This story first appeared in the Information Point newsletter Our World in 2011, the year the Information Point celebrated it’s tenth birthday. To mark the occasion, we asked some parents with children also celebrating their tenth birthday this year, to reflect on the challenges and achievements of their first ten years. Mason’s parents Scott and Staceyanne continue the story below.
After years of infertility and numerous losses, I think that we were some of the most prepared parents EVER for not just the having of a baby, but an entire lifetime of family fun. We had our entire life planned out, from baby announcements, to private schools, to family camping adventures, to a surprise 18th birthday trip to Italy. What we were not prepared for was the shock of losing not just all those dreams, but having to face the reality that instead of taking our new bundle of joy home, we were in for the fight of just keeping him alive.
Mason’s life began with an emergency c-section at 32 weeks gestation. Our local hospital was unprepared for such a premature baby but most especially one born with such obvious neuromuscular issues. And even though Mason never kicked, rolled or moved in utero, and that I myself carry a diagnosis of muscular dystrophy, it was still a complete surprise for all when he came out limp and non breathing. He was immediately whisked away and transported to a larger hospital two hours from our home, all before I was ever able to see him. The transport team was very clear with us that there was little chance of him making it to the other hospital, so we should prepare ourselves for the loss. Six long and agonizing hours later, the dreaded phone call came – they did not expect him to last the night but would do their best to keep him alive until I could come say goodbye. They did not know what was wrong, but suspected a combination of Marfan and Prader Willi. This began a seemingly endless round of genetic testing, horrifying diagnosis and the always present specter of death.
Over the next two months, we ran the gamut of diagnostic hell – Marfan, myotonic dystrophy, mitochondrial dystrophy, spinal muscular atrophy (SMA), congenital demyelinating neuropathy, Weaver Syndrome. To us, they seemed to focus on every type of disorder that guaranteed a horrible and painful demise. We eventually made the decision to forgo further testing and instead concentrated on keeping our boy alive. Through Scott’s diligence and a purely chance stumbling onto another boy’s website, we found a disorder that seemed to fit our son. Just after his 4th birthday, Mason was officially diagnosed with X-Linked myotubular myopathy.
From birth onward, he did not tolerate being on his back, otherwise both lungs would immediately collapse. We watched them turn him side to side every 15 minutes, which would cause the downward lung to collapse, while the elevated side would re-expand. We joked that our kid’s best talent was popping a lung up in a matter of minutes from being turned. Humor has carried us through many a challenge! This cycle continued for months, with the time frame being eventually extended to hourly by the time he was a year old. His lungs alternated their collapses until he was three years old, at which time he went onto the vent. Even then, he built partial atelectasis on a daily basis until he was well past his 5th year.
Mason was only intubated the first 24 hours of his life and then it was CPAP and eventually high pressure canula, all without oxygen. At 6 months old, after being transferred to a higher care hospital, a bronchoscopy showed severe laryngeal, tracheal and bronchial malacia. He had had several life threatening episodes and now the risk for complete upper airway collapse was so significant, we made the decision to have him trached. And although we had fought this procedure for so long, it provided instant relief and at eight months old, we were finally able to bring our little guy home! To this day, Mason still has chronically elevated CO2’s, even with his now being fully ventilator dependant, which they attribute to constant partial upper airway collapse.
Before our discharge, we were warned that he would have to be protected, isolated and closely monitored. Basically, we needed to keep him safely within the bubble of our home, cared for and guarded by the team of nurses eagerly waiting to take over our lives. Three days after we arrived home, it snowed in our town for the first time in over ten years, prompting our nurses to panic in shock and anger when Scott carried Mason out to feel the snow fall on his face. That seemed to set the precedent for our life. We may not be able to give Mason the life we had originally planned but we decided then and there, that we were not going to let others place us in a box, or tell us what our life would be.
Our first rebellion against the restrictions the medical community felt the need to impose, was a five day road trip just after Mason’s first birthday, wandering up the Northern California coast, willy nilly driving the back roads and showing Mason the sights. In hind sight, we now cringe in fear at our own stupidity and arrogance, both in venturing so far off the beaten track with a respiratorily challenged child and our complete lack of preparation and common sense. Somewhere about three hours from the closest town, it finally occurred to us that maybe we hadn’t quite thought things through, as Mason was turning blue and we were desperately trying to find the O ring that we had lost from our ONLY tank of oxygen. That not withstanding, it was an amazing trip and the first time we were ever truly alone with Mason. But more importantly, it was the first of many adventures.
We learned very early in Mason’s life that he was severely immuno-compromised. He did not tolerate even the simplest of illness (he was even life flighted for teething because he could not tolerate even the lowest of fever) and it seemed that he was going to catch every single bug there was. After attempting to attend preschool a number of times, all resulting in a life threatening illness and life flight, we were asked to consider keeping him isolated from children year round and the general public October through May. This was a very difficult decision, having to weigh the quality of life to the quantity and at first it truly felt like just one more loss. But we watched our little boy suffer through ambulance rides, horrifying emergency room visits and life threatening helicopter flights, followed by weeks and months of being trapped and poked and prodded in the intensive care unit and it felt like those brief moments of pleasure at school were so not worth the on average of five months per year in hospital. So, we again faced a different future than what we had expected, even on the medical front. But this time, we had learned from all those traumas and close calls and we determined that we were going to have the best time we could possibly give him, compromised immune system or not. This was going to be one long, love filled adventure… and so far, it has been!
If I had to find words to describe our little boy, I would have to say that he is happiness personified; a living, breathing piece of joyful music. From the moment he discovered his voice through an in-line Passe Muir Valve, he has sang his way through every moment, good or bad, of his life. We sit in hospitals with all these other children and I see their parents suffer as they cry in fear or pain and we thank God for our sweet little man. In even the most traumatizing or painful moments, Mason has a smile on his face, a flapping woo hoo in his hands and a song on his voice. He knows every word of every song of every episode of every show he likes and he sings them every day, all while having a figurine from his favorite show clamped in his teeth. His trach cuff is up when he’s asleep but every morning when we go in his room, his lips are moving, his tongue is clicking and when we let the cuff down, he literally continues the song out loud that he was singing in his head! He has the ability to pick up and learn a song within moments of hearing it begin and if one of the many songs in his memory doesn’t fit his mood, he will gladly do a mixed up medley of whatever his favorite parts of various songs are. And when that fails, he just makes up his own music. Due to severe fungal septicemia when he was three years old, he spent three weeks in a coma and by the time he woke up he had sustained brain damage and had lost partial control of his tongue and jaw, so most people cannot understand his words. But there is no mistaking the melodies, the pitches in tone, or the sheer exuberance of our little musical note! And even the most fearful, ignorant and / or hardened of lookey lou’s cannot help but smile when they hear him singing and see him smiling away.
Mason’s joy in life has inspired us to set aside our own reservations and to fully embrace the fun, silliness and innocence of perpetual childhood. The heartbreak upon learning of his brain damage was soon overshadowed by the blessings that came from it. Our little boy will always sing his innocent songs, will always want to snuggle and cuddle, will always welcome hugs and kisses while returning them back with open-mouthed baby kisses of his own, will always take joy in even the simplest of pleasures. He will never know anger, he will never feel regret, he will never learn that by society’s standards, he doesn’t fit in. But most importantly, he will never know fear – and that is a true blessing. And because for us he will always remain ‘little’, we as parents have the pleasure of experiencing the joys and wonders of every day through his eyes. Our family is well known for our Halloween costumes, our doing silly songs and dances in public, our home full of a million toys and continuous musical children’s programs, our huge yearly bash for Mason’s birthday and our willingness to laugh at ourselves (and others!). Everything makes Mason happy and being around that contagious smile, that piping little boy voice, we can’t help but be light of heart and spirit, too.
Maybe because all our doctors are four hours from our town, but Mason learned to love travel from the very beginning. He loves to go, be it somewhere familiar or somewhere new, he loves to be on the road – as long as his music or TV are playing, that is! He loves amusement parks, especially the parts where everyone is screaming! He loves the coast, with the Redwood trails and the shark petting exhibits. He loves San Francisco, with the traffic and the tall buildings, the street performers and the generally loud noise. Because he has to be isolated from the majority of children and people, we have taken our adventures to the great outdoors. We have hauled this kid’s wheelchair up and down terrifying dirt trails, just to give him a three minute ride on a Sky Tram over the Redwoods. We have parked his chair so his feet hung over a tank, so that the sand sharks could come up and bump his toes. We have gotten stuck in muddy fields on more than one occasion, just so he could feed the fish from the shore. We have even broken through a few federal fences and gates to take him out on restricted jetty’s, because that would help him feel like he was out in the actual ocean. And two summers ago, we hauled him and his eleven carry-ons onto an airplane and flew him to Texas to meet other boys like himself. And being with those boys opened the door for another world and even more adventures.
Mason never showed a desire or aptitude for being in a powerchair, so we had pretty much given up on that particular dream long ago. Maybe if he had been able to attend actual school and could have seen other children driving around, he would have been interested – we’ll never know. But once he did see kids zooming around, that was it for him. One month later and we had a loaner chair and were trying to get him to drive. Now, we are the first to admit that Mason is pretty much allowed anything he wants, which has created a somewhat tendency towards stubbornness. Three months of our working to get him to drive and we were literally having to tie his hand to the control, otherwise he would fling it over the side of the chair – he much preferred to have us walking beside him, driving the chair where he wanted to go. We had just made the decision that after the Holidays, we were going to return the loaner and give up on this idea. But in Mason’s typical fashion, he decided out of the blue to shock us into the next stage of our life.
While at a Christmas tree lot, I turned my back for only a minute and he was gone. My first thought was that I had left him with Scott at the back of the lot but then I remembered I hadn’t. Then I just stood there in shock, because after months of his refusing to even touch the control, I knew HE hadn’t voluntarily gone anywhere. At the same time, abduction has NEVER been a worry – no one is ever going to steal a kid on a vent, much less in a wheelchair that they couldn’t even get into their own car! Ten minutes of me and the staff frantically screaming his name and running through the trees and we finally headed up to the front to call for more help. And there he was, the front wheel of the chair hooked on a bale of hay by a fire pit. And upon hearing my frantic call to him, my precious angel took one look at me and gunned that chair in the opposite direction at full speed. He crossed the tree lot, turned out a gate, drove across a busy street and then navigated the parking lot to park himself at the back of our van – all with me trying to catch up and screaming for him to stop! For the first time in his life, I truly didn’t know whether to hug or smack him, and I can honestly say that if he hadn’t been sitting there proudly woo hooing those hands and humming the ‘I’m so Happy’ song, he might just have gotten the smack! To this day, when Mason feels he’s had enough of whatever we’re doing, or he doesn’t think it concerns him, he makes a break for it, and drives himself back to our van – his subtle way of letting us know he’s ready to go! And if at any time we come across someone in a powerchair, or one of those scooters provided by a store, Mason feels the need to zoom over and carefully bump them, as if inviting them to play. And when they drive away, he follows them about like a puppy, determined to interact with one of his ‘kind’. 🙂
Going into a powerchair has made a tremendous impact on Mason. He receives all his services in-home. School, occupational therapy, physiotherapy, speech – everyone comes to us, in order to help minimize the risk of cross-contamination. But even with these measures, Mason’s immune system has continued to break down and he averages IV antibiotics every 3 – 4 months. This has taken a large toll on his body and we have seen a marked decrease in his ability to be on the go. So, once again, we are entering a new phase of this journey.
Up until this last year, Mason’s life consisted of our traveling as a family, or his being in our living room watching his shows. He has shown no desire or aptitude for games, computer or otherwise, so his TV is all he’s had. But since he started driving his powerchair, he has made several leaps developmentally, and six months ago, we discovered a new world – the world of iPad! We still have our outdoor adventures, they’re just closer to home and for shorter durations, to accommodate Mason’s increased weakness and limitations. But as this new stage progresses, Mason’s world is expanding and unfolding, all within the walls of our home and the vast worldwide App Store!
Our life with Mason has been a wondrous, terrifying, joyful adventure and he has brought such amazing love and fulfillment to our family. We look back at all those plans we had, all those momentous occasions we thought would mark our life as complete and we have to laugh. The experiences of this little boy, the blessing of witnessing his courage, his strength, his determination – all other things in this world pale in comparison to what we have gained from this one miraculous child. At times of trial or challenge, people ponder the places they’ve been, the place they are, what the future will hold, and they question the loss of ideal, the loss of dream, the loss of faith. We say to one and all, what has been lost when you live a life full of love and song…