Raising awareness of covert killer: pulmonary fibrosis

by Campbell North, Pittsburgh Post-Gazette

Despite claiming an estimated 40,000 lives annually - roughly the same number as breast cancer or prostate cancer - pulmonary fibrosis remains one of the least known covert killers in the country.

"Survivors of come together in droves to raise awareness," said Teresa Barnes, vice president of patient outreach and program support for the Coalition for Pulmonary Fibrosis, based in Culver City, Calif. "Pulmonary fibrosis leaves no survivors," except a relative handful who receive .

,Pulmonary fibrosis causes progressive lung scarring and eventually suffocation.

Hope is on the horizon. The first treatment options available, InterMune's pirfenidone and Boehringer Ingelheim's nintedanib, are being evaluated by the Food and Drug Administration and may become available as early as this fall.

The disease has an annual fundraising walk/run in the summer in Pittsburgh organized by Tami Rippy, whose mother died of the disease in March 2009. "Knowing she was suffering, and literally suffocating, that there was no treatment, no idea where it came from or why it happened makes it devastating," said Rippy. "People assume my mom got it because she did this or that or smoked, but she was healthy all these years."

The terminal diagnosis often disguises itself in generic symptoms, such as dry cough and fatigue. It typically affects people over 50 but does not discriminate, debilitating runners and smokers alike. The overwhelming majority of cases are idiopathic, meaning no known cause is ever identified and the condition is known as idiopathic or IPF.

According to the coalition, pulmonary fibrosis patients may have an exaggerated or uncontrolled healing response that, over time, produces excessive fibrous scar tissue - or fibrosis - in the lungs. It's not known what sets this abnormal tissue-repair process in motion.

"This is worse than a cancer diagnosis," said Kevin Gibson, clinical director of a center at the University of Pittsburgh Medical center that specializes in lung disease. "With cancer you're offered treatment, but with IPF there is no treatment apart from transplant." Fewer than 1 percent of these patients receive them.

The two drugs are considered breakthrough therapies, meaning the FDA will expedite the process of their development and review. The drugs work by trying to reduce the onset and progression of the disorder, which also destroys blood vessels, diminishing the ability to circulate oxygen in the body.

"If they're approved it's great, but the treatment's effects are modest at best," said Dr. Gibson. "It is still a long way until a cure."

Although the condition has been recognized for the past century, relatively little is known about pulmonary fibrosis, the "disease course being highly variable and unpredictable," he said.

Despite being five times as prevalent as , which receives about $85 million in federal funding annually, the condition receives only $18 million a year, according to the Coalition for Pulmonary Fibrosis.

3.3 /5 (3 votes)
add to favorites email to friend print save as pdf

Related Stories

Protein molecule may improve survival in deadly lung disease

May 06, 2014

Researchers at the University of Illinois at Chicago College of Medicine have discovered a protein molecule that seems to slow the progression of pulmonary fibrosis, a progressive lung disease that is often fatal three to ...

Two drugs offer hope for fatal lung disease

May 18, 2014

(HealthDay)—A pair of drugs offers new hope to patients with a progressive, fatal disease that robs their breath by scarring the lungs, according to clinical trial results.

Scarred lungs leave trail of beta arrestins

Mar 28, 2011

Targeting a family of signaling proteins called beta arrestins may stop the life-threatening scarring and thickening of lungs associated with pulmonary fibrosis, reports a new Science study in mice.

Recommended for you

Mediterranean diet may help protect kidney health

10 hours ago

Adhering to a Mediterranean-style diet may significantly reduce the risk of developing chronic kidney disease, according to a study appearing in an upcoming issue of the Clinical Journal of the American Society of Nephrology (C ...

User comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.