It was three years ago that Larkell Bradley got her death sentence.

She was a healthy 63, manager of the pastry counter at Summerhill Market, with no plans to retire.

Her doctor sent her to a specialist after a “crackling” sound remained in her lungs following a bout with pneumonia.

After the battery of tests came the result: idiopathic pulmonary fibrosis.

It’s a disease that causes the lungs to scar, and keep scarring, until they become so stiff and thick that the person suffocates. There was no treatment, no cure save a new set of lungs, an average survival of three to five years after diagnosis. The cause is unknown.

“Unlike most people, I knew exactly what it was because my brother was dying from it at that point,” says Bradley, now 66. “When you’re given a diagnosis like that, there’s no hope. You don’t give yourself any hope.”

But then, in October 2012, Health Canada approved the drug pirfenidone, marketed as Esbriet, to treat mild and moderate IPF. The drug was “demonstrated to be effective in decreasing decline in lung function, slowing the progression of the disease and preserving exercise tolerance,” according to its manufacturer, InterMune.

It’s not a cure but, for some patients, it’s a new lease on life.

If they can afford it.

Esbriet costs more than $40,000 a year. While many private insurance companies cover it, no provincial health plans do. So those, like Bradley, without additional insurance or $3,600 a month to buy the drug just don’t get it.

“That was worse than the diagnosis, I think, because you became so hopeful that at last here’s something that’s going to help, and then to find out the government is refusing to fund the drug,” says Bradley, who relies on a pension now that she’s too sick to work.

“I never believed that, in this province and in this country, that if you can’t pay for your medication that you have to die.”

Esbriet went on the market in Canada in January 2013, but in April, the Canadian Drug Expert Committee recommended against listing it to be covered under provincial health insurance. Results from two previous trials were inconsistent, the decision noted, and “there was insufficient evidence to determine if pirfenidone provides clinical benefit for mortality or quality of life.”

Ontario’s Committee to Evaluate Drugs agreed in May 2013.

“The CED has similar concerns as that of the (CDEC) with respect to the lack of data to demonstrate clear clinical benefit from the drug and concerns about the cost-effectiveness of Esbriet,” health ministry spokesperson David Jensen wrote in an email to the Star.

Ontario has “an established rigorous process” for approving drugs to be publicly funded, wrote Jensen.

“Although drug funding decisions will be tempered by compassion, the ministry’s long-term drug funding decisions must be made through informed decision-making based on objective evidence from clinical studies to support a product’s clinical value and/or cost effectiveness.”

The U.S. Food and Drug Administration refused to approve the drug at all, insisting manufacturer InterMune conduct another trial.

Earlier this week, InterMune released results from that trial, indicating the drug was more effective than previously thought.

Dr. Shane Shapera, director of the University Health Network’s Interstitial Lung Diseases Program, said that while the most recent results are preliminary — full, peer-reviewed data is due for release in May — it would be a “landmark.”

“Before this week, we thought that this drug might have some benefit,” said Shapera. “This would be a huge step forward for my patients with IPF.”

It may also trigger federal and provincial drug committees to reconsider funding the drug.

“Although this is a terrible disease and patients don’t have a lot of time, there’s a lot more hope for patients with IPF today than there ever has been in the past,” said Shapera.

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Hugh Detzler, 65, was diagnosed with IPF in the spring of 2012. By that fall, he was quickly deteriorating.

He started taking Esbriet in January 2013, as his doctors arranged for him to undergo screening to see if he was a candidate for a lung transplant — he was that sick. He couldn’t walk any distance; he huffed and puffed on the stairs.

By the time the tests were done in March, he was too healthy to be eligible.

“He has such an improved quality of health,” said his wife, Conny. “To us, it’s like a miracle. It’s just unbelievable.”

“I’m just thinking of a year ago — right now, I figured I’d be dead,” said Hugh. “And here I am; just got back from (a vacation in) Florida. So definitely something’s working.”

But it’s costing the Detzlers, semi-retired farmers near London, Ont., $3,800 a month.

“We’re paying it out of our savings because it’s given him his life back and that’s all that counts, in the end,” said Conny. “We’re just so fortunate that we can afford it. But it’s not fair to those people who can’t.”

Though IPF is rare, it afflicts an estimated 5,000 to 8,000 Canadians. It’s thought about 3,000 die from it each year.

Robert Davidson founded the Canadian Pulmonary Fibrosis Foundation several years after being diagnosed with IPF in 2007. The organization is lobbying hard for the government to fund Esbriet.

“Patients are devastated and they’re desperate,” said Davidson, who underwent a double lung transplant in 2010.

He describes the disease as like being underwater for too long.

“And you’re coming up, you reach the surface and you break through and you breathe, and it’s a really, really great relief,” said Davidson, 66. “With IPF, you don’t break through.”

Bradley went two years after diagnosis with relatively few symptoms. Now, it’s harder to get around; the cold air makes it difficult to breathe. The crackling in her lungs three years ago is like “tearing Velcro” now. She’s always tired.

She tries hard not to cry, apologizes when she does. She wants a chance.

“I’m losing ground and I don’t want to die,” said Bradley. “And I’m angry because they won’t listen.”