It’s a disease with no known cure, no viable treatment and kills nearly 5,000 Canadians each year, but most Canadians have never even heard of it.

Idiopathic Pulmonary Fibrosis, or IPF for short, is a rare lung disease that causes tissues in the lungs to scar over time. The scarring prevents oxygen from reaching vital organs. Patients are left with shortness of breath, tissue buildup and chest discomfort.

A new drug recently approved by Health Canada is giving IPF patients a glimmer of hope, but gaining access to this potentially life-saving medication is proving to be an uphill battle for many.

“Esbriet” is an anti-inflammatory drug that has been shown to decrease tissue scarring and improve lung function in IPF patients with mild to moderate symptoms.

Earlier this year, the Canadian Drug Expert Committee recommended that Esbriet not be listed for coverage under provincial drug plans. The committee found that the drug held “uncertain clinical benefit” and failed to provide significant improvement to patient conditions or life expectancy.

Without sufficient private health coverage, Esbriet can cost patients up to $40,000 a year – a price tag that many patients simply cannot afford.

Larkell Bradley has been living with IPF for the past three years. The 67-year-old grandmother says that without access to Esbriet, her future looks bleak.

“If we don’t get funding for the Espreit, I’m going to die and so will a lot of other people. And the sad part is we’re going to die unnecessarily,” said Bradley.

“There are those of us in the stage where we really need it and the drug is doing wonderful things to people who are on it,” she adds.

Dr. Eve Mate, a Toronto-based respirologist, says that she has at least 20 IPF patients under her care who could benefit from Esbriet.

“There is no other drug which has proven to have a beneficial effect to alter the course of this disease,” says Mate.

For patients like Bradley, there are limited alternatives when it comes to IPF treatments. Lung transplants are the only effective treatment of IPF but procedures are risky and are not be suitable for all patients.

Retired Ontario farmer Hugh Detzler discovered his lung function was declining at a rate of 10 per cent a month because of IPF.

“You don’t have to be a mathematician (to figure out) it was coming to the end of the line at 10 per cent a month it doesn’t give you a lot of time,” said Detzler.

He was constantly tired and out of breath and was about to be put on the lung transplant waiting list. But in January of this year, he decided to buy the drug, paying the $3,600 a month out of his savings.

“I feel better, I can walk further. … I am assuming something is working because I feel better a lot better,” said Detzler.

Recent tests have shown his lung function has stabilized, said his wife Conny.

But still, he worries about his ability to keep paying for it. “It’s $40,000 a year, do the math. The money might run out,” Detzler added.

While no provinces in Canada will currently pay for Esbriet, the medication is covered in many other countries, including France, Japan, Italy and Spain.

But for Canadians like Bradley, the debate around access to Esbriet is a life-or-death situation.

“I just find it unbelievable that in this province, if you can’t pay for a medication, you die – that’s just inexcusable” she said.

With a report by CTV News medical specialist Avis Favaro and Elizabeth St. Philip