Good management maximizes outcome. Priapism is a distressing complication that occurs at all ages, is difficult to treat, and causes a high incidence of impotence. End organ damage occurs throughout life. Chronic pulmonary problems causing respiratory failure and pulmonary hypertension and renal failure are leading contributors to death in older patients. Weight may lag behind that of children without sickle cell disease, however, there is considerable variability.
Height is less in older children and adolescents but differences disappear in adulthood.
Puberty is delayed significantly in males and females with sickle cell disease. Factors contributing to the delay include anemia, fetal hemoglobin levels, increased protein turnover, and zinc deficiency. Treatment includes good general nutrition, vitamin supplementation for deficiencies, and reassurance that normal maturation will occur. Management and Therapy of Sickle Cell Disease. NIH Publication No.