Bacteremia in children with sickle hemoglobin C disease and sickle beta⁺-thalassemia: Is prophylactic penicillin necessary?

Objective: To characterize the incidence of bacteremia and its potential for progression to septicemia in children with sickle hemoglobin C disease and sickle β⁺-thalassemia to assess the need for penicillin prophylaxis. Study design: Retrospective chart review of the frequency and natural history of bloodstream infection in such patients not receiving prophylactic penicillin therapy and followed up in a single institution. Results: During more than 842 patient-years of observation in 242 patients with sickle hemoglobin C disease, 15 episodes of bacteremia occurred in nine patients. Septicemia was fatal in one patient. The overall incidence of bacteremia, 1.8 events per 100 patient-years (95% confidence limits: 0.8, 2.8) in patients with sickle hemoglobin C disease, was similar to that in hematologically normal children. One episode of bacteremia occurred in a patient with sickle β⁺-thalassemia. Conclusions: The incidence of bacteremia is not increased in young patients with sickle hemoglobin C disease and sickle β⁺-thalassemia. Further, unlike its course in children with sickle cell anemia, it rarely evolves into life-threatening septicemia. This probably results from the maintenance of relatively intact splenic function during infancy and early childhood in patients with sickle hemoglobin C disease and sickle β⁺-thalassemia. Prophylactic penicillin therapy may not be required in these patients. (J PEDIATR 1995;127:348-54).