CEDAR FALLS -- When Lois Rippe agreed in 2003 to take part in a clinical trial for a new medicine to treat the symptoms of pulmonary hypertension she didn't think she'd live to see the end of the experiment.

The Cedar Falls woman had been recently diagnosed with the life-threatening condition, a rare, incurable disease that causes high blood pressure in the arteries that supply the lungs. Doctors told her that most patients with the disorder died within two to five years.

"My options weren't good," said Rippe, who suffers from extreme shortness of breath due to the condition. "There wasn't much there for me to do. I just decided to take a step in faith."

The retired Hawkeye Community College accounting instructor started taking 10 milligrams daily of Ambrisentan to manage the disease. She saw an improvement in her health almost immediately. In June, the oral medication, now renamed Letairis, was approved for clinical use by the Food and Drug Administration.

"It was a godsend," said Rippe, 68. "Ambrisentan gave me back my life."

Like most pulmonary hypertension patients, Rippe was frustrated to find her daily activities curtailed by the disease. The condition causes breathing complications, and completing even simple tasks like doing laundry or fetching her family's mail proved taxing.

"But with the drug I was able to walk up the stairs and through the rest of my house," said Rippe, who also has limited scleroderma, a connective tissue disease that often occurs in conjunction with pulmonary hypertension. "I could do more. Plus, I'm convinced that it has extended my life."

Dr. Michael McGoon, a cardiovascular disease consultant at Mayo Clinic in Rochester, Minn., monitored Rippe throughout the clinical trial. She continues to see him to manage her symptoms.

"Although we like to cure and bring people back to normal, preventing them from getting worse is also pretty important," said McGoon, who also serves as chair of the Pulmonary Hypertension Association. "She's achieved that and is doing very well."

More experimental drugs are being tested to combat the disease, and McGoon is hopeful his patients will soon have access to additional treatment methods. But for the time being Rippe is thankful for the gains Letairis has offered her.

The medication, produced by California-based Gilead Sciences, has been shown to help pulmonary hypertension patients walk longer distances and has delayed the disease's progression. Although it can cause liver damage, Rippe has experienced no negative side effects from the medication, which works by preventing the narrowing of the blood vessels in the pulmonary artery.

"She's been able to get out and enjoy coffee with friends," said Rippe's husband, Duane, a retired Cedar Falls industrial technology teacher. "We are able to travel to see our children and grandchildren."

Letairis is only available through the Letairis Education and Access Program. Rippe's prescription, offered free of charge during the trial period, now costs her $4,136 a month. Fortunately, her insurance covers a third of the cost and the Chronic Disease Fund and Caring Voice Coalition have helped cover the remaining amount.

"It's very costly," Rippe said. "But I really feel as if it's helped me, and it could help others, too."

Rippe uses portable oxygen when she is out and about, and between Letairis and the extra air, she is able to enjoy cheering on the University of Northern Iowa football and men's basketball teams. On weekday mornings she meets friends at a local bakery.

Simple tasks, like getting dressed, still wear her out. But even with those challenges, Rippe said she is thrilled to have seen the medication reach a successful end to its clinical trial. Recent data suggests some pulmonary hypertension patients are now able to live with the disease 15 to 20 years or longer, thanks to an increase in treatment options.

"To me, life is a gift from God," Rippe said. "And I'm thankful for every day he gives me."

Contact Mary Stegmeir at (319) 291-1482 or mary.stegmeir@wcfcourier.com.

Pulmonary Hypertension occurs when the blood pressure in the pulmonary artery rises above normal levels. The condition is life threatening. Symptoms include shortness of breath with minimal exertion, fatigue, chest pain, dizzy spells and fainting. Often the disease occurs along with other conditions, including breathing and connective tissues disorders.

In the U.S., between 500 and 1,000 new cases of primary pulmonary hypertension are diagnosed annually. The greatest number is reported in women between ages 20 and 40.

For more information about the disease visit the Pulmonary Hypertension Association, at www.phaassociation.org. To learn more about Letairis, go to www.letairis.com.

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