updated 2/25/2004 6:27:53 PM ET 2004-02-25T23:27:53

A rare, deadly lung condition is so common among people with sickle cell anemia that testing for and treating it could help many patients live longer, healthier lives, a study found.

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Experts called the study a major advance in the fight against sickle cell disease, an inherited blood disorder that affects 70,000 to 100,000 Americans.

“The paper will change the standard of care for sickle cell patients across the country,” said Dr. Clint Joiner, director of the sickle cell center at the Cincinnati Children’s Hospital Medical Center.

One-third of sickle cell patients have the lung condition, which makes them 10 times more likely to die within 18 months, doctors at the National Institutes of Health and Howard University reported in Thursday’s New England Journal of Medicine.

Doctors must now find the best ways to treat sickle cell patients who also have pulmonary hypertension, or high blood pressure in the lungs. It is a condition in which narrowed, thickened blood vessels in the lungs keep the body from getting enough oxygen.

Researchers do not know if pulmonary hypertension caused the increase in deaths among the 195 patients they studied, or was a sign of severe sickle cell disease. But it was present in 63 patients, or 32 percent.

All 195 were in stable condition at the start. Within 18 months, nearly 20 percent of those with both conditions were dead, compared to fewer than 2 percent of those without pulmonary hypertension.

Tests urgently needed
The numbers indicate that patients with sickle cell disease should be tested for pulmonary hypertension, said Dr. Mark T. Gladwin of the National Institutes of Health, lead researcher for the study.

“If they have high blood pressure in the lungs, they should intensify their therapy. And consider treatment with drugs to lower blood pressure in the lungs,” he said.

He and other doctors said tests of various therapies for both sickle cell and pulmonary hypertension are urgently needed.

“I think the number of deaths could be cut by 30 percent or more, easily” if all patients were screened and treated, said Dr. Elliott P. Vichinsky, director of the sickle cell center at the Children’s Hospital and Research Center in Oakland. Other doctors wouldn’t go that far.

And Dr. Johnson Haynes Jr. of the sickle cell center at the University of South Alabama cautioned that any treatments should be done as part of controlled tests, until the best ones are found.

Sickle cell disease got its name because a defect in hemoglobin, which carries oxygen, turns blood cells into hard sickles rather than soft doughnuts. The hemoglobin cannot carry as much oxygen as normal cells to start with, leading to chronic anemia and jaundice, and the sickled cells stack up in and block small blood vessels, causing a host of other ailments.

Sickle cell disease, which mostly affects blacks, used to be a childhood death sentence. Now, half of all patients make it past the age of 50, and doctors are learning what the disease does to the older body.

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