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New drug therapies improve outlook for people with
pulmonary hypertension
Janet Young seemed like a perfectly healthy 47-year-old
woman until a year ago when she began to tire easily.
"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 and Vascular Center 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.
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