A man with cystic fibrosis says the recent death of a young Nova Scotia woman highlights issues with accessing life-saving drugs in Canada.

Chantelle Lindsay died in hospital on Wednesday after a lifelong battle with cystic fibrosis. Her father announced her death on Facebook. She was 23.

In the final months of her life, Lindsay's family and health-care team fought for access to a breakthrough medication for the disease called Trikafta, manufactured by Vertex Pharmaceuticals, an American company.

Trikafta has the ability to treat 90 per cent of cystic fibrosis patients and costs $300,000 a year.

So far, it's only available in the U.S., so Lindsay's health-care team applied for compassionate coverage, which would have granted her access to the drug.

But Lindsay's application was denied earlier this month.

Timothy Vallillee, who has also struggled with accessing cystic fibrosis drugs in Canada, said he reacted to Lindsay's death with "extreme sadness."

"Being only 23 years old with cystic fibrosis and succumbing to this disease when there is a drug available to avoid all that, it's pretty heartbreaking," Vallillee told CBC's Information Morning.

"Our whole country weeps right now in the CF community because this girl is not with us."

In 2013, CBC News covered Vallillee's efforts to create more access for Kalydeco, which can treat the rare mutation of cystic fibrosis he has. While the twice-daily drug was approved by Health Canada in 2012, it costs patients about $300,000 a year.

Vallillee says getting access to Kalydeco instantly changed his life. (Brett Ruskin/CBC)

Vallillee was able to get access to the drug through Vertex's compassionate care program in May 2014, and the next month, Vertex announced it reached an agreement with provincial and territorial governments to enable public drug plans to cover the cost of the drug.

Getting his hands on that drug changed his life, he said.

"I was 46 years old. I'm now 52. My lung function went from about 31 per cent to 52 per cent within just a few weeks," he said.

Drug approvals a 'long process'

Canada's drug approval process is "relatively complex and fragmented," said John Wallenburg, the chief scientific officer of Cystic Fibrosis Canada.

First, the manufacturers submit the drug to Health Canada for approval. Health Canada will evaluate it before approving it for sale in Canada.

Before a drug gets to the public formulary paid for by the provinces, it needs to go through the Patented Medicine Prices Review Board (PMPRB), a body of Health Canada, which sets the maximum price.

It's a long process and it can take many, many years.​​​ - John Wallenburg, Cystic Fibrosis Canada

Once that's established, it needs to go through the Canadian Agency for Drugs and Technologies in Health (CADTH), which does an economic analysis on behalf of the provinces and recommends whether to add the drug to the provincial formularies.

If CADTH recommends adding a drug to the formularies, the pan-Canadian Pharmaceutical Alliance (pCPA) then negotiates the price with the manufacturer on behalf of the province.

"It's a long process and it can take many, many years," said Wallenburg.

Vertex hasn't submitted Trikafta for approval in Canada.

Vertex responds

In an email statement, Vertex spokesperson Christina Cunningham said the company can't comment on drugs that haven't received market authorization in Canada.

But she did suggest changes to the PMPRB approved by Health Canada will affect the country's access to drugs.

Under the new regulations, the board will no longer compare prices with the United States and Switzerland, which have some of the world's highest drug prices, when figuring out what companies are allowed to charge.

The board will also now have to consider a drug's "value to and financial impact on consumers in the health system" when determining if a price is excessive. The federal government said this tweak will save Canadians billions over the next 10 years.

"Vertex has strong concerns that new Canadian medicine pricing reforms have the potential to limit access to treatments for Canadians living with a rare disease," Cunningham wrote.

Wallenburg agrees the new regulations could affect access to drugs in Canada.

"Those changes injected a level of uncertainty into what a manufacturer might expect the price will be in Canada," he said. "And it seems, really, that it's that uncertainty that is putting a chill on the industry. So at this point in time, the manufacturer is hesitating even to bring the drug to Health Canada for a regulatory approval."

In an email, Health Canada spokesperson Geoffroy Legault-Thivierge said the changes will improve drug access and affordability in Canada.

"Evidence shows that lower prices do not limit access to new medicines," he said.

"Countries with lower prices than those in Canada (e.g., France, Italy, Norway, Sweden and the United Kingdom) have new medicines introduced within a similar timeframe as, or even earlier than, they are currently introduced in Canada."

Legault-Thivierge also said while Vertex hasn't submitted Trikafta for approval in Canada, Health Canada has been in contact with the company with regard to the product.

"While Health Canada encourages manufacturers to file drug submissions in Canada, it is up to a manufacturer to decide whether it chooses to seek market authorization for its product," he said.

Chantelle's 'legacy will go on'

Vallillee, meanwhile, said he's seen some "incredible" changes over the years when it comes to treating cystic fibrosis, and he's grateful that a drug like Trikafta exists.

But he said Lindsay's story shows that more progress needs to be made.

"I hope this is the last time this happens. I fear that it won't be the last time this happens," he said.

"But we have to just keep plugging along and remembering people like Chantelle, because her legacy will go on."

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