UVM researchers making strides in treating rare lung disease

Researchers at the University of Vermont Medical Center are making strides in finding treatments and hopefully a cure to pulmonary fibrosis, a rare scarring of the lungs.

"It took me probably six months to come to terms that I was going to die from this disease," said Bruce Towne, who was diagnosed with pulmonary fibrosis 15 years ago.

With no cure, about 50 percent of patients diagnosed with the disease will die in three to five years.

But not Bruce, who says it was a song from Tim McGraw that really spoke to him when he got the news. "He sang, 'someday I hope you get a chance to live like you were dying,'" Towne said. "That's a kind of breathtaking revelation to hear that you have something like that."

More than 200 different diseases fall under pulmonary fibrosis. It can cause a variety of issues, from severe cough to eventually using oxygen.

"I don't walk like I used to because I get short of breath and it becomes an ordeal fairly quickly. And I gave up golf, though I'm hoping to play again this summer," Towne said

Towne was part of a clinical trial at the University of Vermont Medical Center that led to two drugs being approved in 2014 designed to slow the progression of the disease. It is just one of the many research efforts at the medical center and Larner College of Medicine, including the discovery of PF being reversed in mice.

And now, thanks to lung donations from two former patients, researchers can begin to study human effects.

"We're creating these mini lungs and looking at if these new patented catalysts can actually reverse fibrosis in humans, which is such a step forward in the research," said Dr. Prema Menon, a lung specialist at UVM.

Those two lung donations came from patients in a pulmonary fibrosis support group called Breathe Vermont started by Menon. "People don't talk about it. In fact, many people don't know what it is when they come to me and told me they have a lung disease," Menon said.

The group meets every week and includes discussion and education to allow people to see there are others like them.

"It's been eye-opening -- the support group has," Towne said.

Now at 69, it's helping Towne not live like he's dying, but just live. "Feeling like I'd like another 15 years. I'm greedy now," he said.