New guidelines to treat rare Pulmonary Arterial Hypertension

MURRAY, Utah (ABC 4 Utah) - What used to be a death sentence back in the 1980s, now has new guidelines for treatment.  When tiny arteries in the lung become narrow and block the heart from pumping blood normally, it's called pulmonary arterial hypertension.  It’s extremely rare.  Only one in 100,000 people have it, but left untreated, the disease can lead to heart failure.  In our continued partnership with Intermountain Medical Center, we find out the warning signs and the advancements in treatment in hopes to find a cure.

“First of all it was a cough; a cough for about six months. Then it was I couldn't make it to the mailbox,” said double lung transplant recipient Jerusha Daines.

Jerusha Daines just figured she was out of shape, but it was much more.

“One day you could do it all and the next day you couldn't breathe,” said Daines.

After several doctors’ appointments, she was diagnosed with pulmonary arterial hypertension.  The arteries in her lungs were narrowing and putting extra strain on her heart.  The treatment for most with that condition wasn't pleasant.

“A patient had to have an intravenous catheter in that ran 24hrs a day and any interruption in that therapy, literally could spell sudden death for that patient,” said Intermountain Medical Center Internal Medicine Dr. Greg Elliott

Intermountain Medical Center Internal Medicine Dr. Greg Elliott says when he started practicing in the 80s, people were given a year to live.  Better science changed that.

“Therapies really have emboldened the medical community to say we can treat this disease and there is hope for the patient that's newly diagnosed,” said Dr. Elliott.

Dr. Elliott worked to prepare new guidelines to help doctors treat the rare disease.  Now patients can use pills, the intravenous catheter, and a last resort option.

“Lung transplantation is often the last thing we do when all of our available and effective medical therapies have failed,” said Dr. Elliott.

Jerusha's symptoms were only getting worse.  So almost four months ago, she made the tough decision to undergo a double lung transplant.  The gift of life gave her a new lease on life.

“You can live again, you can breathe, you can walk, you can go places, and you don't have anything hooked to you. You’re free to do stuff and even though you get tired you have energy to see things in a whole different life,” said Daines.

The hope in Jerusha's eyes inspires Dr. Elliott that more medical advancements can help those suffering from pulmonary arterial hypertension.  Treatments are better, but there's still no cure.

“We need more studies and science and research in this area,” said Elliott.

Dr. Elliott says there is evidence of a genetic mutation that may lead to pulmonary arterial hypertension.  He says the key is not to ignore persistent shortness of breath and excuse it for getting older or being out-of-shape.  Dr. Elliott says seek your physician if you have any questions.

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