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Researcher Gives an Encouraging Update on Sickle Cell Disease

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Oct. 18, 1999 (Washington) -- Parents of children with sickle cell disease have reason to be optimistic and encouraged, according to experts. "During the past 25 years, research studies have made inroads into increasing the life expectancy, preventing complications, and managing the disease," says Clarice Reid, MD. She presented her findings here recently at the 69th annual meeting of the American Academy of Pediatrics.

Sickle cell disease, also known as sickle cell anemia, is an inherited disorder and is seen almost entirely in blacks. Close to 10% of blacks have a minor form, known as sickle cell trait, that usually does not lead to any symptoms. However, in those with the more severe form of the disorder, the symptoms usually begin during childhood and include pain in certain areas of the body, such as multiple joints and the chest, but also can lead to strokes, heart attacks, and gall bladder disease at a young age. The pain and the more serious consequences of sickle cell disease occur because of the red blood cells changing shape and getting stuck in the blood vessels. Oxygen and nutrients subsequently cannot get to the various areas of the body. The abnormal red blood cells then burst, causing the blood count to fall, which often leads to severe anemia.

One of the most significant advances in sickle cell disease research, according to Reid, has been the development of the drug hydroxyurea. "Hydroxyurea has helped decrease painful episodes, hospitalizations, and transfusion requirements in adults," says Reid. In addition, some studies are now looking at the use of penicillin to prevent pain in the chest due to sickle cell attacks. Researchers are "cautiously optimistic," Reid tells WebMD. Reid is the former research director of the National Institutes of Health.

Reid presented data from major studies performed during the past 25 years that clearly indicate children are growing into adulthood and living longer, more productive lives. Today, 85% of children with sickle cell disease live beyond age 20. The average age at death for females is 48 and for males is 42.

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