Run benefits pulmonary fibrosis research

While some runners might become short of breath at the Violet Rippy 5K Walk/ Run for Pulmonary Fibrosis on Saturday, breakthroughs made by their partners at UPMC and the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease at UPMC will be helping patients with the condition to breathe easier.

“We’re closer to a cure than ever before,” said Daniel Kass, the director of the Simmons Center.

He and other Simmons Center and UPMC researchers say they have developed game- changing breakthroughs in pulmonary fibrosis treatment with the help of Rippy Run funds.

The run was started in 2010 by Tami Rippy of Mars after her mother died of idiopathic pulmonary fibrosis ( IPF — PF’s most common type) the year before. Race participation and donations have generated $ 275,000 for the Simmons Center. Ms. Rippy hopes the ninth annual event on Saturday along Pittsburgh’s North Shore will bring in an additional $ 50,000.

Pulmonary fibrosis “is a death sentence,” she said. The run “is a way to continue to raise hope.”

According to Dr. Kass, pulmonary fibrosis — a chronic, progressive disease — involves scarring of the lungs and kills as many people each year as esophageal cancer or leukemia — 17,400 people.

In patients with the condition, the walls of air sacs in their lungs thicken and prevent the passage of oxygen to enter the blood. When oxygen can’t pass through, people with the disease can slowly suffocate.

“No one knows why it happens,” Dr. Kass said of the disease diagnosed in 50,000 people a year. Most die within four years.

The only life- saving solution is a lung transplant, he said.

In the past, hospitals rejected lung transplants for pulmonary fibrosis patients due to a genetic component the disease sometimes carries. But last year researchers at UPMC found a remedy, Dr. Kass said: the concurrent transfer of donor bone marrow.

Transferring matching marrow with the lungs allows the receiving body to recognize the donated organ as its own, making the viability of transplants more successful,

Dr. Kass said. A new treatment, plasmapheresis — the replacement of patient plasma with donor plasma — also is showing promise in prolonging a patient’s life.

According to the preliminary results of the ongoing UPMC clinical trial StriveIPF, 45% of patients treated with the procedure and a combination of new pharmaceuticals lived for another year after the deadly drop of oxygen levels in lungs.

Reflecting on these advances, Ms. Rippy said, “My mom’s suffering was not in vain.”