Teaming Up to Tackle a Perilous Lung Disease

December 27, 2018

It may be considered a "rare" lung disease, but pulmonary fibrosis accounts for 40,000 U.S. deaths a year – as many lives as claimed by breast cancer.

The disease is so complex and misdiagnosed that the Pulmonary Fibrosis Foundation has named 21 medical facilities "Pulmonary Fibrosis Centers of Excellence," creating a first-of-its-kind network to increase nationwide collaboration on research and patient care.

The University of Kansas Health System is one of them. It provides treatment to patients with pulmonary fibrosis from Kansas, Nebraska, Iowa, Oklahoma, Arkansas and parts of Texas and Missouri. (Barnes-Jewish Hospital in St. Louis is the nearest of the 20 other facilities.)

Pulmonary fibrosis is a devastating, relentlessly progressive disease. Lung tissue becomes thickened, stiff and scarred. Causes range from autoimmune diseases to occupational exposures.

When the cause can't be determined, it is classified as idiopathic pulmonary fibrosis, the worst form. It typically strikes people over the age of 65, with men accounting for 70% of patients. Median survival is 2-3 years after diagnosis.

Our hospital was named a Pulmonary Fibrosis Center of Excellence because of its multidisciplinary approach to caring for patients with the disease.The facility also participated in a clinical trial involving Esbriet, a drug approved to treat the disease just last fall.

With the new designation, the hospital's experts in pulmonary fibrosis – Mark Hamblin, MD, and Lucas Pitts, MD – will have more access to a wealth of resources for both patients and caregivers.

"It certainly puts our program on a national level. We're now in contact with our field's major experts so we can take a more organized approach to the disease," says Dr. Hamblin. The designation also might attract more pulmonary fibrosis clinical trials, in addition to the 5 such trials already under way here.

Dr. Hamblin believes the new focus on pulmonary fibrosis means more breakthroughs are on the way, much like other lung diseases have attracted new treatments over the past decade.

"As a field of medicine, pulmonary fibrosis is where pulmonary hypertension was 10 years ago," he says. "Over the next 10-15 years, we'll see a dramatic change in our treatments and understanding of the disease."

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