Idiopathic pulmonary fibrosis or IPF is a condition in which no known cause can be found to explain the scarring associated with pulmonary fibrosis. Pulmonary fibrosis is a progressive, fatal condition where the tissues around and between the air sacs (alveoli) in your lungs scar and thicken overtime, making it more difficult for oxygen to pass into your bloodstream. As pulmonary fibrosis worsens, you become progressively more short of breath.
The scarring associated with pulmonary fibrosis can be caused by a multitude of factors – including airborne toxins in the workplace, certain lung diseases, radiation directed to cancers in the lung or breast, and even some types of medical treatments. But in most cases, doctors can’t pinpoint what’s causing the problem. When a cause can’t be found, the condition is termed idiopathic pulmonary fibrosis (IPF).
Traditionally, there were no medication treatments proven effective in stopping the progression of the lung scarring that occurs in pulmonary fibrosis. Treatment plans are generally intended to improve symptoms temporarily and slow the progression of the disease. Many patients are treated with high dose of a corticosteroid such as prednisone and sometimes in combination with other drugs to suppress the immune system such as methotrexate or cyclosporine. Non-medication treatments such as oxygen therapy and pulmonary rehabilitation can not stop lung scarring, but are aimed to improve patients’ daily functioning and general well-being. Lung transplantation is the last resort for younger patients with severe pulmonary fibrosis who no longer benefit from other treatments. (Click here to read the American Thoracic Society guidelines for IPF)
There are several emerging therapies for IPF. One medication – pirfenidone – has been demonstrated in many research studies to reduce disease progression and improve survival. Pirdenidone in Canada has been shown in many clinical trials to effectively reduce the annual decline of vital capacity (lung function) in IPF patients.
Researchers are also developing and testing new treatment approaches for pulmonary fibrosis. The National Institutes of Health (NIH) offers information at www.clinicaltrials.gov for those interested in identifying Pulmonary Fibrosis related research at medical centers across the United States. It provides the most up-to-date research information for patients including clinical trial sites, patient inclusion/exclusion criteria, and contact information for each trial.
Scarring of the lung tissues are a result of inflammation and fibrosis in the lung. Several studies have proven that the beneficial effects of pirfenidone are mediated by its anti-fibrotic and anti-inflammatory properties. Pirfenidone is an antifibrotic drug that inhibits the synthesis of transforming growth factor (TGF)-beta, which plays a role in cell proliferation and differentiation. In a therapeutic setting, pirfenidone reduced fibrocyte (cells that cause fibrosis) pool size in the lungs, and this reflect the beneficial effect of pirfenidone on pulmonary fibrosis.
In the ASCEND (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis) study published in May 2014, 278 patients with idiopathic pulmonary fibrosis were randomized to receive pirfenidone 2403 mg/day for 52 weeks, and 277 to receive placebo (sugar pill). Patients in pirfenidone group had 47.9% less death rate and worsening of lung function than in the placebo group. The proportion of patients who had no decline in lung function was 132.5% higher in the pirfenidone group than in the placebo group. (Click here to review the complete ASCEND study)
Pirfenidone is among the most promising of the limited therapies available for IPF. In 2008, it was first approved in Japan for the treatment of IPF after clinical trials, under the trade name of Pirespa. In October 2010, the Indian Company Cipla launched it as Pirfenex. In 2011, it was approved for use in Europe for IPF under the trade name Esbriet; it was approved in Canada in 2012 and in United States in October 2014 under the same name.
The introduction of pirfenidone gives hope and opens up a new path in the treatment of IPF patients
Our Pirfenidone Program
We have been helping patients with access to pirfenidone long before its belated approval in the U.S. in 2014. Due to the current catastrophic costs for Esbriet (pirfenidone) in North America (over $8000 per month in U.S. pharmacies), we are helping many IPF patients with affordable alternative access to this life-saving medication. Our Program offers a team of dedicated experts working together to provide affordable and reliable access to pirfenidone (maintenance dose of 2400mg/day) under $300 per month along with comprehensive support services, including help with reimbursement, medication delivery, and counselling about IPF and pirfenidone. The program is designed to benefit those with IPF and the healthcare professionals who care for them.
Please click here for more drug information for Pirfenidone or Esbriet in Canada or call us toll-free 1-888-488-9965 to consult our pharmacist for free.
Idiopathic Pulmonary Fibrosis Pulmonology Specialist Dr. Charles Chan explains IPF treatment
Blogs About IPF
- PBI-4050 showing early promises in Phase-II study for IPF treatment
- FG-3019 (Pamrevlumab) to show reversal of pulmonary fibrosis in Phase-II study
- Study shows statins can improve IPF patient outcomes
- Esbriet (pirfenidone) found to reduce IPF progression in all disease stages
- What are the most effective therapies for pulmonary fibrosis?
- Pirfenidone related photosensitivity – Prevent sun burns this summer
- Living with Idiopathic Pulmonary Fibrosis
- What You Might Not Know About Pulmonary Fibrosis
- Smoking and Pulmonary Fibrosis
You can also visit the following organizations for information and resources on pulmonary fibrosis: Pulmonary Fibrosis Foundation, Coalition for Pulmonary Fibrosis, and National Heart, Lung and Blood Institute