Background and aims Children with sickle cell disease (SCD) are at an increased risk of developing bacteraemia and other serious bacterial infections, which can be associated with significant morbidity and mortality. Fever, however, is a common symptom in children with SCD and can occur with both infection (bacterial and viral,) and sickle cell crises. This study aimed to look at the incidence of bacteraemia and bacterial infections in children with SCD presenting to a North-East London district hospital with a fever of 38.5 degrees or higher. Methods A retrospective analysis was performed on all children (aged under 16 years,) with SCD presenting with a fever of 38.5oC or higher over a 1-year period. Data was collected for each febrile episode on age of child, type of SCD, final clinical diagnosis, initial White cell count (WCC) and C-reactive protein (CRP) levels, blood culture and microbiology results, length of stay and clinical outcome. Children were divided into those having a definite bacterial infection, suspected bacterial infection (clinically suspected but no microbiological confirmation,) or no bacterial infection. Definite bacterial infection was defined as bacteraemia (the isolation of a non-contaminant bacterial from the blood culture,) or other bacterial infection with positive microbiological confirmation. Results Over the 1-year period there were 88 episodes analysed in 59 children. Definite bacterial infection occurred in 8% of febrile episodes of which 3.4% had bacteraemia. (Streptococcus pneumonia, Salmonella hartford, Salmonella typhirium.) Suspected bacterial infection occurred in a further 33% of episodes. In 59% of episodes the final diagnosis was either a sickle cell crisis or viral illness (no bacterial infection.) Diagnosis did not vary significantly by haemaglobinopathy. One death occurred from Salmonella typhirium septicaemia. Average length of stay varied from 3.6 days in the group with no bacterial infection to 8.9 days in the group with definite bacterial infection. Conclusion Bacterial infections continue to be a significant problem in children with sickle cell disease. Salmonella infection is a growing concern in this group of children. Further work is required to identify risk factors and predictors for bacterial infection, and ascertain optimal prevention and management strategies.

Complex interplays among host, pathogen, and environment determine the severity of infection ranging from harmless nasopharyngeal colonization to bacteremia, meningitis, sepsis, and lethal disease. The inflammatory response includes proinflammatory and anti-inflammatory responses in innate and adaptive immunity. The net proinflammatory state causes endothelial dysfunction and activation of the hemostatic response. Within the wide range of illness severity, deposition of fibrin throughout the microcirculation could result in multiple organ dysfunction, skin grafts/amputation, and death. Meningococci hold unique properties to promote adhesion, colonization, and invasion into the bloodstream.