Larkell Bradley has learned to do things differently since she was diagnosed with a rare, progressive and fatal lung disease three years ago.
Something as simple as walking can leave the 67-year-old Bathurst Street and Drewry Avenue resident breathless. And if she does venture out for a stroll, Bradley must be familiar with the area and know where she can sit down if needed. Perhaps most heartbreaking is not being able to play frisbee or hide and go seek with her six-year-old grandson, who loves to run around.
Bradley suffers from idiopathic pulmonary fibrosis (IPF), a disease that scars the lungs, killing nearly 5,000 Canadians a year, including her brother three years ago at age 68.
According to the Canadian Lung Association, the scars in the lungs cause the lungs to become stiff, making it hard to breathe. Over time, the scarring becomes so thick the lungs can’t take in oxygen.
There are many different kinds of interstitial lung disease, or pulmonary fibrosis. When a cause of pulmonary fibrosis can not be found, the disease is called idiopathic (of unknown cause) pulmonary fibrosis.
The cause of IPF is not completely understood. No one knows what causes it or why some people get it.
But the disease is linked to a number of risk factors, including genetics, tobacco smoking, acid reflux, and viral or bacterial lung infection. It tends to affect people aged 50 to 80, and is more common in men.
A sign of hope came in October 2012, when Health Canada approved a new drug called Esbriet (pirfenidone), the first and only treatment that slows the progression of mild to moderate forms of IPF. It was released in January.
But there’s one potentially deadly problem: the Canadian Drug Expert Committee (CDEC) has recommended Esbriet not be covered under provincial drug plans.
Despite funding for Esbriet in 10 other countries, the committee found the drug held “uncertain clinical benefit” and failed to provide significant improvement to patient conditions or life expectancy.
Without coverage, the drug costs $115 a day, or close to $42,000 annually, a price Bradley can’t afford.
“Right now (Esbriet) is the only hope we have,” she said, adding her pulmonary function has dropped 20 per cent in the last six months. “I personally know two people on the drug and they are doing extremely well.”
Bradley, who quit her pastry manager job in January, said she doesn’t have the funds to pay for the pricey drug.
“The saddest part in all this is people are dying and they shouldn’t have to,” she said, becoming emotional. “You shouldn’t have to die because you are poor. There is something available that can make significant difference in your life and you can’t have it because you don’t have the money.”
Bradley’s respirologist, Dr. Eva Mate, said the only other treatment is lung transplant, but that would be for patients with end-stage IPF.
“(Bradley) is at the stage where we would initiate (drug) treatment,” she said. “We are hopeful treating it early on will prevent mortality.”
Robert Davidson, president and founder of the Canadian Pulmonary Fibrosis Foundation (CPFF), said he’s disappointed Esbriet is not covered in Canada.
“We have lost too many too soon to this dreadful disease to give up our fight, and continue to be hopeful that the (Ministry of Health and Long-Term Care) will do the right thing,” he said in a release.
“Currently, this is the only treatment available for Canadians with mild to moderate forms of IPF and it’s disheartening that those individuals who need it the most, still can’t access it. We are urging the ministry to reconsider its position and help those who suffer from this terrible and deadly disease.”
An email sent from the office of Deb Matthews, minister of health and long-term care, said the drug’s manufacturer has been made aware of the ministry’s final decision and the concerns raised by the review committees.
“A final decision on whether to fund this drug publicly in Ontario cannot be made until the manufacturer provides more information to these advisory committees. As always, the ministry will happily review any new information and evidence for this drug that manufacturer brings forward,” the email said.
Esbriet is currently covered in almost a dozen countries, including France, Japan, Italy and Spain.