Twenty years ago an eight -year-old Lexington boy, Nathan Matuszewicz ,lost his life to a disease affecting his lungs. August 18th,his family and friends are once again sponsoring a 5k run-walk event at Keeneland to raise awareness of the disease.
A lot has changed over 20 years in relationship to the disease once called “primary pulmonary hypertension”. Dr. David Booth, director of pulmonary hypertension services at the Gill Heart and Vascular Institute at University of Kentucky, says this pulmonary vascular disease which causes high blood pressure in the lungs is now referred to as idiopathic “pulmonary arterial hypertension” or P-A-H .
Dr. Booth says people of any age or any gender can have the disease. He says, in the United States, the first drug for P-A-H, Epoprostenol, was FDA approved in 1995. “Over the last 12-15 years there’s now been several other drugs approved for the treatment of pulmonary hypertension and these are effective drugs. And the way that we diagnose pulmonary hypertension, the test is what we call a right heart catheterization where we directly measure the blood pressure in the lungs, is really what’s necessary in order to diagnose it.”
Dr .Booth says for people treated the average survival is four and a half to five years.
He says every bit of research is worthwhile because the current medications are highly palliative, not curative.