Prophylaxis with Oral Penicillin in Children with Sickle Cell Anemia

Abstract

Children with sickle cell anemia have an increased susceptibility to bacterial infections, especially to those caused by Streptococcus pneumoniae. We therefore conducted a multicenter, randomized, double-blind, placebo-controlled clinical trial to test whether the regular, daily administration of oral penicillin would reduce the incidence of documented septicemia due to S.pneumoniae in children with sickle cell anemia who were under the age of three years at the time of entry. The children were randomly assigned to receive either 125 mg of penicillin V potassium (105 children) or placebo (110 children) twice daily. The trial was terminated 8 months early, after an average of 15 months of follow-up, when an 84 percent reduction in the incidence of infection was observed in the group treated with penicillin, as compared with the group given placebo (13 of 110 patients vs. 2 of 105; P = 0.0025), with no deaths from pneumococcal septicemia occurring in the penicillin group but three deaths from the infection occurring in the placebo group. On the basis of these results, we conclude that children should be screened in the neonatal period for sickle cell hemoglobinopathy and that those with sickle cell anemia should receive prophylactic therapy with oral penicillin by four months of age to decrease the morbidity and mortality associated with pneumococcal septicemia.

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

“…The trial was halted early with an average 15 months of follow-up, after an interim analysis showed an 84% relative risk reduction, with 13 and 2 episodes of pneumococcal septicemia in the placebo and treatment group, respectively. Further, three deaths occurred from invasive pneumococcal infection in the placebo group and no deaths occurred in the treatment group [34]. As a result of this trial, children with SCA in high- …”

“…A single center trial [33], followed by a multicenter, randomized clinical trial unequivocally demonstrated the benefit of penicillin prophylaxis for preventing SCD related pneumococcal sepsis. In the "PRophylaxis with Oral Penicillin in children with Sickle cell anemia" (PROPS) trial, children with SCA younger than 3 years were randomly assigned to receive either 125 mg of penicillin V (105 children) or placebo (110 children) twice daily [34]. The trial was halted early with an average 15 months of follow-up, after an interim analysis showed an 84% relative risk reduction, with 13 and 2 episodes of pneumococcal septicemia in the placebo and treatment group, respectively.…”

Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance

“…Moreover, the acute painful episode is the number-one cause of hospitalization of patients, especially adults, with SS [1]. With the advent of newborn screening [2][3][4], penicillin prophylaxis [5], routine immunization, and improving health care [1,6], most children with SS are now surviving into adulthood. With age, new complications of the disease emerge, chronic pain syndromes evolve, and progressive organ failure sets in [7][8][9].…”

Effectiveness of preoperative screening for sickle cell disease in a population with a newborn screening program: a cohort study

“…Despite a lack of randomized controlled trials of newborn screening for SCD, 16 there is clear benefit to newborn screening in certain populations. [17][18][19][20] Similarly, the feasibility of an adequately powered randomized trial for the purpose of evaluating preoperative screening is unlikely, and we may have to rely on large epidemiological studies. The external validity of our results may be limited, as the prevalence of SCD may vary amongst different populations, as may screening techniques, social attitudes towards screening, available resources, and surgical caseloads.…”