Pulmonary Complications of Sickle Cell Anemia

One of every 650 African Americans (0.15%) is born with sickle cell disease and about 8% are heterozygous for the sickle cell gene. According to the United States Census Bureau there were 35,509,000 African Americans counted in the year 2000, which would indicate that there are approximately 50,000 patients with sickle cell anemia in this country. Thirty five per cent of the African American population was between 30 and 54 yr of age in the year 2000. This demographic explains the increasing number of adult patients with sickle cell disease. However, the increased number of adults with sickle cell anemia is due only in part to population growth. Mortality rates for children with sickle cell anemia have declined because of penicillin prophylaxis, Haemophilus influenzae and Streptococcus pneumoniae vaccination, widespread implementation of newborn screening programs for early detection, and improvements in parental education (1–5). Despite significant improvements in the life expectancy of patients with sickle cell disease the median age at death is 42 yr for men and 48 yr for women (2). Pulmonary complications account for a large proportion of deaths among adults with sickle cell anemia (Table 1) (2, 6–8). According to the Cooperative Study of Sickle Cell Disease (CSSCD), a prospective multicenter study of 3,764 patients, more than 20% of adults likely had fatal pulmonary complications of sickle cell anemia (2). Acute and chronic pulmonary complications of sickle cell anemia are common but often underappreciated by healthcare providers. These conditions have clearly emerged as major threats to the well-being and longevity of patients with sickle cell disease.