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Fighting for Reign: Alberta boy’s family says he needs $2.8M treatment for rare disorder

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Fighting for Reign: Alberta boy needs $2.8 million treatment for rare disorder
WATCH ABOVE: Health Matters July 14: Reign Johnston is one of at least two Edmonton babies who need a multi-million dollar treatment for their rare disorder. And as Su-Ling Goh reports, Reign's family hopes increased awareness will help their boy. – Jul 14, 2020

Reign Johnston’s family met Kaysen Martin’s when both babies were in Edmonton’s Stollery Children’s Hospital. The boys have the same rare disorder and their families say they both need the same expensive treatment.

“We actually made a fairly substantial contribution to Kaysen’s campaign because we want to see him get Zolgensma,” Reign’s father, Ryan Fengstad, told Global News.

Both Edmonton boys have spinal muscular atrophy (SMA) type 1. The rare disorder affects about 1 in 6,000 babies, and causes muscles to waste away. Most die of respiratory issues before their second birthday.

READ MORE: Edmonton moms raising money for toddler needing $2.8M life-saving drug 

Zolgensma, a new gene therapy, replaces the function of a child’s missing gene. Studies show 91 per cent of patients who received the one-time infusion were still alive at 14 and 18 months, with no need for permanent breathing support.

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The therapy costs $2.8 million and is not yet approved in Canada. Reign’s parents have been raising money for him to go to the U.S. for treatment, but COVID-19 has made fundraising difficult and travel impossible.

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Another barrier is that Zolgensma is only approved by the U.S. Food and Drug Administration for babies under age two because it is deemed too risky in older children.

That means even if Canadian families can be approved through Health Canada’s Special Access Program, the age limit would likely remain.

Time is running out for both Edmonton patients. Reign will turn two years old on March 5, 2021. Kaysen’s second birthday is on July 17, 2020.

The manufacturer of Zolgensma, Novartis Canada Inc., emailed a statement to Global News about the challenges posed to some Canadian families seeking the treatment.

“We are committed to working collaboratively with regulatory, pricing and reimbursement authorities across Canada, including the Government of Alberta, in providing eligible patient access to AVXS-101 [Zolgensma] as quickly as possible,” the statement reads in part. “Novartis has heard from a number of families in Alberta and we understand their wish for the best possible outcomes for their children.
“We are actively in discussions with the province and share Minister [Tyler] Shandro’s desire to find a solution.”
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Reign’s parents said they reached out to both Novartis and Alberta Health for help months ago, but have not heard anything since.

Tom McMillan, Alberta Health’s assistant director of communications, emailed Global News and said that if Health Canada does not approve the use of Zolgensma for babies older than two, the province will ask Novartis to consider providing access through a clinical trial.

Reign’s mother, Alex Johnston, wants the decision-makers to know her son “is worth it.”

Reign’s father dreams of a day when there are no barriers to life-saving treatment.

“[I hope] no child ever has to go through what Reign or what Kaysen are going through… [that] no family ever has to go through this again.”

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