Sickle cells tend to become trapped and destroyed in the liver and in the spleen. This results in a shortage of red blood cells called anemia which, when severe, can cause the patient to be pale, short of breath and easily tired. Sickle beta thalassemia is a less common form of sickle cell disease. The effects of sickle cell disease vary greatly from one person to the next. Infants and young children with sickle cell disease are especially vulnerable to severe bacterial infections, such as those that cause meningitis and pneumonia.
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 the deaths that result from them. 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, some 30 states are testing newborns for sickle cell disease. Newborn testing alerts physicians to begin treatment before dangerous infections occur.