Thursday, February 26, 2004

Strained Lungs: A study today in the New England Journal of Medicine says that patients with sickle cell disease are at risk for pulmonary hypertension, a condition which can be fatal (not to mention difficult to treat):

Gladwin's team followed 195 patients, average age 37, for an average of 18 months. They screened each with Doppler echocardiography to assess the pressures within their pulmonary arteries, which carry blood to the lungs. It detected high blood pressure in the lungs in 32 percent of the patients.

Over the next two years, 20 percent of those with pulmonary hypertension died, while all but two of those without it survived.


The patients in the study who were found to have pulmonary hypertension were referred for treatment during the course of the study, so the mortality rate could be much higher in people with sickle-cell anemia whose pulmonary hypertension goes undetected. The NEJM concludes that doctors should screen sickle-cell disease patients yearly for the complication:

"I think every adult with sickle cell needs an echo every year," Vichinsky says. But he and Gladwin agree that persuading insurance plans to pay for the test, which Gladwin estimates costs $500 to $800, is not always easy or possible.

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