Age 20 | Plymouth, UK
Living with XLMTM
"Be grateful for what you have." - Zak
The brits love football, and Zak is no different. But like many fans, he plays from the comfort of his living room—controller in hand—a look of intense concentration across his brow. There he often spends hours perfecting the timing of his favorite players and challenging himself to develop new strategies. A game with Zak usually ends up one way—he the victor. “He takes it very seriously,” Says Simon, Zak’s father. “I think he let me beat him once.”
For Zak and many young adults faced with physically-limiting illnesses, gaming provides a unique outlet for social interaction, growth, and not to mention, it’s incredibly fun. However Zak’s interest goes beyond fun, transforming into a path of further study and potentially a career. Now 20-years-old, Zak is enrolled at Plymouth University where he is taking his passion for gaming to the next level. “I [am] studying software development,” he says boldly. He wants to use those new skills to design video games, what type of video games do you want to make? Why? working towards a bachelor's degree in game development. He admits that creating video games is difficult work. There is an immense amount of competition with other developers. But if anyone can achieve this feat, it’s Zak, who is no stranger to battling the odds. “I look at things fairly normally,” says Zak. “Just like anyone else would.”
Despite his oft-expressed nonchalance, Zak represents a tiny percentage (less than 1% ) of boys affected by x-linked Myotubular Myopothy (XLMTM) who make it to adulthood. Now he faces new challenges, choosing a career and transitioning into a more independent lifestyle. “I am not dramatic,” Zak explains. “In terms of what I do at Uni, or later on in my life.” He seeks self-sufficiency—a thing more rare than XLMTM itself. With an experience-backed smile, he shares his life’s motto; “Be grateful for what you have.”
Being grateful is a lesson that Zak and his family have been learning and experiencing together for years. And the fact that they have had so much time to learn this is yet another thing to be grateful for. Recalling the early days, before many resources existed for XLMTM, Simon and Wendy were extremely grateful for anything they could find. “The reference points were few and far between,” admits Simon. In many ways, the family became pioneers in the management of Zak’s disease. Now, more and more families with growing boys look to them as a model for what to expect come teenage years and the transition into young adulthood.
“When you have a child with serious disabilities, you don't look years ahead,” says Wendy. “You take it day by day.” However, Zak is getting better and better at taking care of himself, especially around respiratory complications, which is crucial to his ability to live on his own. “More than ever before, we've been able to plan ahead. We've taught him how to manage his chest. He can pinpoint on his chest where the mucus is... He knows his chest better than we do.”
Wendy and Simon have reinforced the belief that Zak can and will be as self-sufficient as possible. “You need positive things around you when you're dealing with a rare disease,” says Wendy. “We want him to just be a normal teenager. Find himself, find life, find fun, be able to chose.”
In 2006, Wendy, along with Anne Lennox, another mother in the XLMTM community, started the Myotubular Trust, a UK-based charity that helps support families affected by the condition. The trust has availed numerous resources in the UK to families with XLMTM, and also helped Zak network extensively with other affected boys. Wendy has since stepped back from daily operations of the trust, but remains committed and involved.
Despite the tinge of normalcy in the air at the family's home—the smell of fresh bread in the kitchen and photos on the bookshelves displaying some of Zak’s favorite hobbies: kayaking; mackerel fishing; surfing and antagonizing his younger sister Sophie—things have been anything but easy in recent years. As a young teenager Zak experienced some difficult growing pains. He lost the ability to walk at around age 14 and sat down into his power wheelchair, that he still uses today. At the age of 16, Zak underwent back surgery to correct an extreme case of scoliosis that developed as he grew. It had gotten so bad that he was having trouble breathing at night, even with the help of his BiPap machine. The surgery went well, but things got complicated post-op as the hospital staff wasn't able to extubate him and get him breathing normally.
“That was the first time we had ever thought about the possibility of a tracheostomy,” says Wendy. “But once you get a trach, it's hard to go back.” The family made the difficult decision to not trach Zak, but instead to transport him to another hospital where he could receive specialized care. Their decision paid off, and after ten days in the ICU, Zak was able to return home, fragile, but determined to recover fully.
After the surgery Zak realized that he needed to take on more responsibility for his own well being. “There is quite a lot of exercise,” explains Zak, who goes to hydrotherapy multiple times a week, as well as to see a muscular-development therapist, focused on building strength using bands. The more exercise he is able to do, the better. Occasionally, on weekends, Zak feels well enough to plays wheelchair football or tennis with Powerback, a group which organizes fun activities for wheelchair users. “But he's still in recovery,” admits Simon. “When you go through something as major as that, life becomes more about the little things. For him to be able to eat—something as simple as that—is a massive pleasure,” says Wendy.
Just like Zak's muscles, the rest of his family work together to build their resilience. “It either binds you together or blows you apart,” says Simon, reflecting their situation. “You kind of have to put your own emotions and feelings aside,” says Wendy. Together they have pulled together in tough times and continue to grow along with their talented son.
Yet, as anyone who has had the pleasure of speaking with Zak can attest, his inner strength and level head is as rare as his symptoms in that he is seemingly incapable of but a bad attitude, let alone surrender.
Despite setbacks, Zak is undeniably filled with life. To date he has experienced more in his 18 years than many do in a lifetime. Some of his hobbies include , kayaking, bread making, and even surfing! Occasionally, on weekends, he plays wheelchair football and tennis with Powerback, a group which organizes fun activities for wheelchair users (He hopes soon to acquire a standing wheelchair).
Zaks mature yet light-hearted outlook on life is an inspiration to anyone. His ability to face life’s challenges without losing his center is a skill nearly all of us face, yet few succeed at maintaining equanimity with as much grace as Zak. He shares his life’s motto with an experience-backed smile; “Be grateful for what you have.”