Sickle Cell Disease

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Hemoglobin electrophoresis is the most commonly performed test to determine a person's hemoglobin type. Widely available in the United States, it uses an electric charge to differentiate between normal and abnormal types of hemoglobin and is performed after a simple blood draw. Hemoglobin electrophoresis is performed on all newborns in more than 40 states.

Early diagnosis of SCD is critical so that children who have the disease can begin receiving the proper care as soon as possible—and so their parents can become educated about the disease. Evidence indicates that early identification of children with SCD, along with appropriate treatment, significantly improves their chances of survival during childhood.

Some groups have suggested that student and professional athletes be screened for the sickle-cell trait, based on the belief that those with the trait are more likely than those without it to develop certain complications of vigorous exercise and dehydration, such as heat stroke, exhaustion, and muscle damage due to overexertion. This notion is controversial, however, because many physicians believe that athletic coaches and trainers should safeguard all athletes, regardless of their sickle cell trait status, against complications of vigorous exercise—and that athletes with the trait should not be singled out for special treatment. Moreover, U.S. deaths due to such complications still occur predominantly among athletes without the sickle-cell trait.

SCD also can be diagnosed before a child is born with a fetal blood draw—an option some parents choose, particularly if they both carry the hemoglobin S gene, or if one carries the hemoglobin S gene and the other carries a different abnormal hemoglobin, such as hemoglobin C. This test tells parents whether their child will be born with one of the types of SCD. If SCD is diagnosed, parents may choose to terminate the pregnancy.

Other tests

To optimally manage their disease, people with SCD are required to undergo a number of regular tests and monitoring procedures. Some tests are age specific; others are necessary throughout life.

Last reviewed on 1/28/10

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