What is the current risk of bacteremia in children and young adults with sickle cell disease?


What is the current risk of bacteremia in children and young adults with sickle cell disease?

The Study

In June 2023, the Journal of the American Medical Association (JAMA) Network Open published a study that answered this question.

Prior to the development of the pneumococcal vaccine and hydroxyurea treatment children and young adults diagnosed with sickle cell disease (SCD) had a mortality rate of 15-20% due to bacteremia. As a result, current guidelines suggest aggressive fever treatment, including laboratory tests, cultures, and intravenous antibiotics.

The study was a retrospective analysis of the Pediatric Health Information System database. Examining 35,548 emergency department visits from 11,181 patients with sickle cell disease (not trait) across 36 children’s hospitals in the United States.

The study covered data from January 2016 to December 2021.


  • Under age of 22
  • Both children and adults
  • Median age 6 years
  • 53% were male
  • 47% were female


  • Returns to the ER within 3 days
  • Transfers from another hospital
  • History of bone marrow transplants

What did they find?

Primary Outcome: Diagnosis of Bacteremia

Secondary Outcomes:

  • Resources at initial visit (Lab, Imaging, Admission)
  • Additional diagnoses (acute chest syndrome, septic joint, meningitis, osteomyelitis, and UTI)
  • Length of stay
  • ICU admission


  • Risk of bacteremia was 1.1%. This is higher than the reported risk of bacteremia in febrile children age 3-36 months without sickle cell disease who present to the ED, 0.42%.
  • History of bacteremia, central line associated bacterial infection (CLABI), or apheresis had higher odds of a new diagnosis of bacteremia.
  • Sickle cell disease genotype, age, race and ethnicity, sex, and insurance type were not associated with a greater risk of bacteremia in the ED
  • 127 of 405 children with bacteremia were discharged from the ED and subsequently hospitalized with bacteremia. Only 1 of them required the ICU, and none of them died.


“In this cohort study of children and young adults with SCD presenting to the ED with fever or a workup consistent with fever, bacteremia was an uncommon diagnosis. A history of bacteremia, CLABSI, or apheresis appeared to increase the odds of bacteremia. Prospective studies on children and young adults with SCD presenting with fever are needed to develop decision models and risk stratification tools to refine our approach and avoid unnecessary antibiotic exposure and hospitalization in this population.”

Read the original article:

Rineer S, Walsh PS, Smart LR, Harun N, Schnadower D, Lipshaw MJ. Risk of Bacteremia in Febrile Children and Young Adults With Sickle Cell Disease in a Multicenter Emergency Department Cohort. JAMA Netw Open. 2023 Jun 1;6(6):e2318904. doi: 10.1001/jamanetworkopen.2023.18904. PMID: 37338904; PMCID: PMC10282882.

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