A Manitoban is losing his ability to move his muscles. A drug might prevent that, but the province won't pay
Jeremy Bray has use of just 1 thumb, without which he cannot use a wheelchair, keep working
The first thing Jeremy Bray does every morning is check whether he can still move his left thumb.
He wakes up daily with the fear he may have lost the only way he has to operate his wheelchair, maintain a job and live independently.
Bray has spinal muscular atrophy, a rare degenerative disease that's gradually robbed him of the ability to move his muscles.
At age 29, he can no longer move his head, arms, hands or legs. He's down to the use of just one thumb, but time is running out before he loses that too.
"I can't imagine that I have more than a few months to a year left like this," he said, his soft voice trailing off.
Bray, who lives with his parents in the small southwestern Manitoba community of Rivers and works full-time as a data consultant, has noticed his thumb becoming weaker over time. He's got less energy. It's harder to get through the day.
"No matter what I'll do, eventually I'll lose," he said, "or my disease will progress throughout my whole body, all the voluntary muscles."
There's no cure for spinal muscular atrophy, though treatments can stop its progression and often lead to some improvement.
But the Manitoba government told Bray in an Oct. 30 letter it won't pay for the hugely expensive drugs, citing "clinical recommendations" that it says it is "following and will continue to follow."
That ruling is "devastating," Bray said, because without treatment he'll soon be incapable of movement and will need to rely on others, and the health-care system, for everything.
$300K for 1st year of treatment
He has the Type 2 version of the disease. While SMA can significantly shorten life expectancy, people with Type 2 can have a relatively normal lifespan with treatment, according to Dr. Xavier Rodrigue, a Quebec doctor who treats patients with spinal muscular atrophy.
The government's Oct. 30 letter to Bray doesn't reference the cost of the drug he wants — risdiplam, sold under the brand name Evrysdi — but he believes the high price is behind Manitoba's decision to deny access. Bray's family and Rodrigue estimate the price tag at around $300,000 for the first year.
Manitoba would pay for risdiplam if Bray was under the age of 25.
Health Canada has approved the use of risdiplam for people aged two months and older, but Canada's Drug Agency doesn't recommend covering the treatment for adults over 25 because no clinical tests were conducted involving people from that age group.
The federal agency — which is responsible for co-ordinating drug policy among provinces and territories — also stated there was "lower efficacy" in patients age 18 to 25, compared with younger demographics.
Quebec has no age restrictions, while all other provinces follow guidance from Canada's Drug Agency and only consider requests to fund treatments for those over 25 on a case-by-case basis.
Susi Vander Wyk, executive director of the patient advocacy group Cure SMA Canada, said Alberta and Ontario have paid for treatment in certain cases, and Saskatchewan has done so in every case she's aware of.
Bray, who lives with his parents, Darren and Tara, said his family is seriously considering moving to Saskatchewan if the Manitoba government doesn't reverse its decision.
"It's very frustrating that the province I love isn't willing to take those steps that other provinces are and have been taking," Bray said.
"I never wanted to leave Manitoba, but if something doesn't happen soon, I don't know what else to do."
Future studies unethical: doctor
The province's letter to Bray says the government encourages pharmaceutical manufacturers to present updated data that would support extending coverage to adults over 25, but Rodrigue says Manitoba is waiting for studies that will never come.
"That won't happen anymore because there's so much evidence right now that it's working," said the Quebec doctor.
It's unethical to conduct a study in which some patients receive treatment and others don't, since the drugs have already been proven to work in Canada and other countries, Rodrigue said.
He hasn't treated Bray, but has worked on a research study of other adults with Type 2 spinal muscular atrophy that showed positive effects from taking risdiplam.
Around 90 per cent of his adult patients with spinal muscular atrophy have shown benefits from treatment, he said.
By the time risdiplam was available in Canada, Bray wasn't eligible because he was already older than the recommended cut-off age of 25.
He asked Manitoba's former Progressive Conservative government for an exception in 2022, but was turned down.
The current Tory MLA for his area, Grant Jackson, said that was a mistake, since experts in the field have demonstrated the value of risdiplam for adults.
"Regardless of which government is in power, the answer from the bureaucracy has been the same — and I think that's wrong. I think the bureaucracy needs to get out of the way and provide this treatment to Jeremy," the Spruce Woods MLA said.
Strides made in treatment
Pharmaceutical companies have made strides in treating spinal muscular atrophy in the last decade. Health Canada approved risdiplam in 2021 and Spinraza, another drug, in 2017.
As a toddler, Bray pulled himself onto furniture, but then started regressing and eventually stopped trying to stand, his father said.
"We kind of thought he was just comfortable sitting or doing whatever, but this seemed to continue," said Darren Bray.
Jeremy was diagnosed with spinal muscular atrophy shortly after his first birthday.
He's never known a life without that diagnosis, but Darren said he remains in awe of his son's resilience.
Jeremy was top of his class in high school and earned a degree in computer science at Brandon University. He works remotely as a data consultant for Louis Riel School Division in Winnipeg, and in his free time goes out to restaurants and bars, and watches movies with friends.
Jeremy said he's got a good life, but he worries about becoming a burden.
Without the treatment, "I'll be living on assistance, I'll be in the hospital more," he said. "And as my respiratory system gets compromised, they'll have to deal with those additional costs."
He wants a meeting with Health Minister Uzoma Asagwara to explain what's at stake.
Asagwara said in an interview they're "more than willing to meet with Jeremy," but didn't show a willingness to change the government's position unless Canada's Drug Agency updates its recommendations.
"We follow the guidance of experts and the recommendations of the CDA, which is informed by researchers and experts who really live and breathe this work," the health minister said.
Jeremy's father said his son has taken the gradual loss of his muscles in stride and strives to live as normal a life as possible.
"He's always had tons of friends," said Darren. "Growing up, the kids migrated to him in classes."
Now some of those same friends are getting married and having kids. It's a lifestyle Jeremy won't have.
"That's been tough as a parent, right? You feel responsible," said Darren, choking back his emotions.
Through it all, he said it's been in the back of Jeremy's mind that his health is progressively worsening.
"It's all going to come to an end without a treatment," Darren said.
"Getting on treatment, experiencing some bit of gain in strength, as minimal as it might be, he loses that worry of regressing further — and that's everything, right?"
ABOUT THE AUTHOR
