“I still remember the phone call many years ago. My mother answered the phone, and I was sitting on the couch. When I heard the words Battens Disease – my heart sank to my toes. Although I didn’t know much about the disease at that time, I did know it was fatal. I couldn’t come to terms with what it would mean over the next few years, and as I held my daughter (who is only a year younger) I cried and I cried. My heart went out to my cousin Natalie – who I knew would face the next years bravely, but at the same time – It would be the hardest thing she would have to do.”
The above quote was taken from a short article written by Chantelle Mac Isaac of The Gulf News. The Gulf News is a newspaper that is based out of Port Aux Basque, Newfoundland. For her the short article was very personal, and for me, very moving to read.
Why do I continue to tell these stories in my blog? What could possibly be the benefit of taking the time to write about children and young adults whose stories generally end the same way? Batten disease has no cure. Yes, there are some very positive things happening in the community and there is reason for guarded optimism but a cure seems so far away. When a child is diagnosed with the disease, they along with their families, begin a journey that will end with the child gaining their wings.
Although the outcome is generally the same, each story is filled with details that I am moved by. They create these “moments” that I experience with every story that I have done. I was chatting with my friend Pamela about this and I stated that I fall in love, one family at a time. In reply she said that “Yes it will do that to you, one family at a time.” She would also say that “tears are part of our life....this coming from a lady who never shed tears till Casen” (her grandson that was lost to Battens). She added that now, for her, they never stop. That to her is also for every child that has faced the disease.
There is plenty to be concerned with and to be moved by these days. I often spend other parts of my day reading war stories. They are most always filled with sadness, sacrifice, and dying. Why is it that I am so moved by the stories of these children? It’s the details of love and devotion on the part of the families that I find so compelling. It is also knowing what these children go through and how they battle through each day with dignity, honor, and grace. Each day they fight like the little warriors that they are. Often times they do it while keeping a smile on their little faces. Each child and each family create a story in which the details are different. They take place all over the world, under varying conditions and circumstances. These children and young adults are the reason! Telling their stories has become my passion. If you read my blog on a regular basis you might think that I sound like a broken record by repeating thoughts like these. All I can say is to get used to it because I feel as if they are always worth including.
Even from as far away as here in the Pacific Northwest in the United States, Channel-Port Aux Basques appears to be an extremely beautiful place. It is a town that is growing and which has a population of just over 4300 residents. It’s shoreline views are just breathtaking and the channel is home to a port that has provided a means for major shipping to Newfoundland and Labrador since the late 1890s. It is a proud part of the country of Canada, our neighbors to the North. Within the larger story of such a busy place, there are always other stories that for me are no less significant and therefore worth telling. The life of little John Mac Isaac Harvey is one such story.
John was born in Cambridge, Ontario on April 21st of the year 2007. He was a healthy and beautiful baby boy. He had all his little fingers and toes and he was covered in that pink and perfect baby skin. His appearance into this world brought with it a lot smiles from his all family, but especially from his mother Natalie. She had every expectation that John would develop and grow into the little man that she had dreamed of. In fact little John would continue to achieve all his milestones except in the area of his speech. In that area he was a little off but Natalie was assured that John’s speech would come around as he continued to grow.
At eight months of age John would move with his mother back to Newfoundland where she would begin to raise John as a single mother. Life happens and it almost always brings challenges with it. Like any little person John was the center of attention. All the things that he did were just adorable to watch. You could see his inquisitive little mind at work.
As he played, he began to take in all the wonders of his surroundings. There is no doubt that he was filled with questions that began with the words What and Why. His big cousin Chantelle would say this about little John in her article for The Gulf News,
“When John was born he learned to walk, to talk, to play and to laugh just like any other child. You would not know or suspect an underlying disease. Like many children, he would jump into your lap to show off his toys, to be read to, to snuggle and to be tickled.”
It has been a long time but I still remember what it was like to have little people around. With the exception frequent illness’ that they sometimes bring, they are a constant source of joy and amazement. Kids running and being kids in public still bring a smile to my face. I can visualize what it must have been like to have little John in the house. The future looked so bright for him and who would expect anything else?
That was until March 5th of 2010, which was just before his third birthday. That was the day that John had his first Grand Mal seizure. He would go on to have up to 20 in less than a year. His mother, Natalie, would of course seek medical help for getting answers.
The testing on little John would include blood work, an MRI, and a CAT scan. All of the tests results were inconclusive. The neurologist wanted John to be tested for ALS, however Natalie insisted that he be tested for Batten Disease. What? Why Batten Disease?
All the stories that I have done so far have talked about families on a search for answers. Their search would lead them to the shocking revelation that their child had a disease that was fatal and had no cure. The search most often times leads to a misdiagnosis and then to finding out that their child has Batten Disease. It often takes a long time and is agonizing to the parents and family. But this time things were different. You see Natalie knew what Batten Disease was and it was in the back of her mind as being a possibility. Why?
Well you see Batten Disease ran in her ex-mates family. His father's first cousin had two children that had been diagnosed with Battens long before she would get pregnant with John. At that time they were told that in order to pass Battens on they had to be closely related.
Counting thirty years of family history, there have been six confirmed cases of Battens. All who were affected were 3rd and 4th cousins. This is what led Natalie to demand that her son be tested. She had first hand knowledge. There's another little girl in their town that is John's 3rd cousin. She was diagnosed a year ago. All of them had or have CLN2 (Late Infantile Batten Disease).
The MRI and other testing was performed during October of 2010. The means of testing for Batten Disease came in the form of a muscle biopsy. The test results would come back in less than two weeks and they would confirm Natalie’s worst fears. They revealed that little John had Batten Disease. Just like with every other young mother of a Batten child, the date of diagnoses would be firmly placed in her memory forever. That day was March 23, 2011.
Regardless of the fact that Battens was in the back of Natalie’s mind, she was totally in shock and completely devastated upon receiving the news. She knew exactly what the diagnoses meant. Natalie knew the road that they would travel as a family and what the outcome would be for little John. She was thinking at the time that no parent should have to watch their child die a slow painful death. There were so many questions that filled her mind. Natalie had no idea how she could get through it as a single parent. The entire situation brought on feelings of sadness and concern for the future.
Together the family would begin a journey. It would be tragic in a sense but it would also be one that would teach them so many things. Things about love and life. Things like how to serve and care for the most precious of possessions under the most difficult of circumstances. Sacrifice.
Natalie would become a warrior mother. Like every other responsible parent that is called to this type of action. She would stay by John’s side when needed, day and night. Whatever he needed, she was there. The medical community often times brings with it a mixed bag when it comes to physicians and their level of compassion. Natalie would tell me that the neurologist would suggest that John be placed in a home or hospital.
She put it to me like this, “I Won't lie, I flipped out at the doctor as he suggested, and continued to suggest every time we went for appointments, that I should really consider placing John in a home or hospital and leave him there. His reasoning was this was going to be a long battle and I should not put myself or my family though it. Needless to say he found out who I was.” That wasn’t going to happen! Way to go Natalie!!!
She would go on to say this, “The last time he suggested it I totally lost it and said things that I honestly don't remember”. Natalie would tell me that she used some pretty choice words and scared everyone that had accompanied her on that visit. How do you spell warrior mother? The same way you spell Natalie!
While on this journey Natalie would find real love. She would meet a man named Kelly Francis. Most men, I think, would have been afraid of the level of commitment necessary to be in a relationship with Natalie at such a time in her life. To a lot of men, taking on a woman's children in a relationship is a necessary inconvenience. What if one of them is a child with special needs? Even more challenging, what if the child has a terminal disease? Natalie has told me that Kelly not only took on the role of stepfather to little John but that he would do an excellent job of it. I have no doubt that he helped to steady Natalie during a most difficult time. This could say a couple things. One is that Kelly is a very caring and selfless individual. The other is that Natalie was well worth the extra commitment. From what I can see, both statements are true!
The biggest challenges would be met by little John. Like all the other little Batten warriors before him he would show people his strength and resilience throughout his time in the battle. John would first lose his ability to walk unassisted within two months of being diagnosed. He was able to walk with help and crawl and his mom said that he could get around faster than she could. Natalie would tell me that his first six months after the diagnoses were not really that bad. Then in November of 2011 little John would end up getting Kawasaki disease. Kawasaki disease causes inflammation in the walls of medium-sized arteries throughout the body. It primarily affects children. The inflammation tends to affect the coronary arteries, which supply blood to the heart muscle. Natalie almost lost him at that point but amazingly, he recovered. She would put it like this, “He showed everyone just how strong he was. He bounced right back.”
In doing these stories, I am constantly amazed at how tough and resilient these little people are. How they hang in and persevere. They teach us, in their innocence, how to face battles of the most difficult kind. Little John would be no exception as the disease continued to takes it’s course. Soon, he no longer could even walk with assistance but was still able to crawl. “He did amazing for a long time, with the normal ups and downs.”
Natalie would tell me that by April of 2012 his doctors were worried about his weight and the seizures he was having. He was having severe jerking from Myoclonic seizures. Little John could not gain weight. That is when they decided it was time for his g tube. This is a familiar event in the course of the battle that is Battens. She would go on to say that he was still doing great physically and health-wise. Natalie would recall yet another date. On May twelfth of 2012 his tube was put in. Little John, however, was still able to eat orally for about a year after that. At that point, he was starting to have trouble swallowing. The disease continued to take it’s course but John would continue to show everyone what a fighter he was.
By September 2012 John lost most of his ability to speak. Even with this, John continued to laugh until about late 2013 or early 2014. At that point he ceased to do so and that was another very sad event for Natalie and the family. During this time he would begin to lose his eyesight and Natalie noticed that John would begin to get withdrawn.
One of the many things that these little warriors and their family have in common with one another is the amount of equipment needed to care for them. When the battle is over the equipment remains. By the middle of 2013 Natalie was unable, due to his size, to carry John up and down stairs. He became bedridden. As she would put it, “My living room was turned basically into a hospital room for him.”
This is something that I have talked about before. The amount of care that is required and the amount of love that is shown by these young mothers, as well as, other members of the family. You remember that John’s neurologist suggested that he be placed in a home or hospital. I know that in some situations, there may be no choice. However, for Natalie to do that would have completely erased the beautiful picture that is painted by her actions. Undying love demonstrated on a daily basis. John also had a caretaker.
These kids are so precious.They battle and continue to show their tremendous worth. Every bit of care received is so worth it. In her article Chantelle talked about this very thing, “He became bed ridden and in need of home-care support. But he still went to school, gaining friends, many he might not have known about, but they all knew him. Right up until his passing, he knew the voices of those closest to him. You could see the sparkle in his eye when being spoke to, or having his hair played with.”
In the fall of 2013 John had his first round of pneumonia. It was scary and very sad for Natalie and it would not be his last bought with it. John ended up on oxygen permanently in 2014. He would, however, fight and continue to show his resilience. Natalie put it like this, “He was a major fighter. He fought hard and bounced back after every round of pneumonia and hospital stay.” To live the story day in and day out would make it understandable in regards to the level of recall by Natalie. By 2015 John was legally blind in left eye. He could, however, see bright colors and lights in his right eye until the very end.
Some people might raise questions as to why we would include all the details of the disease as it took it’s course with John. Well for starters it helps people to know what a wretched disease Battens is. It also shows what this family went through. Words like “warrior” and “battle” are not used lightly. It is truly a battle and these kids are true warriors. They are precious in God’s mind and they are in mine as well. Awareness, awareness, awareness!
I have three sons and I love each one of them equally. However, my youngest son Benjamin has special needs. He relies on us for help in things like meals and bathing. We spend a lot of time together and therefore the bond is extra strong between us. The bond exists because of the joy that he brings to the house but it is also because of the fact that he needs us for help in the most basic things. I assume that he will be with us for a long time. This raises a question. What is it like to care for a child that has Batten Disease? To help with the most basic needs of life? To do it for the child's very survival knowing what the eventual outcome will be? To pour yourself out for that child and then to have to say goodbye? This while holding them in your arms or being close by their side.Those questions are what drives my passion for these kids and their families. This is why we advocate for a cure and spread awareness. This is also why I want to honor these families with my writing.
According to Natalie, Little John got sicker more often in the last year of his journey. He would always bounce back (warrior) but the disease really began to take it’s toll on his body. He was slowly getting weaker causing more seizures and pain.
I think that for a parent in this situation they have to be completely torn in two. They anguish over the thought of losing the little person that they had poured themselves out for while caring for them. And yet they want to see the suffering come to an end. They want their child to be at peace. They so wished that their child could be whole again but they realize that the journey is almost over.
I asked Natalie if she could tell me about her final time with John. She said that the end was more than difficult for her. The neurologist took little John off of one his meds in order to add three more. One was morphine and the other two were for seizures. Since the drug that was removed was a narcotic, John suffered wicked withdrawals.
The day that they lost John, Natalie took him to the hospital because he had a high fever (41 degrees centigrade) that would not come down. With the fever came convulsions and seizures. Mercifully John’s family doctor managed to make him comfortable after administering three lots of rescue meds and an IV for pain. Natalie said that the testing they did came back normal, however, the fever would not go away.
It was then that Natalie, along with Kelly and John’s doctor, would have to make a decision that no parent should ever have to consider. Natalie wanted what was best for John. Together they decided that it was in John’s best interest that they not resuscitate him in the event he passed. It was time. He had fought this monster so bravely. It was time for him to receive his heavenly reward.
It shouldn’t amaze me but it does that Natalie remembers specific times of the day for certain events. I have left some of them out. At 12:30 pm John’s doctor (Dr. Thomas) and the nurses urged Natalie to go home to shower and get some rest. John’s personal care giver was there and he was being checked on frequently. Natalie had been up for over 36 hours at this point. She agreed to go home and shower. She wanted to be right back but ended up falling asleep for a couple of hours. At 3:23 pm the nurse called and told her that little John’s breathing had changed. Within the 5 minutes that it took Natalie to get back to the hospital John left for heaven.
It bothered Natalie that she wasn’t there at the time, “That was the hardest thing ever. I wanted to be with him when he went. To hold him and let him know it was okay, and that we would be okay”. Natalie told me that she wanted to tell John that she loved him with every beat of her heart!!
You know what Natalie? He knew. He knew how much you love him and he still does. Your voice is the one that comforted him more than any other through his journey. You were there for him like no one else. You are the one that fought for him. Your bond with little John is one that is like none other and one that will never be broken. To little John there is no person more special or more important that his mommy. He knows!
Natalie, Kelly, John’s big sister Caitlyn, family and friends stayed with their little warrior man until 8:30 pm. Then they had to go.
In Natalie’s words, “John taught so many people so many things about themselves. Things about life and love. He was and will always be A HERO to everyone that knew and loved him.”
I believe in heaven. We often speak in metaphor when describing what we think a child is experiencing when they are taken from us. Please have no doubt, they are experiencing things that the human mind cannot comprehend. Things that are wonderful and beyond words. The type of thing we do understand is spoken so eloquently by John’s cousin Chantelle in her article for The Gulf News,
“He is no longer suffering. That part is left now to family and friends as they mourn the loss of a fighter, who persevered beyond his life expectancy. John has taught us many things in life including just how precious it is, and that at any time it could be taken away from us. John, I know you are up there running, laughing and playing, just as you did years ago and that leaves me with a smile.”
Little John’s family includes his mom Natalie and his stepdad Kelly. His big sister is Caitlyn. She is almost 14 and is pretty as can be. She will be tested to see if she is a carrier of Batten Disease when she is 16 years old. John’s littlest sister is Alexandra. She is three and a half and she is cute as a lady bug! Together they ARE John’s family!
This is little John's story. It was written with the help of two cousins, Chantelle and John's mom Natalie. It was, however, mostly written by the life of little John. His life is and will always be the reason this story exists. These stories change us. At least they change me. Little John, you are the story and you are the reason this story exists.
Little Lydia is one of the little sweet people whose journey I am following. Her mom Alicia Headrick is a single mother who is trying to gather funds to help with Lydia's needs. Lydia is such a Princess!! I just wanted to make people aware of the GoFundMe account that her mom set up for her. The amount is not astronomical and can be done if people help. Here is the link:
I hope that you were touched by this blog article. I know that I was.
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:
Forever Royal - The Final Version
One child in a family with a rare disease is a challenge. What if there is two? Heartbreak multiplied you pick up the cause and do your very best to make a difference. That is how a normal family becomes Forever Royal ...
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
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