The natural history of homozygous S sickle cell anemia in two sisters

This article describes the natural history of homozygous S sickle anemia in two sisters who have completely different morbidity patterns. Medical records beginning in May 1976 from the Urban League Sickle Cell program and my office, hospital records, and personal interviews were used. The sisters were older than 20 years when the diagnosis was made. Both had two essentially uneventful pregnancies, and neither was treated with prophylactic transfusions during the pregnancies. One sister has a hemoglobin F level of 23%, has been hospitalized only 15 times to date, and has no gallstones. The other sister has a hemoglobin F level below 3%, has been hospitalized more than 148 times to date, and had a cholecystectomy in 1979. Clinical symptoms in sickle cell patients with high levels of hemoglobin F is reportedly mild, and this has been supported by this study. Likewise, the conclusion by some investigators that prophylactic blood transfusions during pregnancy are unnecessary also has been supported. While many exciting investigative approaches relative to management and prevention of sickle cell anemia continue, it is equally important to maintain emphasis on the variables that encourage and facilitate positive psychosocial adaptations by these patients.