The Child with Sickle Cell Disease

Abstract

Sickle cell disease is primarily a condition of childhood. Though some patients reach adulthood, the mortality rate is high, even when the standard of medical care is good (1). Death may result from intercurrent infections, especially tuberculosis, cardiac failure, or thrombosis or hemorrhage in vital tissues. Some may die with uremia or some with severe anemia (2). Defined by Sturgis as a chronic, hereditary, hemolytic anemia, sickle cell disease is confined predominantly to Negroes or those with some Negro blood (3). It is considered a syndrome rather than a single entity, as any organ may be affected(4). The disease is thought to be due to a chemical defect in the hemoglobin of the erythrocytes and is characterized by the appearance of a considerable number of sickle-shaped red blood cells in the circulating blood. Anemia, recurrent leg ulcers, cardiovascular manifestations, acute episodes of abdominal pain, jaundice, nausea, and vomiting may be evi-