peter tries to be the rock of his family and I know that he is that. He shared just a little with me, “Life is hard some days but I keep telling myself if it beats me then it’s won. I stay strong for Violet, Michelle, Millie, and Luca. I will not let it win without a fight and will do everything I can.” Peter had been working successfully in the plumbing profession since leaving school at age 16. He left that job to work as a Taxi driver last year at Christmas time. This was in order to be more flexible for Violet. Peter regularly accompanies Violet during her infusions and that says a lot to me about the kind of dad that he is. The bond that he and Violet share is a special one but this hasn’t been easy for Peter. He had to leave a profession that he loved because of the love that he has for his daughter. This creates more down time for him and that is difficult for a guy who is used to working longer hours. He is a rockstar dad in this writer’s opinion. Peter speaks more about this experience with this, “Me and Violet have a strong bond. She’s always snuggles in with me and we have loads of laughs everyday. The day we got told the news was the worst time of my life so far. Not knowing what would happen. Every time I thought about it I would cry. Thinking, what have we done to deserve this?” And yet, he kept going. You can be sensitive and strong at the same time. Michelle does feel that this experience has drawn them closer together as a family but life is harder for them. It is more difficult to spend time together with the rigorous schedule associated with the infusions and other things related to Violet’s care.
The New Normal
Things have changed drastically in the last 3 years but Peter has emerged as the stable force in the family. Michelle reiterates some of what has already been stated about Peter with this, “He was sent to me for a reason. I could never ask for a more loving, supportive husband. He's an amazing dad to all three kids, but his bond with Violet is priceless. She will only settle in his arms at night time. She only drinks if he holds her cup and she smiles for him more than anyone in the world. He's the most loving, kindest person I have ever met and everyone who meets him loves him.” Michelle continues while mentioning the other part of her support structure, “I'm lucky we are a strong family. Peter always picks me up when I fall, and we are surrounded by the best people. Family and friends are always there for us.” You know we haven’t talked about big sister Millie yet. I had to ask Michelle about her oldest daughter because I want to know more about her. She stated that Millie is the most caring and beautiful person, inside and out. Millie is the image of her father but Michelle says that she is feisty like her mum is. Being quiet and shy, her confidence is growing as she gets older. Her favorite place is at home with her family but she has plans for her own future. Millie is mature for her age and that is a credit to her parents. She might have reason to misbehave or act out. This, because of the attention paid to Violet or the trauma that her condition causes for others.
For Millie, this has never been the case. Instead, She is a help to her mum and dad and to her siblings as well. I bet she realizes that she needs to be that. Michelle shares a little more about Millie with this, “I've never had a call from school with any concerns, and every time I speak to teachers at parents' evening it's always lovely feedback on what a nice, polite girl she is. On days we are out of the house at silly times traveling for treatment, she sets her own alarm and takes herself to school. I never have to remind her to do anything. Perhaps only taking the dog for a walk ha ha. She's an amazing big sister to both her siblings, but she's also an amazing daughter who I'd be lost without. She's my best friend.” Michelle thought back to the time that she lost her mum at age 14 to cancer. The trauma of having something like that happen greatly affected Michelle’s attitude and attendance at school. While understandable for a young girl, Michelle has had no issues at all with Millie in that way. Millie is exceptional and mature beyond her years.
Violet is without a doubt, a beautiful little girl and it’s easy to see even from a distance why she is so loved by her family. I have already stated in so many words but I will do it again here. A child's life should be filled with innocent fun and laughter as life's lessons are learned. It breaks our hearts when they are not left to enjoy life in the way that they should. And still, these kids that battle are so resilient. Michelle shared, “Violet Jones is our inspiration. That little girl blows me away with her daily battles, that she faces every single one with a smile.” Those of us who follow these families will notice something. That is the profound type of love that surrounds these children. Love mixed with heartache produces a type of love that is profound and undying. That is what we see in these families when they are on this type of journey with their little warriors. More will be dealt with than could be expected from anyone. Respect, love, and admiration is due to people like the Jones family. Like so many families that battle, they provide their daughter with everything possible in order for her to fight against Batten disease. Michelle again shares, “Violet may have one of the most devastating conditions, but she does have the most loving team/family behind her that will never stop fighting for her. Her fight is our fight.” Several times I’ve received comments from moms or mums saying that they’d do almost anything to help their little warriors. They would go as far as trading places with their child if they could.
In her own words, Michelle would do exactly that if she could, “I'd swap in seconds with Violet. I wouldn't even have second thoughts, if I could take this away, she would be living a normal, happy life right now.” There is always hope when there are people that care. I have seen changes in the search for a cure since the time that I first started to follow kids with Batten disease. What I had seen first was an increase in the rate of speed at which doctors would find a diagnosis. That was so critical. The start of the clinical trial for enzyme replacement therapy was another big milestone. The hope for the longest time has been that gene replacement therapy would be realized soon. That is one of the best avenues for a cure. It’s been promised but has not yet come about. As of late, there have been greater advances in research and that means gene replacement is close at hand. Maybe this is helped by the fact that the pandemic known as Covid-19 is not as big of a threat as it once was. Who knows! There is real hope in the air for children like Violet. Let’s hope and pray for the best. In the meantime, the Jones family will do their absolute best for this darling girl of theirs. Wouldn’t we all do the same? Michelle speaks of what other people see in Violet, “Everyone who meets her falls in love. She makes us both proud every single day. She is the most loving little girl and has her daddy wrapped around her little finger.” To be honest, I’d be wrapped around her little finger as well!
When I post on social media or write in this blog, I often repeat myself frequently in order to place emphasis on certain thoughts. The following words may seem like just a catchy little phrase but they really do come from the heart. “These kids, they are the most special and they are the most amazing.” Yes, there are other types of battles that affect children but of this one, I was first made aware. These kids are so worth the time and they need to be honored through every means that is possible. I had originally written so much of what I repeat for the kids that battle Batten. These words however, can be extended to children that battle other rare diseases. I understand that this is not an alpha male activity that I’ve involved myself in and I don’t care. I first gave a piece of my heart to one little girl and that affected my way of thinking and the way that I go about living my life. There truly is something special about a kid that battles. The resilience that is present in a child is brought to the surface in a big way when we see them fight their battle. They are so amazing to me! They do produce feelings in us that would not otherwise be present. Violet is precious and I’d hoped for some time to have her story in this blog one day. I’m so glad to be given this opportunity to tell others about her. This is another special family from the United Kingdom and one that I had hoped to write about. Thanks to Peter and Michelle. Thanks to everyone for taking the time to read this and for sharing this family’s story with your friends. Very sincerely,
Your friend and Benjamin’s daddy,