Therapies give normal life, despite rare, life-threatening condition

Pulmonary hypertension is a condition in which high blood pressure occurs in the lungs. Patients are short of breath and fatigued.

Twenty years ago, there was no treatment for PAH patients, but they now have options that can help them live a normal life.

PAH patient Tonya Tyler remembers when, a decade ago, walking short distances made her short of breath.

"I'd have to go sit down, lie down. My heart rate went up," she described.

Tyler wasn't diagnosed with PAH until she was 34 weeks pregnant.

"Unfortunately, many women (with PAH) don't survive pregnancy," said Dr. Victor Tapson, a pulmonologist with Duke University Medical Center. "Tonya was fortunate and has done very well."

It is not recommended that women with PAH get pregnant, due to the risks. However, many of the disease's early symptoms might be mistaken for being out of shape or having asthma. If you are unusually short of breath and easily fatigued, see a doctor.

With PAH, the walls of tiny vessels in the lungs tighten. "As these pressures get higher in the lung, the right side of the heart gets bigger and has more trouble pumping blood into the lungs and lead right to heart failure," Tapson said.

The condition is typically caused by a lung disease, connective tissue diseases or heart failure.

Tyler's case is rare because there's no known cause.

She has responded well to the drug Flolan, which is administered by an IV and catheter to her heart. The drug keeps her lungs open. She uses a CADD pump and mixes her medicine every night.

However, Tyler has started a clinical trial for a similar but longer-acting IV medicine called Remodulin.

"We hope that ultimately Tonya can be on a pill, instead of IV therapy," Tapson said.

The FDA recently approved an oral medication, Adcirca or tadalafill, for PAH. While other oral therapies require three pills a day, Adcirca requires only one.

"Things are getting better, and there's more drugs available. And that's our hope for the future – I'll be on oral medication," Tyler said.