Sickle cell anemia (SCD) is a chronic hemolytic anemia affecting 1 in 400 African Americans in the United States. End-organ damage from the persistent anemia and vaso-occlusion from irreversibly sickled cells has been well documented. One of the earliest organs affected is the spleen, placing children with SCD at increased risk for overwhelming bacterial sepsis. Patients with SCD also have poor opsonization of encapsulated organisms, related to decreased serum concentrations of opsonic antibodies. Asplenic patients require higher concentrations of antibody to effectively eliminate organisms, placing SCD patients at even higher risk for sepsis.

Streptococcus pneumoniae is the most common cause of sepsis in SCD patients. Early detection with newborn screening programs, allowing for early institution of penicillin prophylaxis, and widespread availability of immunizations against pneumococcus and Haemophilus influenzae type b have dramatically reduced the incidence of bacterial sepsis. Nonetheless, SCD patients with fever demand special consideration and treatment to prevent adverse clinical outcomes.