HEALTH & FITNESS

By Tim McNellie | Photo by David Pinchot

Madeline Kosky.

After more tests, including a cardiogram, lung scan, and heart catheterization, Dr. Srinivas Murali diagnosed her with pulmonary hypertension, a condition in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels and can become life threatening. As the disease progresses, the arteries become damaged, which can eventually destroy the heart and lungs.

“I couldn’t walk 10 feet without having to sit down,” Kosky says. “I couldn’t breathe, I wanted to lie down all day. It was an effort to even eat. You’re just tired all the time, and your whole life slows down.”

Though the situation at the time seemed bleak, Kosky, then 64, was enrolled in a drug trial for Remodulin. While not pleasant – the treatments involved feeding medicine into her torso through a catheter-like feeding pump – the drug did help. She persisted with that treatment until another drug, Tracleer, became available in pill form a few years later. Kosky made the switch, and has regained some energy and independence.

It wasn’t surprising that the general practitioner couldn’t identify the condition. It’s so rare that most physicians will never see a case in their lifetimes. The Pulmonary Hypertension Association lists only 16 doctors specializing in the condition in all of Pennsylvania – including Murali, a nationally-renowned expert in the field (he’s written more than 100 publications on the disease and won numerous awards). Only a few hundred thousand people in the world have it. Pulmonary hypertension can be caused by emphysema, bronchitis, congenital defects, liver disease, and diet drugs like fen-fen; some cases seem to have no cause at all. The condition doesn’t discriminate, though. Forbes magazine recently profiled a 37-year-old mother who had it. Kosky personally met a 10-year-old girl who had gone through organ transplantation because of it.

Kosky’s experience has made her eager to raise awareness about pulmonary hypertension so that better treatments can be found and so that others in her situation may be diagnosed sooner. She’s not alone. One conference that she and husband John attended in June attracted more than 1,000 people. Secretary of State Condoleezza Rice gave a piano performance at one pulmonary hypertension benefit. New drugs are allowing people to live with the condition for longer periods of time, and if nothing else, Kosky is evidence that with persistence, hope, and, especially, the right treatments, pulmonary hypertension doesn’t mean one can’t live a full life.

“At first you think, ‘Well, this is it for me,” she says. “But your situation can be manageable, and now there are some days I don’t even think about it. I was a very active person before, and while I still have to walk up steps very slowly, or I get out of breath, I’ve gotten back to doing things I like to do.” •