The following is a transcript of a report by medical editor Marilyn Brooks that first aired Oct. 9, 2006, on WTAE Channel 4 Action News at 5 p.m.

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Just about everyone knows someone with high blood pressure or hypertension, but you should also know about primary pulmonary hypertension, which is high pressure in the lungs, and until recently, there hasn't been much help for it.

It is a disease that affects 4,000 nationwide, and the numbers are growing. About 400 new cases are discovered every year, 80 percent being premenopausal women.

At 73, Madeline Kosky is something of a miracle. Not only because she shows no sign of shortness of breath or fatigue, but because she is alive.

"I've been blessed, I've been truly blessed," said Kosky.

"There's been such a great improvement in Madeline, it's just unbelievable," said Kosky's husband, John.

If you could step back in time to August 2001, you would see a different Madeline, sick from primary pulmonary arterial hypertension -- a disease in which pressure rises in the pulmonary artery that connects the right side of the heart to the lungs. High pressures cause shortness of breath and fatigue because the heart works harder to oxygenate the blood.

"These symptoms progress over time and eventually, these patients all develop congestive heart failure," said Dr. Srinivas Murali.

Eventually, they all die. In 2001, Kosky's life was extended with Remodulin, a new drug infused 24 hours a day through a pump in her abdomen.

There's a relatively new drug that turned out to be a magic bullet for her though: Tracleer. It works by blocking a certain hormone called endothelin, which is found in very large amounts in people who have pulmonary hypertension.

Tracleer doesn't work for all of them. Patients vary, and so does the cause of their pulmonary hypertension. If Tracleer doesn't work, doctors have others, and more are coming down the pharmaceutical pipeline.

"There's days that I forget that I even have it and I say there's nothing wrong with me," said Kosky.

There's going to be a conference at the Omni William Penn Hotel on Oct. 14, not only for health professionals and patients, but experts who are coming from all over the country to hear and be heard.

For more information on the event, visit www.PAHsymposium.com.

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