Breathing New Hope For IPF Patients
Research and Innovations Idiopathic pulmonary fibrosis patients face a deadly prognosis, but two new drugs may allow patients to live longer.
IPF is a relentless and deadly lung disease that affects 10,000 to 15,000 Canadians and has touched legends like actor Marlon Brando, daredevil Evel Knievel, and comedian Jerry Lewis. There’s no cause and no cure except for a lung transplant. “The average survival rate after diagnosis is three to five years. That is a worse prognosis than almost all cancers, except lung and pancreatic,” says Dr. Shane Shapera, Director of the Pulmonary Fibrosis Clinic at Toronto General Hospital and an Assistant Professor at the University of Toronto.
No cause or cure
IPF, which typically affects people over 50, causes a buildup of scar tissue in the lungs, leaving patients breathless and coughing. Eventually they become unable to breathe.
Two new drugs
“It’s a very exciting time for patients with IPF... only a year and a half ago we had no drug therapies for this disease,” says Shapera.
Pirfenidone was released in Canada in January 2013, and a drug called nintedanib is under review by Health Canada. Both were approved by the U.S. Food and Drug Administration in October.
Neither drug is the cure, but both can slow the progression of the disease.
“It’s exciting for these drugs to be available, but the cost is so high, they can be out of reach for most Canadians,” says Shapera. He says it’s an “enormous advancement” that pirfenidone was tentatively allowed into the Ontario Government’s Exceptional Access Program, which covers expensive medications.
A lung transplant is the only real cure, but only a few patients are lucky enough to get one. “You’re a candidate for a lung transplant if your lungs are in very bad shape and the rest of you is perfect.” says Shapera.
Other treatments include exercise, vaccinations (when something as simple as the flu can be fatal, vaccinations are crucial), antacid medicines, and as the disease worsens, oxygen.
The initial symptoms of coughing and breathlessness could be due to almost any heart or lung disease, says Shapera, so patients sometimes wait months for a diagnosis.
For new IPF patients, he recommends contacting the Canadian Pulmonary Fibrosis Foundation, which he says is doing great work to educate patients and raise funds for research, in the hopes that one day there will be a cure for this devastating and fatal disease.