Background This is an update of a Cochrane Review first published in 2002, and previously updated in 2012. People with
sickle cell disease are particularly susceptible to
infection. Infants and very young children are especially vulnerable, and the 'Co-operative Study of
Sickle Cell Disease' observed an incidence rate of 10 per 100 patient years of pneumococcal septicaemia in children under the age of three.Vaccines, including customary
pneumococcal vaccines, may be of limited use in this age group. Therefore, prophylactic
penicillin regimens may be advisable for this population.Objectives To assess the effects of prophylactic
antibiotic regimens for preventing
pneumococcal infection in children with
sickle cell disease.Search methods We searched the Cochrane
Cystic Fibrosis and
Genetic Disorders Group Haemoglobinopathies Trials Register, which is comprised of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 26 June 2014.Selection criteria All randomised or quasi-randomised controlled trials comparing prophylactic
antibiotics to prevent
pneumococcal infection in children with
sickle cell disease with placebo, no treatment or a comparator
drug.Data collection and analysis Both authors independently extracted data and assessed trial quality.Main results Five trials were identified by the initial search, of which three trials met the inclusion criteria. All of the included trials showed a reduced incidence of
infection in children with
sickle cell disease (SS or Sβ0Thal) receiving prophylactic
penicillin. In trials which investigated initiation of
penicillin on risk of
pneumococcal infection, the odds ratio was 0.37 (95% CI 0.16 to 0.86), while for withdrawal the odds ratio was 0.49 (95% CI 0.09 to 2.71). Adverse
drug effects were rare and minor. Rates of
pneumococcal infection were found to be relatively low in children over the age of five.Authors' conclusions Prophylactic
penicillin significantly reduces risk of
pneumococcal infection in children with homozygous
sickle cell disease, and is associated with minimal adverse reactions. Further research may help to determine the ideal age to safely withdraw
penicillin.