Seeking New Treatments for Pulmonary Arterial Hypertension

Sharon Allen hadn’t felt well for a while.

She was often out of breath and felt weak. But stress tests and blood work showed a healthy heart.

Then she passed out, at the airport of all places, hit the pavement hard and was hospitalized.

It was then, after much testing, that she was diagnosed with Pulmonary Arterial Hypertension (PAH).

PAH is caused by a narrowing of the blood vessels that deliver blood from the heart to the lungs in order to pick up oxygen. It is a pulmonary vascular disease, common in patients with autoimmune diseases such as lupus and scleroderma.

Pulmonologist Michael Scharf, MD

Jefferson pulmonologist Michael Scharf, MD

Sharon, a 57-year-old Bloomsburg, PA resident began treatment with her local pulmonologist until he ran out of options and referred her to Jefferson pulmonologist Michael Scharf, MD, Director of the Pulmonary Vascular Disease Program and Pulmonary Outpatient Services, for the advanced care she needed.

“Unfortunately, PAH is rare and the treatment options are not plentiful nor is the condition curable, but we continue to find novel ways to treat the disease and allow these patients to have a normal life,” says Dr. Scharf.

Sharon drove the two and a half hours to Philadelphia and was in Dr. Scharf’s office the following week. He prescribed for her what she calls her “miracle drug”.

Sharon was the first in the region to receive Veletri, a prostacylin medication that opens the vessels to improve blood flow in the small arteries of the lungs, allowing blood to flow more easily through the lungs.

Veletri, like all prostacylins to date can only be administered by infusion or inhalation.  Delivered through infusion, she had to have a catheter implanted in her chest to receive the medication. Sharon carries a bag over her shoulder with a “cassette” of her medication inside, a powder and water mixture she prepares each night.

“It has changed my life,” she says.

After two and a half years on Veletri, she feels almost back to her normal self.  “I can breathe great and do almost anything I want,” she reports.

A new second generation delivery system now allows her to prepare her medication a week in advance, store it in the refrigerator and switch out the cassette each night.

Jefferson is now enrolling patients in a new clinical trial (GRIPHON) to test the efficacy and safety of a new oral prostacyclin for the treatment of PAH.

“This is an exciting trial for patients, like Sharon, who have longed for an easier, more convenient way to get the medication and treatment they need,” says Dr. Scharf.

Sharon continues her busy career at a Christmas bow manufacturing plant and prepares for the birth of her third grandchild.

“I am grateful to Dr. Scharf and his superb care,” she says.  “Life is good.”

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