NHLBI stops study of pulmonary hypertension treatment in sickle cel...(. An estimated 70000 to 100000 people in...),Health

Date:7/28/2009

. An estimated 70,000 to 100,000 people in the United States have sickle cell disease, primarily African Americans and, to a lesser degree, people whose families come from South or Central America. Patients with this disease have abnormal hemoglobin molecules in their red blood cells. The abnormal molecules deform the red blood cells, causing them to clump together and block blood flow through blood vessels, leading to painful sickle cell crises, organ damage, and anemia. Life-threatening complications include infections, acute chest syndrome, stroke, and pulmonary hypertension. Painful crises are the leading cause of emergency room visits and hospitalizations of people who have sickle cell anemia.

There are currently no established guidelines for treating pulmonary hypertension in patients with sickle cell disease. Pulmonary hypertension can lead to heart failure and death as the heart must work harder to push blood into the lungs. In general, intensive management of sickle cell disease through hydroxyurea, blood transfusions, and/or bone marrow transplantation may be indicated. Other treatment options may include blood-thinning medicines, diuretics, and oxygen therapy.

"As new treatments have evolved over the past several years, sickle cell patients are living longer than in previous decades, presenting new challenges for managing chronic problems and complications such as pulmonary hypertension," noted Susan Shurin, MD, NHLBI deputy director and a hematologist. "The NHLBI continues to maintain a strong commitment to supporting research in adults as well as in children to discover new and better ways to improve quality of life and survival for patients with sickle cell disease."

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Contact: NHLBI Communications Office NHLBI_news@nhlbi.nih.gov 301-496-4236 NIH/National Heart, Lung and Blood Institute Source:Eurekalert

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