As Someone With SMA, the Fight for My Life Never Ends

It’s become a sort of ritual.

Once every few weeks around 3 p.m., my mom will drop by my room. “So,” she’ll say, with ridiculous amounts of forced brightness.

“So.” Code word for “We need to talk.” Code word for “I just checked the mail, and you’re not going to be happy with what I found.”

My application for Evrysdi (risdiplam) has been an absolute nightmare. From verifying my diagnosis to refuting false insurance claims, I’m no closer to starting the potentially lifesaving treatment than I was in October. Insurance claims are notoriously painful, so I can’t say I’m surprised. But it’s starting to get frustrating.

Every month I spend in limbo is a month of disease progression — of unnecessary physical decline.

I’m used to fighting for my life. You don’t live with a progressive neuromuscular disease for 24 years without fighting for everything, from assistive technology to vent support. Not to mention it runs in the family — my mom was one of the first people to secure full coverage for a motor wheelchair. She’s taught me to stand my ground, to use my voice and demand what I deserve.

So, I’m used to it. But it doesn’t get any easier. It doesn’t get any less exhausting, physically or emotionally. It’s gotten to the point that whenever my mom drops by or starts a sentence with the heart-stopping “So,” I assume it’s bad news.

In my defense, I’m rarely ever wrong.

A couple weeks ago, my mom dropped by.

“So.”

“So,” I echoed.

“You got denied for Evrysdi again.”

I groaned. “What was it this time?”

“According to the letter, you’re a whole eight months past the target age range. There’s no literature to support Evrysdi’s effectiveness in patients ages 25 and older.”

I was expecting something easy. Something I could refute with my eyes closed. But that took me by surprise.

Eventually, my surprise gave way to grief. I know better than to pin all my hopes on an experimental drug therapy. It’s not like I was expecting to regain fine motor control in my hands. But the development and subsequent approval of Evrysdi gave me hope. It wasn’t a cure. It wasn’t even a guarantee. But it was something.

It was a chance, and it was mine.

Grief gave way to rage. Yes, rage. A cursory Google search proved that Evrysdi is for patients 2 months and older. There’s no age limit. The age of 25 was more or less an arbitrary number, cherry-picked from one of the clinical trials, which included participants ages 2 months to 25 years. Because Evrysdi is a relatively new treatment, there’s no literature beyond the handful of clinical trials — period.

If I wasn’t eight months past the arbitrary number of 25, I might understand. But it turns out that patients over the age of 25 have been approved for Evrysdi. Michael Morale, senior director of multichannel content at Bionews, the parent company of this website, is 55, and he was approved for Evrysdi.

Not to mention that when the U.S. Food and Drug Administration wrote the label for Evrysdi, they included the adult population. Moreover, there is no specified age limit.

I’m writing this column on March 1, the Disability Day of Mourning (DDOM). Initially, the DDOM was dedicated to victims of filicide — disabled folks killed by their own family members. But DDOM has come to represent a far broader understanding of “mourning.”

Every year on March 1, we mourn victims of filicide. But we also mourn the chronically ill and immunocompromised folks who have died of COVID-19. We mourn the hundreds of thousands of Black disabled folks killed by police brutality. We mourn a community that faces constant ableism, yet displays unthinkable amounts of resilience.

It’s not that our lives are sad; it’s not that our disabilities make us any less worthy. It’s that we spend so much time and energy fighting for our right to food, shelter, treatment, love, respect — the list goes on and on.

Today, I’m mourning the months of disease progression that could’ve been prevented. Tomorrow, I’ll rest, because fighting for your life is exhausting. The day after that, I’ll return to the fight.

In many ways, the fight is all I’ve ever known.

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Note: SMA News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of SMA News Today, or its parent company, Bionews, and are intended to spark discussion about issues pertaining to spinal muscular atrophy.