Sickle cell disease (SCD) is a widespread genetic illness that puts patients at a heightened risk of severe bacterial infection due to weakened immune system function. On account of this predisposition, bacteremia, the intrusion of bacteria into the bloodstream, has been a historically common acute infection leading to death among many SCD patients, especially in the pediatric population. While strides in pneumococcal vaccines and targeted clinical practice guidelines for juvenile SCD patients have theoretically curbed bacteremia’s incidence and mortality, few studies have corroborated their impact.

One study demonstrating the efficacy of such changes was published in the June 2023 edition of the Journal of the American Medical Association (JAMA) by Stephen Rineer and colleagues, who conducted one of the largest analyses of bacteremia incidence among young SCD patients presenting with fever in US emergency departments (EDs). This study detected bacteremia in only 1.10% of clinical encounters with no recorded deaths. GlobalData epidemiologists forecast an increase from nearly 65,000 diagnosed prevalent cases of SCD in the population between 0 and 17 years old in 2023 to over 68,000 cases by 2030 in the US. Given the disease’s rising prevalence in this vulnerable population, Rineer and colleagues offer encouraging insight into the management of bacteremia’s incidence.

Rineer and colleagues observed the incidence of various bacterial infections in a multicenter retrospective cohort study between 2016 and 2021. The patient population, composed of 11,181 patients diagnosed with SCD, included children and young adults up to the age of 22 years old. Using the US-based Pediatric Health Information Systems database, which draws from approximately 50 tertiary care pediatric hospitals, the authors analysed 35,548 clinical encounters coded as ED visits for fever. Among them, 371 patients were diagnosed with bacteremia, representing only 1.10% of visits, compared to 4.60% from the last major study on its incidence in this clinical population. Furthermore, only one patient among them was admitted to the ICU and no deaths were recorded in this group. The authors attribute the declining incidence of bacteremia to improved prophylaxis in the past decades, namely through advancements in pediatric pneumococcal and meningococcal vaccines. Moreover, emergency departments have improved their interventions for febrile SCD patients through standardised care regimens. While the study’s indicators are encouraging signs of improvements in prevention and care over the past 30 years, Rineer and colleagues propose that experts research a clinical practice plan based on stratified risk categories for pediatric SCD cases, which may yield promising results for outpatient care.

As the largest recent study on the incidence of bacteremia among febrile SCD patients in an ED setting, Rineer and colleagues’ publication confirms the efficacy of prophylactic and clinical care standards for a population that is highly vulnerable to severe infections. Given the efficacy of these practices on a wide scale, it is critical to educate the parents of children with SCD on the importance of seeking care in case of fever, as well as vaccination. Furthermore, emphasising adherence to existing clinical care standards among ED staff can reduce the risk of severe illness and mortality among pediatric SCD patients.

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