UPMC to join pulmonary fibrosis network
Jim Uhrig's lungs were in such bad shape that his doctor said they sounded like Velcro being pulled apart.
“I felt like I had the flu all the time,” said Uhrig, 68, of Peters. “I'd be tying my shoes and I'd be out of breath.”
A lung specialist diagnosed him with pulmonary fibrosis, a condition in which lung tissue becomes thick and scarred, preventing the lungs from transferring oxygen into the bloodstream. The disease has no cure and can be deadly.
The prevalence of pulmonary fibrosis is grossly underestimated, said Dr. Kevin Gibson, director of UPMC's center for interstitial lung disease. To get a better grasp on the illness, UPMC will become one of nine sites in the country that will establish the Pulmonary Fibrosis Foundation Care Center Network and Patient Registry.
“To make progress with this disease, we need a multidisciplinary approach by teams of expert medical professionals. We need more data and we need to track the natural history of the disease,” said Dr. Daniel Rose, CEO and chairman of the board of the Pulmonary Fibrosis Foundation, which is overseeing the projects.
The foundation says the network will enable medical centers to gather information about the disease, which can be as deadly as some cancers. It intends to expand the network and registry to include 40 medical centers by 2015.
Gibson said the project should help doctors assess treatments patients receive and which ones work. The foundation estimates about 50,000 new cases of so-called idiopathic pulmonary fibrosis — the most common type — are diagnosed annually in the United States. The disease kills as many people as breast cancer, or roughly about 40,000 people a year.
Experts say pulmonary fibrosis is not necessarily increasing in frequency but rather being diagnosed more often because of the widespread use of CT scans, by far a better diagnostic tool. Gibson's clinic was forced to expand hours to nine half-day clinics from two half-day clinics 10 years ago.
“Suddenly we're recognizing that a lot of patients were given other lung diagnoses and turned out to have pulmonary fibrosis,” Gibson said, citing patients diagnosed with asthma or chronic obstructive pulmonary disease, known as COPD.
“The big joke about lung disease is that all patients have two symptoms: shortness of breath and cough,” he said. “Symptoms alone don't distinguish lung disease.”
Uhrig said he had not heard of the disease when doctors diagnosed him at age 63 in May 2008. By the following year, his condition had worsened and he needed oxygen 24 hours a day.
Doctors recommended a lung transplant, a solution they mentioned early on. He received the double- lung transplant in April 2009 and his surgeon told him his own lungs were like bricks that might have lasted only another two months.
“I still feel like I'm getting stronger,” he said.
Luis Fábregas is a Trib Total Media staff writer. Reach him at 412-320-7998 or firstname.lastname@example.org.