Certain conditions, such as enlargement of the spleen and infections, may worsen the anemia by speeding up destruction of red blood cells. Infections also can reduce red blood cell production. There are several forms of sickle cell disease. The effects of sickle cell disease vary greatly from one person to the next. Some affected people rarely are ill while others are frequently hospitalized.
Infections are the leading cause of death in children with sickle cell disease.
However, early diagnosis and treatment dramatically reduce the risk of infections and death. In 1987, a National Institutes of Health panel recommended that all babies be screened at birth for sickle cell disease. All states now screen babies' blood for two or more inherited diseases, and the test for sickle cell disease is done using the same blood sample. To date, more than 40 states are testing newborns for sickle cell disease. Newborn testing alerts physicians to begin treatment before dangerous infections occur.