Imagine being diagnosed with a mysterious and painful disease that has no known cause or cure. There’s a drug available that can extend your life and help alleviate the symptoms, but you can’t afford it and the government won’t fund it.
This is the case for thousands of Canadians who suffer from a little-known disease called Idiopathic Pulmonary Fibrosis (IPF), a rare and progressively fatal lung disease that scars the lungs so severely that patients are no longer able to breathe.
An estimated 30,000 Canadians have some form of pulmonary fibrosis and about 5,000 die from it each year. Most are given a life expectancy of only 2–5 years after diagnosis.
The Canadian Pulmonary Fibrosis Foundation (CPFF) is using the occasion of Rare Disease Day on Feb. 28 to call on provincial governments to fund Esbriet, a drug that represents patients’ only chance of survival.
“You can’t imagine a worse disease than IPF because everything starts with your breathing,” said CPFF president Robert Davidson in a video campaign to raise awareness about the illness.
“[Governments] can’t understand, otherwise they’d do something about it, they would make access to these treatments easier. You can’t send people home to die when there’s a treatment there that can help them—it’s wrong.”
Esbriet, (aka pirfenidone), the first and only treatment for IPF, is an anti-inflammatory drug that has been shown to decrease tissue scarring and improve lung function in IPF patients—helping alleviate symptoms such as severe shortness of breath, spasmodic coughing, and frequent lung infections.
The drug is expensive, however, costing about $3800 per month, or up to $50,000 per year. Lung transplants are the only other effective treatment for IPF but the procedure is risky and not suitable for all patients.
Esbriet was approved by Health Canada in 2012 but last year the Canadian Drug Expert Committee recommended that it not be listed for coverage under provincial or territorial drug plans, saying it held “uncertain clinical benefit” and failed to provide significant improvement to patient conditions or life expectancy.
But at least 13 European countries have decided to publicly fund Esbriet, and all 28 EU member states have been granted marketing authorization, based on the same information provided to Canadian reviewers.
The European Medicines Agency concluded that the drug has a “modest” effect in slowing the disease, and approved it in part because no alternative treatments are available. It is also approved in Japan, Korea, India, Argentina, and China under different trade names.
Dr. Shane Shapera, a respirologist at Toronto General Hospital, said the high cost of Espriet leaves most Canadians who suffer from IPF in a life-or-death situation.
“As it stands right now, I have two tiers of patients: those who have private insurance and can access treatment, and those without private insurance who cannot access treatment,” he said.
“In actuality, IPF patients are running out of time, and sadly, many of them will likely die before they get a chance to start on therapy for their disease.”
Larkell Bradley lost her brother to the disease and was diagnosed with IPF herself four years ago. She can’t afford the medication and worries that her time is running out. Her biggest concern is leaving behind her 6-year-old grandson who she helps raise along with her daughter, a single mother.
“The day I was diagnosed, I thought, ‘this is my death sentence,’” said Bradley in the IPF video.
“I shouldn’t have to die, nor should anyone else, because we don’t have money.”
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