My faraway friend, Jessica Montville, became a member of the Batten community when her beautiful little daughter Nora Skye was diagnosed with the disease. This happened after she began experiencing symptoms. Jessica’s search for answers was not as long and exhausting as many of the others were although the answer that she received was nonetheless very distressing. I love her daughter’s name! Nora Skye is a name that could have easily been taken by an actress who wanted to be known as someone more than of the ordinary. It is the type of name that would endure through time and would be associated with someone who possessed great beauty of a rare kind. The name will always be remembered because of who it belongs to!
There have been several blog stories that I have done in which I have drawn a comparison between my own experience as a father of a special needs child and one who has a child with Batten Disease. In the truest sense, there is no comparison. The knowledge of the fact that a child has a disease for which there is no cure brings with it circumstances that stand alone. The only reason that I have written in this way is to try to relate my own experience as best that I can in an attempt to try and understand what those in the Batten Community go through. I know that my attempt falls short of this.There are of course, other types of little warriors out there that deserve to have their stories told. My son Benjamin has a seizure disorder that is, for the most part, controlled by medication. We suspect that he still suffers from absence seizures in times when things are out of order. His mobility, among other things, has become an issue. I have mentioned it in brief before that there was a time that Benjamin experienced a bout with aspiration pneumonia.
Benjamin came down with the stomach flu or a virus when he was seven years old. Without giving too many details I will tell you that he aspirated into his lungs and not just a little. Whereas this was a one time event for Benjamin it was a very serious one. I know that young children with Batten Disease often experience aspiration pneumonia and it is always a very serious thing. When this happened to Benjamin, we called 911 and our son was taken by ambulance to the nearest hospital. He was almost immediately Life Flighted to a hospital that had the type of equipment needed to treat him. This consisted mainly of a special type of ventilator. Within a few days, he was transferred to a to another facility and put on a less aggressive machine. After he had settled into the first hospital, we consulted with the physician that was treating him. The doctor said that Benjamin couldn’t be any sicker than he was at that time. Of course, he would recover and I am so glad that he did. Where am I headed with all of this?
During the time that we were at that first hospital, we received a pastoral visit. He stopped by to offer support and to check on us and Benjamin. It has been a long time now but I can remember how he had commented about how upset or alarmed we must have been by what was going on. It was a fact that, prior to this time, we had already spent a lot of time with our son at the doctors and at hospitals for a variety of reasons. So our response had apparently taken him by surprise as we might have appeared over confident in our own ability to handle the situation on an emotional level. Perhaps our attitude seemed a little flippant or out of character. It was like if we were saying, “don’t worry, we’ve got this.” I am sure that if things had become worse and we were to have lost Benjamin, we would have been devastated. I think in hindsight, that perhaps we were a little bit naive. For the child with Batten Disease, if aspiration happens, it comes in a time when the disease has progressed into a later stage and the damage can be irreversible. Something like aspiration comes at a time when the family has been through the earlier stages of the disease and all the devastating circumstances that go with it. There is no way that a Batten parent can posses a flippant or careless attitude when this happens, and they never do.
I have often talked about the bond that has developed between me and Benjamin as time has gone by. It has been mentioned before that I actually have three sons and that Benjamin is the youngest. Even if I haven’t always demonstrated it by my actions, I would like to think that I love all three of my son’s equally. In the case of Benjamin, things are a little bit different though. Both his brothers are on their own and are self sufficient, while Benjamin has been under our care his entire life. All the time that we have spent together has, of course, created a special bond. How much more intense is the bond between a Batten warrior and the family? The bond that is created as a result of giving everything that is required for a Batten warrior’s care cannot be broken. This is my observation. Such is the case with Jessica and Nora. The following is a brief account of their story and some of it comes from a previous blog that I did. I felt that Nora deserved a little better than I had previously done for her in February of last year. I wanted to give a little more detail and make it a little bit sweeter because she more than deserves that from me. Here goes,
Baby Nora must have been something to behold as her parents held her in their arms. They were so happy to see her after welcoming her into this world. She was born at Heywood Hospital in Gardner Massachusetts on April 21, 2005. There was only one thing keeping her parents from looking ahead to all that she could accomplish in her life. That was the fact that they were mesmerized by her tiny and most perfect appearance. She was flawless and beautiful. Nora Skye had all her tiny fingers and toes and she was covered in brand new baby skin. It was no small thing that this baby girl had made her entrance into the world as her mother, Jessica, was having problems conceiving. After consulting with doctors and after much time and difficulty the purest of perfection was delivered.
There is no bond that exists like that which is between a mother and her child. Especially between a mother and her daughter. As little Nora Skye continued to grow she captivated her family by all the the very cute and precious things that they observed in her. They had no reason to believe anything other than that she would fulfill all of the dreams that they had for her. Certainly she would make her own decision one day for her future but her family thought nothing other than that her future would be bright. There was no way that Jessica could know what would lie ahead for them. I have now heard the testimonies and comments made by many parents that are either facing the daily struggle of caring for a child that has a rare disease or one who has lost a child to such. The absolute heartache that has been told to me by the now many is heartbreaking to the reader if it is allowed to sink in. I feel that I am personally drawn into these families stories as I write and I do my best to empathize with what they face. My emotions often get the best of me as I think about each one.
Jessica had to be treasuring every moment of little Nora's life as she watched her grow. I am sure that the strong bond between the two of them continued to develop as Nora reached all the normal milestones that each parent looks forward to seeing their child achieve. Each new day would bring another memory to be treasured as the entire family continued to look forward to the future. It wasn’t until the age of three that a sign of what was to come appeared. Nora told her mommy that she could not see. This would be alarming to any parent. It should also be noted here that Nora as a baby experienced tremors and that Jessica was told that Nora was merely vitamin deficient at that time. She did not know that what her baby was experiencing is a piece of a puzzle that no parent would want to have to put together.
The truth, however, would soon be found out as changes started to take place. Nora was taken to her primary care doctor who felt she was fine. It was while she was at a neurology appointment that Jessica was advised that she should have Nora’s eyes checked and that was not all. The most notable, and certainly the most alarming, concern was that Nora began to have seizures. Not just one or two, but more than could be counted accurately. This would lead to an early diagnosis of Uncontrolled Seizures. Like a lot of parents that go through such an experience, the exact day becomes etched in a person's memory. Jessica remembers that day which she will never forget. June the 25th, 2009. You’ve heard the stories before about how a parent will go on an exhausting search for answers, however for Jessica, that would not be the case. The Neurologists that were consulted would be quick to determine what was going on with precious Nora Skye. The early diagnoses would eventually give way to the news that Nora had Late Infantile Batten Disease (CLN2). Jessica was informed that her daughter has a disease that is fatal and for which there is no cure. She was all of a sudden faced with the knowledge that her child could be gone as early as age 6. The variant of Batten Disease that Nora had would bring with it a life expectancy of no more than twelve years at the time she was diagnosed.
What would you do if this was your child? How would you respond? Jessica was seized by emotion after receiving the diagnosis. Nora was her firstborn child and one that she had dreamed about having. She came into this world through a fair amount of difficulty and she was Jessica’s darling Nora Skye. Jessica would tell me that she spent the first day overwhelmed and in tears after receiving the news. Those emotions, however, would give way to determination. That determination would drive her to do whatever she had to do to save her daughter. Jessica would tell me that her thinking at that moment in time was that she would save Nora, even if it killed her while doing so.
As somebody that doesn't know Jessica and her family really well, I can only use the examples of others whose stories I have heard in understanding what the news they received did to them at that time. How devastated they must have been to know that their most perfect little person would go through all the stages of a most dreadful childhood disease such as Battens. My heart aches for any parent that receives this kind of news. Anybody that is a parent of a Special Needs child understands that strength of the bond that develops as a result of their child's dependency on them. However, for the parent of a child that has a rare and incurable disease it becomes even stronger. I am thinking that the constant care for the child's very survival on a daily basis and the time spent by their side only serves to increase the bond exponentially. We are not at all members of the Batten Disease community, but I love my special needs son Benjamin so much! I have to tell all of you that are parents of a rare child how much I respect you. The love and respect increases with each story that I hear or read about. To know that there is a clock that is ticking and that your time is limited. To know the eventual outcome and to make every moment together memorable. The determination to fight for your child in every way possible. There is nothing to compare it with. When it comes to Nora and Jessica, it needs to be said that Jessica battled for her daughter in every way that she could. If you were a doctor or a nurse, a teacher or a bus driver, you better have had your act together when it came to your care for Nora. Professionals of all kinds needed to have their facts straight or this momma bear would see through everything.
It was apparent to those who followed the journey of Nora Skye that emotions often ran high. This was due to all of the frustrating circumstance that surrounded mother and child as they sought to fight off the monster that is Batten Disease. Nora Skye often led a lonely existence as other children and adults avoided her out of pure ignorance. These people do not understand what a tremendous gift that each one of these children are to us. Each carries with them the title Prince or Princess. They are rock stars, each and everyone. To stand in service to them is indeed a great privilege that should never be taken for granted. They are little warriors in the truest sense. Nora Skye was and forever will be beautiful to those who followed her journey. I know that she is to this guy. She would understand the love that those who knew her best had for her.
There was never any doubt! Nora would go through all of the decline that one would expect with Late Infantile Battens. This included loss of most all of her abilities, seizures, and lung congestion. But you know what? She battled hard and she did so with a smile on her sweet face for most of her life. Jessica tells me that she battled through the later stage of Battens for many years. She was a fighter and Jessica was a warrior mom because she loved the daughter of her dreams. Jessica did everything possible to help Nora fight. This would include the use of Cannabis derivatives for the control of seizures. To this day, Jessica is an advocate for their use and she is an expert on the subject. Even with the physical decline, Nora’s mind stayed sharp for a very long time. Jessica would tell me that everytime Nora was tested, her mental capacity was way above average.
Sometimes I think about what it would be like to no longer have Benjamin in our house. Benjamin always has his little space or room no matter where we live. His room is cluttered with all his favorite things. At the center of it is his computer which he uses to play his John Madden football game. He doesn't play it like a normal 27 year old person would but he does so to his satisfaction and pleasure. He is surrounded by all his favorite stuff. Magazines and books with pictures. Toy cars and school buses. New England Patriot signs, football cards, and stuffed animals he has received over the years. What would it be like if he was missing and his room was still filled with this stuff. More days than not, I walk into his room after returning from a long day at work. I will wrap both arms around him and put my face right up next to his. I tell him things like what an awesome person he is and how lucky I am to be his dad. I like to kiss him on the bridge of his nose and then back away slightly as I continue to talk to him. Most of the time he will push his face close to mine as if to tell me to give him another. What if he was no longer there? What if I couldn't wrap my arms around him anymore?
What would it be like to be the parent of a child with a rare incurable disease. Your days are filled with the constant care of your child. As the disease progresses the level of care needed increases. The trips to the doctors and the stays at the hospital increase. The level of home care and procedures increases but you do it because of your love for your child. Simple things like bathing become increasingly more difficult. The number of procedures required on a daily basis and the medications needed for the child's very survival go up. Your house may become more cluttered with medical equipment and perhaps the gifts from friends and well wishers fill your child's room. As a result, the bond gets stronger as you battle through each day. Your child is a little warrior and you warrior on as well. And then....some have had to say goodbye, while others have precious warriors that continue to battle.
I am so glad that progress is being made but much more needs to be done. Many families that are dealing with CLN2 are benefitting from Enzyme Replacement Therapy. I am so happy that this became available to them. These kids are just amazing to me!
Nora Skye Montville completed her journey on February the 2nd of 2017.
It was well over three thousand miles away from Jessica’s home that one of my favorite stories took place. This in Plymouth of the United Kingdom. It is the story of Elsie Clark and her grandson Marshall. Elsie was very lovingly given the care of Marshall by his awesome mum, Lara Clark, because of the special bond between Marshall and Elsie. This and also because of the qualifications that Elsie had as nurse by profession. She left her job in order to care for Marshall full time. The entire story, called No Ordinary Love, is so incredibly sweet and it will always be an out of the ordinary kind of beautiful to me. What is most special is how Elsie spoke about what Marshall had waiting for him as he completed his journey. She spoke of the freedom and pure enjoyment that he would experience once he had finished his battle. The words that Elsie spoke about Marshall as he was soon to gain his reward will always stay with me. I would like to think that her mention of things like strawberries and playground slides are just the tip of the iceberg for these kids. Both Elsie and Jessica exemplify the love relationship that exists between Batten warriors and their closest caregivers. This is especially true when we are talking about their closest of family members. That is a special bond that cannot be equaled and it is one that never fades away. There is no love like that which exists between a mother and her child. And yes, this same kind of love can exist between a grandmum and her most suave little grandson. Nora Skye, I know just where you are.
As I was preparing to post this story, I received word from a friend that little Brianna Skriver had gained her wings. Her family has a page for her on Facebook called Friends of Brianna. As I looked at my news feeds I saw many people reacting to the news of her loss. I also saw that some of the other Batten mom’s had the opportunity to meet Brianna and her mom. No doubt, it was at a Batten conference. How beautiful is Brianna! One very special video was taken of Dixie Bergeron’s Jacoby as he met Brianna. Oh my gosh! Another very special pic is one of Jessica with Brianna and her mom Tammy. The love that these families have for each other is evident. Only they can truly understand what they go through. It is true, however, that this outsider does the best that he can to understand. I hate Batten Disease but I love the families that battle it. Fly high Brianna!
It is the natural outcome that this blog would spread awareness. Anytime you put something on the world wide web, people will see it. That, however, was never my original thought. My original intent was to tell the stories of love and sacrifice that come as a result of caring for these kids. Their lives are truly special to me. They change people. They will have the biggest effect on those around them even when their lives are cut short. To me, they have the awe factor like none other. The love that exists between Nora and her momma will never fade away. Jessica would go on to have four additional children within Nora’s lifetime. Wow! There is no doubt in my mind that each one is special to Jessica, each in their own little way. The bond between her and Nora is different though. The strength of it cannot be compared to any other. Nor should it be. Batten families may think that they are just regular people that are trying to survive the day. You may have been pretty regular as the journey started, however, you became anything but that along the way. At least you became that to me. You are extraordinary!
I hope that I did good for you this time baby girl!. Nora Skye, you ARE beautiful and I know just where you are!
Blogger, Advocate, and
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
*Journey Unexpected - The Rich Family
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