Idiopathic pulmonary fibrosis (IPF) is an uncommon, yet deadly respiratory disease, characterized by a progressive decline in pulmonary function caused by lung scarring — the cause of which remains unknown today.
Patients diagnosed with IPF along with their significant others recently received the good news that they are finally getting access to the first approved treatment for their condition. The announcement came after 2 years of hopeful, indignant lobbying.
Esbriet (pirfenidone), the first and only approved IPF treatment in the country, was first launched back in October 2012. IPF patients were ecstatic, until the Common Drug Review (CDR) ruled that Canadian provinces exclude the drug from public funding. This recommendation restricted drug access to countless desperate IPF patients.
Earlier in May, the optimistic findings of a phase 3 trial of Esbriet was published in the New England Journal of Medicine (NEJM), entitled, “A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis.”
According to the publication, the drug greatly lessened lung function impairment by 47.9%, with 27.5% of the participants, showing improved 6-minute walk distance (6MWD) results. These findings significantly tipped the scale in favor of public funding.
Just last August 19, 2014, it was finally enacted that Esbriet would be funded through the Exceptional Access Program (EAP). According to this ruling, IPF patients who qualify according to a set of criteria, and who depend on Ontario’s Public Drug Programs, will gain access to this breakthrough drug.
The president and founder of the Canadian Pulmonary Fibrosis Foundation (CPFF), Robert Davidson, said the decision was long overdue and that the Ontario government made the right decision. What is saddening is the number of patients who passed away while waiting and fighting for their right to a cure.
IPF patients in Ontario can now breathe a sigh of relief, but the fight for access to Esbriet has not ended for many other Canadian provinces. Lobbying IPF groups along with experts at the CPFF are still putting pressure on the CDR to make a favorable and timely funding decision for their only hope at survival.
In spite of significant unmet needs in IPF treatments, research still continues. A few months before the May 2014 publication of Esbriet trial results, a neuroscience student from UT Dallas spent 3 months researching the progression of IPF at the John Hopkins University School of Medicine (JHU). While his short time at JHU was clearly not enough to produce any significant findings on the disease, many are confident his piqued interest in IPF will guide his future research endeavors, ultimately for the benefit of people struggling with this deadly disease.