Recently, a possible cause of vaping-related illnesses has been identified, and scientists warn consumers around the world to beware of vitamin E acetate. In comparison, there has been little progress in identifying the cause of idiopathic pulmonary fibrosis (IPF), a chronic lung disease that affects about nine in 100,000 people across North America and Europe.
A new report sheds light on the challenges of managing this disease and calls for immediate action to address patient needs.
Currently, it’s not known what causes the progressive scarring of lung tissue (fibrosis) which makes it difficult for a patient to breathe. The non-specific signs and symptoms such as shortness of breath, chest pain and chronic coughs are easily confused with asthma, bronchitis, chronic obstructive pulmonary disease (COPD) and emphysema. For this reason, patients may live with undiagnosed IPF for years, enduring frustrating visits to the doctor and missing days of work due to worsening symptoms.
The report, prepared by biopharma Roche and The Canadian Pulmonary Fibrosis Foundation, describes how misdiagnosis is one of the main challenges facing IPF patients.
“Since IPF symptoms can be attributed to common ailments, awareness remains low,” said Dr. Martin Kolb, a professor of medicine in respirology at McMaster University, and contributor to the report. “Few physicians are trained to identify IPF at first instance, creating a delay in the diagnosis that can lead to patient suffering. Increasing awareness and understanding of the disease are critical to help prevent late diagnosis, reduce the cost of care and ultimately improve patient outcomes.”
Lung transplant is the only proven therapy to extend survival in those with IPF, but many patients presenting with co-morbidities are deemed ineligible for a new lung. Similarly, the report found that barriers to accessing designated treatment centers (interstitial lung disease centers) and pulmonary rehabilitation services remain high and variable between Canadian provinces.
The IPF drug market is largely untapped, occupied by Roche and Shionogi’s Esbriet (pirfenidone) and Boehringer Ingelheim’s Ofev (nintedanib), both approved in the US, Canada and the EU. Not all patients are eligible for these drugs, nor can they afford the approximately $10,000 price tag per bottle.
Recently, Roche announced plans to acquire Massachusetts-based biotech Promedior in an effort to expand its IPF therapeutic pipeline. The company’s lead candidate, PRM-151, was found to exert anti-fibrotic activity in not just IPF, but in other fibrotic diseases — liver fibrosis, chronic nephropathy and age-related macular degeneration. The US Food and Drug Administration (FDA) granted Promedior Breakthrough Therapy Designation earlier this year for IPF.
While IPF is relatively rare in North America, there are too many unknowns that make this disease particularly dangerous. Indeed, the Centers for Disease Control and Prevention reported a mysterious uptick of IPF cases among dental personnel (dentists and dental technicians), potentially attributed to occupational exposure to pathogens, chemicals, dust and radiation. The five-year median survival rate hovers around three to five years.
As researchers continue developing novel therapies for IPF, authors of the new report urge stakeholders to focus on expanding patient access to care and improving education and awareness of IPF.