66-year-old bikes from B.C. to Quebec to raise awareness of treatment barriers for rare spinal condition

Advocates living with a rare degenerative condition are lending their support to a grandfather who has set out on a two-month bicycle journey from B.C. to Quebec to raise awareness of inconsistent treatment coverage.

Bernard McNeil, 66, had made it to Kamloops, B.C., by Friday evening, after several days of cycling in gruelling heat since leaving New Westminster on Sunday — including travelling through Lytton, B.C., as temperatures soared to 37 C mid-week, joking on Facebook he "had underestimated the heat at the 'Hottest Spot in Canada.'"

McNeil is dedicating his trip from New Westminster to Montreal to his nine-year-old grandson Malik, who has spinal muscular atrophy (SMA).

The rare condition can severely damage nerves, weaken muscles and make swallowing and even breathing a challenge.

But B.C., like most other provinces, restricts the ages of who can get medications, which can cost as much as $750,000 a year per patient.

Before he set off on Sunday, McNeil said his trip across Canada is a way to advocate for better coverage and raise funds for more research.

"Every trip I do on my bike, I think of him," he said of Malik, who was three months old when he was diagnosed with SMA. He was not expected to live past the age of two, but has done so thanks to new medication.

But even though there are several treatments now available for SMA, coverage is inconsistent between provinces and include age cut-offs that have long frustrated those with the condition.

"The most frustrating part of the disease … is the arbitrary guidelines and limitations which have been set on these treatments," said Taylor Danielson, of Langley, B.C., who spoke at McNeil's sendoff Sunday.

"Every day I get weaker and I find that my condition is progressing in a way that is less stable as I get older."

Now 28 years old, Danielson, who said he was diagnosed with SMA as a baby, no longer meets B.C.'s age cut-off of 25 for the condition's expensive medical coverage.

Along with other advocates, he says it's time for all provinces to fully cover treatments for people of all ages with the condition.

Coverage in B.C.

People with SMA, a hereditary condition, do not produce a protein needed by spinal cord nerve cells.

It's a leading cause of genetic-related deaths of infants and toddlers, and last year B.C. said it would expand screening newborns in the province for SMA. 

Also last year, the province expanded coverage available for one drug to treat the condition, at a cost of what it said was up to $354,000 for some patients a year, under the B.C. Expensive Drugs for Rare Diseases (EDRD) program.

But it's still only available for patients up to age 25.

The criteria for who can access the costly medications are based on advice from the province's Drug Benefit Council, and the federal government's drug advisory agency, a health ministry spokesperson told CBC News.

The ministry said it considers factors such as the drugs' effectiveness, their cost and whether there is an "unmet need" for them.

"The ministry recognizes the challenges that people with SMA experience and the importance of access to innovative and effective treatments," the spokesperson wrote in an emailed statement.

In 2019, the B.C. government announced it was expanding coverage for some people with SMA for another drug that it said costs more than $700,000 per patient in the first year of treatment, and half that annually after.

The coverage for that drug is only for those under age 12.

The province says under the EDRD program some drugs and patients "may be eligible for coverage on a case-by-case, last-resort basis."

Quebec is the only province that makes those drugs for SMA available to all ages, according to a non-profit organization that advocates for better funding for treatment.

McNeil's cycle journey, which his supporters are following live on a workout tracking map, is helping raise funds for the advocacy non-profit Cure SMA Canada.

"Every G7 country in the world has access to these treatments," Susi Vander Wyk with the organization told CBC News. "But Canada does not for all patients — even though the disease is the same for all types and all ages."

McNeil hopes to raise $20,000 during his two-month journey to the Montreal area, where he lives.