Walk/run celebrates possible breakthrough for pulmonary fibrosis

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 breast cancer 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.“

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 possible breakthrough will be celebrated with the fourth annual Violet Rippy 5K Walk/Run this Saturday on the North Shore. Tami Rippy of Mars, the event organizer, has worked with Simmons Center for Interstitial Lung Disease at UPMC and the Coalition for Pulmonary Fibrosis to promote the event named for her mother, who died from the condition 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 Ms. 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, like 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 pulmonary fibrosis 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 the Simmons Center, “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 causes scar tissue to develop in the lungs and destroy 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 cystic fibrosis, which receives about $85 million in federal funding annually, the condition receives only $18 million a year, according to the Coalition for Pulmonary Fibrosis.

Ms. Rippy hopes the Violet Rippy 5K Walk/Run will change this and be “a celebration of life. Everyone is there sharing emotions, we honor different families, have them speak along with doctors and representatives from the coalition. There are people who have the disease who have walked and spoken.”

“All my mom wanted to do in life was help people, I promised her this when she passed, so I am finally able to carry on her wishes,” said Ms. Rippy.

The event will begin at 10 a.m. on Saturday at the North Shore Riverfront Park. To register or donate visit www.firstgiving.com/coalitionforpf/4th-annual-violet-rippy-5k-for-pf.