CINCINNATI—Hundreds of new pulmonary arterial hypertension (PAH) cases are diagnosed each year in the United States, pushing the total number of cases over the 200,000 mark.
But unfortunately the progressive disease, often under-recognized and sometimes misdiagnosed, has no cure.
However, there are new treatments that can improve the quality of life for some pulmonary hypertension sufferers, says Jean Elwing, MD, UC Health pulmonologist.
Pulmonary arterial hypertension is a blood vessel disorder of the lungs in which pressure in the pulmonary arteries, the blood vessels that lead from the heart to the lungs, rises above normal levels.
Symptoms include chest pain, dizziness, fatigue and shortness of breath which can occur even during routine activities such as walking across the room.
Elwing says people suffering from pulmonary arterial hypertension mistakenly think their symptoms are caused by aging, being out of shape or weight gain.
“Pulmonary arterial hypertension is a serious disease and can be life-threatening,” she says. “Symptoms should not be ignored.”
Two new medications were recently approved by the U.S. Food and Drug Administration for the treatment of World Health Organization (WHO) Group 1 PAH: Tyvaso (Treprostinil), an inhaled medication, and Adcirca (Tadalafil), an oral therapy.
WHO Group 1 cases include those that are idiopathic (arising spontaneously), genetic or are associated with other diseases including lupus, rheumatoid arthritis, HIV and sickle cell anemia.
“Tyvaso was approved for PAH therapy in July 2009,” says Elwing. “It is administered via a specialized nebulizer four times daily. It has been shown to increase exercise tolerance in PAH patients.”
Elwing says Adcirca is another treatment option that is taken once a day in the form of two tablets and has also been shown to improve patients’ exercise tolerance.
“Both of these medications treat the illness while allowing patients to lead active lifestyles,” she says.
With Pulmonary Hypertension Month being observed in November, Elwing urges the public to be aware of this treatable illness.
“PAH often goes unrecognized and is often undertreated,” Elwing says. “It is a chronic disease that requires close monitoring. The quicker we diagnose the illness, the sooner we can impact the patient’s quality of life.”
“There’s no way to prevent the development of this illness,” she adds. “Recognition is the key.”
Elwing said people should see a doctor if they experience the following:
- Shortness of breath with minimal exertion
- Decreased exercise tolerance
- Chest pain
- Dizzy spells
She said echocardiograms are generally used to check for the illness, but heart catheterization is needed for confirmation.
“The average life expectancy for someone with untreated pulmonary arterial hypertension who is diagnosed in the later stages of the disease is only about three years,” she says. “But if it’s detected in the early stages and treated, people can live a semi-normal life. We want people to be aware of the symptoms of PAH and ask their physician for help.”
Elwing receives funding from the makers of Tyvaso in the form of a patient education grant. She also serves on the advisory board of United Therapeutics, the maker of both medications.