Innovative Therapies New Hope for Pulmonary Fibrosis Patients

Ed Duncan shouldn’t be alive. More than 10 years ago, he was diagnosed with idiopathic pulmonary fibrosis (IPF), a deadly lung disease that kills about 40,000 Americans each year. “My doctor at National Jewish Health told me that half of IPF patients die within five years,” Ed said. “When I asked him what happened to the other half, he said they die within 10 years.” At that moment, Ed added another goal to his bucket list: living 10 more years. He also vowed to celebrate his 50th wedding anniversary, nine years away.

Those resolutions seemed ambitious, given that there were no approved medications for IPF. However, at National Jewish Health, Ed was enrolled in a clinical trial of the experimental medication pirfenidone. Six years later, in the spring of 2014, National Jewish Health faculty and their colleagues around the nation reported that pirfenidone and another medication, nintedanib, had slowed the rapid loss of lung function among IPF patients and may extend survival. Both medications were approved in October 2014 by the U.S. Food and Drug Administration.

While the treatments don’t reverse the disease, they represent a major step forward, said Kevin K. Brown, MD, Vice Chair of Medicine at National Jewish Health and co-author of one of the trials.

This important step forward could not have happened without the expertise and dedication of physicians and researchers at National Jewish Health, who see more IPF patients than almost any other medical group. National Jewish Health houses unparalleled clinical data and tissue samples collected for decades from patients treated here.

Building on this wealth of experience and information, physicians and researchers at National Jewish Health have described who gets the disease, its natural progression and the basic mechanisms underlying its relentless scarring. They have helped guide the research agenda and raised awareness of the disease. IPF patients at National Jewish Health participate in clinical trials of most new treatments and medications.

In October 2013, Ed and his wife celebrated their 50th wedding anniversary. In June 2014, he checked living 10 more years off his bucket list.

 

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