SALT LAKE CITY — Early diagnosis might be the key for those inflicted with a rare but fatal lung disease as Utah researchers aim to help save the lives of many with proper treatment.

The 200 or so patients being treated in Utah hospitals for pulmonary arterial hypertension — as well as the thousands yet to be diagnosed — will likely take heart with the findings of the national study, as improved treatment could lead to less suffering.

The study, released Monday by researchers at Intermountain Medical Center, reveals that more than 21 percent of all patients with the disease actually suffer for more than two years before being correctly diagnosed with PAH.

"For a lot of patients, that means the treatment is more difficult and the damage is irreversible," said Intermountain pulmonologist, researcher and lead author of the study Dr. Lynnette Brown. "Finding out which patients are getting a delayed diagnosis is the first step in identifying them earlier, when treatment is easier and hopefully more effective."

Research also found that patients younger than age 36, as well as those who had previously been diagnosed with a common respiratory disorder (such as obstructive airway disorder or sleep apnea), were most likely to receive a delayed diagnosis for PAH, a rare but fatal disease that occurs when small arteries in the lungs become narrowed and unable to carry as much blood as healthy arteries.

Pulmonary arterial hypertension can end up crippling the heart, as pressure builds within the heart while it works extra hard to move blood into the lungs. Effective treatment exists, but is sometimes worthless for those who receive a later diagnosis.

Brown and other researchers examined the medical records of nearly 2,500 PAH patients nationwide, including many of those currently being treated at IMC or University Hospital, the only facilities in the region that are capable of treating the complicated disease at an advanced stage.

The research, which is published this week in the July issue of Chest, the journal of the American College of Chest Physicians, cites various possible reasons for the delay in diagnosis, including a list of all too common symptoms that can be related to other, more common disorders, as well as the fact that younger patients experience less severe symptoms that lead to doctors not suspecting the worst possible scenario.

Younger patients also make up one of the largest groups of uninsured Americans, making them less likely to seek treatment for potentially less severe symptoms, according to the study.

"We have a lot more medications available to fight pulmonary arterial hypertension, but we can't use them all if we don't get to patients early enough in the course of the disease," said Dr. Gregory Elliott, chairman of the Department of Medicine at IMC. He said that treating PAH patients sooner could lead to increased survival rates.

"If a young person comes in complaining of shortness of breath, it's alright to suspect something common. But if a patient is getting worse and not responding to treatment, it's time to look for something else," Brown said.


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