by Shane Burcaw
Would I run a marathon? Would I drive to the beach and go surfing? Would I jump on a trampoline? Climb a tree? Go swimming? Kick something? Try skydiving? Each new idea gave me a surge of pleasure, as I imagined myself being able to explore the world with zero limitations.
All of these activities are things I would love to try, but as I lay there that night, alone in the dark without the ability to even roll myself over or turn my head, I realized something important about myself: The ability to walk would not affect my happiness.
When I was younger, this was not the case. I hated not being able to play sports and climb trees and ride bikes. To counteract this nasty feeling of inability, I developed adapted methods to involve myself in whatever my friends were doing, but there was always that wish that I didn’t need to do things differently.
As I grew up, the activities I couldn’t do shifted to things like driving a car, attending parties at houses that had steps, and staying out all night like my rebellious friends. Don’t get me wrong; I wasn’t plagued by constant sadness, but I did occasionally find myself thinking that life would be better if I could just stand up and walk.
But today, when I ask myself this hypothetical question—what’s the first thing I’d do if I woke up tomorrow completely cured of SMA?—I have an entirely different answer.
I’d walk to my kitchen and make myself a cup of coffee. I love that jolt of caffeine into my bloodstream. It’s good for the soul. Next, I would go outside and read a book in the early morning sunshine, sip my coffee, and listen to the world wake up around me. This has become a favorite activity of mine, and it helps my brain prepare for a day of work. The rest of my day would consist of doing nonprofit activities with my wonderful coworkers, eating delicious food, and jumping in the pool for a quick swim (only now I wouldn’t immediately sink to the bottom of the pool!). In the evening, I’d make a fire outside with my friends.
That is what I would do first, if suddenly cured of my disease. No skydiving. No massive displays of physical ability. I’d just do all my everyday activities, because I really love my life.
We all go through stretches of time when life places us in undesirable situations. But I think it’s cool that my desires have adapted over time to become more comfortable and satisfied with my disability.
I’m never going to be a professional skateboarder. That’s a dream of the past, and it no longer upsets me. So from now on, when people ask me what I’d do with a miracle cure, my answer will be: I’ll wake up and make myself a cup of coffee.
Because this is the life I know and love.
Chapter 21
Spinraza
I will most likely be dead before I turn 30. Even that estimate is a generous one. I have a disease called spinal muscular atrophy type II that has been slowly destroying all the muscles in my body for the last 18 years, 11 months, and 354 days. Eventually I will catch a cold, it will turn into pneumonia, and my body won’t be able to fight it off, at least that’s what all my doctors subliminally imply every time they tell me I’m lucky to have stayed out of the hospital for almost a year now. Look at that, you already know my deepest fear, the one that hits me like a train every single night when I’m trying to fall asleep; I don’t know how much longer I have to live. We’re practically dating.
—Excerpt from my very first blog post, June 1, 2011
As I reread my earliest blog posts (something I try to avoid because most of them make me cringe—how did I ever think “hella” was an adequate synonym for “very”?), I often wish I could go back in time and tell my younger self not to worry so much about dying. My writing serves as a kind of time machine, allowing me to peer into my past and observe how my mind handled the challenge of a wasting, weakening body and the ever-looming threat of death.
Most of the time, my solution was humor.
When I lost the ability to hold my head upright on the first day of school, I posted: “Trust me, life can throw you lots of shit, but if you can take a step back and laugh at your situation, everything becomes a lot easier to deal with.”
When strangers stared at me in public, I reasoned it was because I looked like “an alien-like pterodactyl creature with a human head that uses a wheelchair.”
When trying to understand why women seemed uninterested in me, I posted: “Oh, also the tiny fact that unless a miracle cure is found in the next few years, I’m going to eventually die, and that isn’t a turn-on for most women.”
When my jaw muscles began to weaken and affect my ability to talk, causing me to make a garbling fool of myself in front of my entire class, I posted, “Everything that had just unfolded was so completely awkward that it was hilarious.”
My tendency to reframe all negativity in a funny way was not done without self-awareness. In one of my early posts, I wrote: “I’ve gotten really good at putting on fake smiles when everything is not okay, pretending I’m oblivious to the fact that my condition is constantly getting worse.”
The world saw me as a happy-go-lucky funny guy who spouted rainbows of positivity, but inside my head, I was facing a nonstop loop of thoughts about death and decay. Fun, right?
In a way, I can place bookends on the early blogging days of my life and call those years the Gloomy Period. Don’t get me wrong, I was having a blast—my blog exploding into the spotlight, a book deal, starting a company, documentaries, girlfriends!!!—but taken as a whole, much of my time between 2011 and 2014ish was spent fearing the physical demise that I fully expected to arrive at any moment. I spent many a night alone in my bedroom because everything just felt hopeless and grim. My humor in those days was probably a necessary distraction as much as anything else.
As I grew up, my experiences began to provide mounting evidence that my fears of imminent death might be less warranted than they seemed. I met more and more adults living with my disease who were kicking ass in their forties, fifties, and sixties, who had families and careers and didn’t bat an eyelash at the abilities their disease had taken away, but who thrived on whatever abilities they did possess. I was told by more than a few of them that it was time for me to drop the melodramatic sob story they saw on my blog.
At first, I felt attacked. How dare they question my deepest fears and vulnerabilities? But the more I thought about it, the more I realized they weren’t aiming to assassinate my character; instead, they were trying to wake me up and free me of the shackles I was creating for myself by situating every happiness I experienced as “happiness despite my wretched circumstances.” These people weren’t suggesting I ignore the difficult realities of SMA, but rather showing me that my life need not be defined by that hardship.
Their urgings ultimately helped me embrace my disability, and my inner torment about the future transitioned into a kind of passionate refusal to be victimized by my disease. I went to a doctor’s appointment in early 2015 and was informed that my breathing had gotten objectively worse since the previous year. About that experience, I posted:
… after the initial jolt of discovery, this time, all I felt was a relaxed, calm acceptance. I turned and smiled at my girlfriend, who was with me at the appointment that day. She smiled back. Yup, I’m getting worse. It’s a slow worsening, just like it always has been, but I’m living with more intensity than ever before. There’s just no sense agonizing in the darkness of things you can’t control.
My humor, in turn, was granted some freedom to explore new subjects that did not directly relate to my disease. I posted a 621-word essay about a stink bug watching me poop. I got a job for the local paper and penned columns about soccer, staying healthy, and spreading kindness. I even started writing fiction that had nothing to do with me, and holy shit, I loved it!
An enormous weight was lifted off my chest when I realized that my disease did not need to be the focal point of my every waking thought and action. It was possible, I was learning, to just be “Shane the writer” or “Shane the boyfriend” as opposed to “Shane the disabled writer” or “Shane the disabled boyfriend
.” I felt an abiding sense of peace with this discovery.
With this new perspective in my cognitive arsenal of defense against SMA woes, I was better equipped to deal with a difficult 2016. My lungs were getting weaker and making it hard to sleep, my arms were getting weaker and making it hard to drive my wheelchair, and my jaw was getting weaker and making it hard to eat even the softest of foods. All of these losses took their toll, and I had plenty of moments where my disease took center stage for days at a time. Hannah played a crucial role in helping me through those periods, nudging me to embrace change as it came: “Will it really be so miserable to be spoon-fed your favorite soups by me for the rest of your life?”
Even with her support, I spent the year on an emotional roller coaster—one day on top of the world, ready to publish a hundred books and travel the world no matter what my disease had in store for me, and the next, when I discovered the loss of some ability, hardly able to muster a joke. Such was the unsettled, complicated nature of my existence until everything changed on December 23, 2016.
It was a Friday night, and I had just returned to my home in Pennsylvania after spending a week in Minneapolis to meet Hannah’s family for the first time. I was physically exhausted after seventeen hours in the car, but emotionally glowing with the satisfaction of being so thoroughly in love.
I opened my email, and as I scanned my inbox there was a subject line that stopped me short: “FDA Approves First-Ever Treatment for Spinal Muscular Atrophy.” I reread it three or four times as my brain struggled to make sense of those particular words in that particular order.
My heartbeat moved up into my throat as I opened the email and read its contents. In a matter of a few paragraphs, my worldview was flipped upside down and shaken violently. There was a drug called Spinraza, being studied by a company called Biogen, and what they had discovered, it seemed, was that injecting this drug into the spinal cords of people living with SMA could effectively stop the progression of their muscle-wasting. The FDA had reviewed Biogen’s findings and confirmed the drug’s effectiveness. The treatment would be commercially available within a few weeks.
My first thought: This isn’t a treatment, this is a fucking cure!
Spinraza is basically fake DNA that tells the body to stop being stupid and start creating the correct proteins for muscle growth and maintenance. It stops the progression of muscle loss, and in many recipients, there was even some reversal of the loss, meaning people gained back strength and ability.
Describing for you the intensity of the shock and disbelief I felt in the minutes that followed reading that email would be a useless task. Words are comically inadequate for what I felt. Instead, I’ll tell you what happened next.
“We’re about to jam a needle into the most precious and fragile part of your body. Smile!”
My brother came into the kitchen. I said to him, with a voice that was fluctuating in an effort to remain steady, “So, they … apparently found a cure for SMA?”
He stopped. “What do you mean?”
Don’t cry, Shane.
“Like, they’ve been studying this drug? And it apparently stops the disease?” I said.
Don’t you dare cry, Shane Burcaw.
“Like, your muscles won’t get any … worse?” he asked.
I nodded, but I happened to notice his wide eyes had the beginnings of tears forming in them and that sent me over the edge. My face opened its floodgates, and I cried the happiest, laughingest cry I’ve ever cried.
It wasn’t long before Andrew broke the seriousness of the moment with a joke. “So then I don’t have to help you pee tonight, right?”
The same basic scenario replayed itself several more times that evening as I told Hannah and members of my family. My brother ran to the store for champagne, and we celebrated well into the night with bright exuberance.
Several days later, after the initial excitement began to fade, I was left with a vague feeling of confusion about the drug. I wasn’t as joyous as I thought I would (or should) be, but I couldn’t put my finger on why. For so much of my life, the biggest obstacle to my freedom, my happiness, my safety, my well-being, my comfort, etc., has been my disease. As seen in my early blogging, my disability was such a big aspect of my identity that it started to become the defining aspect of my identity. Only recently had I started to transition away from that way of thinking.
But now, out of nowhere, there was a drug that promised to halt the progression of the disease and give me confidence in living an average life span without worry of getting worse. It would basically take away the scariest part of living with SMA—the decline. Even crazier, reports suggested that I could even gain back some previously lost strength. Wasn’t that alone reason enough to wake up every morning and scream joy?
I was excited. But it seemed like everyone I told was significantly more excited than me. In fact, countless times in the months between reading the FDA announcement and getting my first injection, I found myself downplaying the significance of Spinraza in conversation, which often elicited disturbed expressions in response, as if I’d said I had won a billion dollars but wasn’t taking the money. Ironically, I had recently published an article based on the “If I Could Walk” question. As you’ll remember, the idea was that my life wouldn’t change much, since I was already living pretty happily and successfully with my disease. Spinraza, to some extent, confirmed my arguments in that piece, and the arrival of a “cure” really didn’t faze me. More than any other feeling, it made me uneasy.
It wasn’t until I began receiving the injections almost a year later that I finally pinpointed why I felt so ambivalent about the drug. The potential to be “cured” of my disease had the unintended effect of causing everyone around me to see anything less than a complete cure as an unfortunate failure, which totally opposed the healthy embrace of my disease that I was working so hard to adopt.
Imagine a person was born with six legs. Having six legs made his life different, and at times, difficult—odd looks from strangers, clothing fit incorrectly, more toenails to cut—but, for the most part, he got used to having six legs and just wanted to go about his life normally. That’s fair, right? It wasn’t even very hard to do so, because the six-legged person was surrounded by friends, family, and acquaintances who supported this plan. Then one day, a brilliant scientist came along and said she had pioneered a method for removing extra legs! Everyone rejoiced, including the person with six legs. After all, living with six legs was annoying a lot of the time. Having two legs would be pretty sweet. But then, when it came time for the leg removal surgery, the surgeon realized she was only going to be able to remove one of the extra legs. The person who now had five legs figured this was still one less leg to lug around, so he was still thrilled with the surgery! Even if none of the legs had been removed, it didn’t really matter, since life with six legs had been normal and even pretty great most days, like when he found cheap shoes or his girlfriend gave him a blow job. However, to his surprise, many of his friends and family and acquaintances seemed bummed that the surgery didn’t work, asking him constantly if there was any possible way those extra legs could be removed someday. This made him wonder how genuinely everyone had supported his six-legged life before the surgery.
Spinraza was a drug that I’d be taking for the rest of my life, and its effectiveness varied from person to person. For instance, some people gained back arm strength after a single injection; others noticed increased stamina after a year of injections; many saw no increase in strength, but also saw no decrease, which is still a powerful outcome with a disease like SMA.
After my first two injections, I saw no difference in physical ability, and many people who received this information from me reacted with subtle sorrow.
“Don’t give up, okay?”
“I’ve been praying for this for years. I believe it’s going to work.”
“When do you think it will kick in?”
At one point, even my dad couldn’t help himself, usi
ng a flawed breathing test score to share happy news with our relatives. “It’s finally working!” he wrote to everyone, despite the two of us having concluded that the test had been done incorrectly and that I felt no actual difference in lung strength. The texts of celebration that started to pour in after his family-wide announcement were tough to read without getting angry.
I get it. Everyone was excited for me and wanted the absolute best outcome, but when their ideal “best outcome” basically meant “not having SMA,” it made me wonder if all these people, my closest friends and family, harbored negative attitudes and assumptions about my life prior to Spinraza being invented.
From my point of view, I’ve been living with spinal muscular atrophy since the day I was born. I’m used to it. I’m comfortable with it. I can and will live an extraordinary life full of laughter and love and success, regardless of whatever amount of strength I have at my disposal. I’m happy for the treatment, but don’t necessarily need a cure.
If Spinraza happens to mutate me into an ultra-strong behemoth, great, catch me at the gym, but nothing else is going to change.
Chapter 22
StankTour
In the summer of 2017, Hannah and I spent a month traveling around the United States together on a massive road trip that we later named the StankTour, referring to how little showering we did along the way. When we returned home in August and told people about our epic vacation, we received the same stupefied response time and time again: “Wait, so, it was just the two of you? Like, she did all of your care? No nurses? No medical staff?”
Hannah and I had been dating for just over a year when the road trip idea was born, and although that year was technically a “long-distance” relationship, we somehow managed to make a visit happen almost every month of that first year, so she was already thoroughly acquainted with how to keep me alive. In fact, during our first year, my physical care had become one of the strongest facets of our relationship.
