David Lederer, MD, MS
Pulmonary fibrosis is a fatal disease -- and each week I see patients struggling with a disease that simply has no therapy other than lung transplantation. But with fewer than 1,000 lung transplants performed each year for people with Pulmonary Fibrosis in the United States, tens of thousands will not receive a lung transplant. We hope that our innovative approach will identify a new treatment that could saves tens of thousands of lives each year.
Pulmonary Fibrosis is a life-threatening lung disease that affects over 90,000 Americans over the age of 50. People affected by Pulmonary Fibrosis develop progressive scar tissue in the air sacs of their lungs, which leads to shortness of breath, disabling cough, and most people die from lung failure within a few years of their diagnosis. Pulmonary Fibrosis has taken the lives of a number of famous people, including Marlon Brando, Robert Goulet, and Evel Knievel.
Pulmonary Fibrosis has been linked to Sleep Apnea, a breathing problem that occurs during sleep and known to contribute to microscopic lung tissue injury. We believe that this microscopic lung tissue injury heals with scar tissue in some people, leading to Pulmonary Fibrosis. Our project is a clinical trial of CPAP -- a standard treatment for Sleep Apnea -- to treat IPF. We hope to find out if we can reduce microscopic lung tissue injury in IPF and also decrease the tendency of lung tissue to scar, improve lung function and oxygen levels, relieve shortness of breath, and make people feel better.
Why is this important?
Until recently, there are no known treatments for Pulmonary Fibrosis. Our project will establish whether or not CPAP -- a simple and safe treatment -- can treat this devastating disease. By directly treating a possible cause of Pulmonary Fibrosis, our project will also help us understand whether Sleep Apnea causes Pulmonary Fibrosis. The identification of a treatable cause of Pulmonary Fibrosis could change the face of this disease and may help reduce injury and scarring of the lungs of over 90,000 Americans.
Who will benefit?
Since we are trying to identify a new treatment for Pulmonary Fibrosis, people with pulmonary fibrosis will directly benefit from the results of this study. If we find that CPAP helps to protect the lungs, the 25% of adult men and 9% of adult women in the U.S. who have Sleep Apnea may also stand to benefit -- perhaps even by preventing Pulmonary Fibrosis if they use CPAP.