" I started having trouble breathing," she said. "I got light-headed very easily and slept a lot more. Something was wrong, I just didn't know what."

Young, from Marion, Iowa, decided it was time to see her family physician, Mark Tyler, M.D., of Cedar Rapids.

Tyler performed an EKG and, based on his overall evaluation, referred her to two other Cedar Rapids physicians, Darrel Dennis, M.D., a pulmonologist, and Lawrence Cook, M.D., a cardiologist. Their tests that confirmed a diagnosis: primary pulmonary hypertension.

Pulmonary hypertension is a debilitating condition in which the patient has trouble pushing blood through the lungs, where it oxygenates. It often strikes people during their 40s and 50s.

"Until recently, these patients have fared poorly," said Jess Mandel, M.D., a University of Iowa Health Care pulmonologist. "Half the patients diagnosed with primary pulmonary hypertension died within two or three years. There were no effective medications available, so there wasn't a lot that could be done."

Mandel specializes in treating patients with the condition, having trained at the Harvard Medical School program and directed the pulmonary hypertension program at Beth Israel Deaconess Medical Program in Boston. He joined the UI Hospitals and Clinics staff last year to co-direct the Pulmonary Hypertension Program with Ron Oren, M.D., a UI Heart Care cardiologist.

" I'm very excited to be here because there have been some terrific breakthroughs recently in terms of new medications and we have a very organized system for treating patients effectively," he said. "Our multidisciplinary team includes specialists in cardiology, pulmonology, nursing, rheumatology, and thoracic surgery."

Among the UI program's patients is Young, whose lifestyle improved dramatically after she began treatment with a continuous infusion of Flolan® (epoprostenol). Flolan is a naturally occurring molecule ( prostacyclin) that has been approved by the FDA for treatment of patients with pulmonary hypertension.

" I'm much better off than I was before," Young said. "I could barely walk 10 feet without being exhausted. Now I can be out all morning and not have any trouble. I don't huff and puff as much. It's wonderful … my life is much better than it used to be."

Oren said the benefits of newly developed medications have significantly improved the lifestyles and outlook for patients with primary pulmonary hypertension.

" We still have a lot to learn but Flolan has been a terrific breakthrough," Oren said. "So has another new FDA-approved drug, Tracleer® (bosentan). It has fewer side effects and is easier to administer, although there is less worldwide experience with its use. We hope these advantages will allow use much earlier in the disease process and limit disease progression."

Mandel said the symptoms of pulmonary hypertension are so nonspecific--shortness of breath, for instance--that it is often misdiagnosed as asthma or some other condition. "The good news, however, is that with these new therapies, many patients are being helped in ways that were impossible a few years ago."

Mandel said UI Health Care's Pulmonary Hypertension Program serves as a resource for physicians and patients in Iowa and adjacent states.

" We spend a lot of time on the phone with physicians, getting involved in helping these patients navigate what is sometimes a difficult road toward evaluation and treatment of this very difficult disease," he said. "We do everything in partnership with physicians in the community and with the patients themselves."

At a glance

• Primary pulmonary hypertension (PPH) has been linked to the use of appetite suppressant drugs or may occur without an obvious cause.
• Pulmonary hypertension may result from another condition such as heart disease, lung disease, or liver disease, as well as HIV infection or systemic connective tissue disease (such as scleroderma).
• Pulmonary hypertension may have a genetic component. PPH affects more women than men and an estimated six to 10 percent of cases run in consecutive family generations.

For more information about the program, patients and families should contact UI Health Access and ask for Traci Stewart, R.N., in the Pulmonary Hypertension Program. For consultation or referral, physicians should call UI Consult.

Janet Young's activity level is far greater following specialized treatment for pulmonary hypertension.

For more information:

Ron Oren, M.D.

Flolan®