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Sickle Cell Patient with Fever

Pathway Background and Objectives

Patients with sickle cell disease (SCD) are at increased risk of severe bacterial infections, in large part due to reduced or absent splenic function. Repeated episodes of sickling and infarction lead to ‘autosplenectomy,’ often by age three years. Functionally asplenic patients with SCD are particularly prone to infection with encapsulated bacteria, most notably S. pneumoniae, as well as H. influenzae, N. meningitidis, and salmonellae. A fever may be the first subtle sign of a serious bacterial infection and must be treated as a potential medical emergency in all patient with SCD. Although vaccinations and antibiotic prophylaxis have made bacteremia increasingly rare, with recently reported rates of 0.8% or lower, the risk of overwhelming bacterial infection necessitates that all fevers be treated with empiric antibiotics while bacterial infection is ruled out. The specific objectives of this pathway are to:

  • Improve emergency department and outpatient triage and initial management of fever in patients with sickle cell disease through consistent application of current best practice
  • Decrease the time from initial presentation to first evaluation by a provider and administration of empiric antibiotic therapy
  • Decrease the duration patients receive intravenous opioids
  • Decrease the number of patients admitted to the hospital by appropriately discharging patients who can be managed outpatient with close follow-up


Download Fever in a Patient with Sickle Cell Disease Pathway Algorithm

Quality Metrics

  • Percentage of eligible patients treated per clinical pathway
  • Length of time from arrival to ED/clinic to administration of antibiotics OR length of time from first fever documented, while inpatient, to administration of antibiotics
  • Percentage of patients receiving correct antibiotic (choice of antibiotic and dose)
  • Length of stay in ED/clinic (minutes) and hospital (days)
  • Percentage of patients appropriately admitted to the hospital

Educational Module

Coming soon…

Key References

Pathway Contacts

  • Natalie Bezler, MD
  • Donna Boruchov, MD


The clinical pathways in the above links have been developed specifically for use at Connecticut Children’s and are made available publicly for informational and/or educational purposes only. The clinical pathways are not intended to be, nor are they, a substitute for individualized professional medical judgment, advice, diagnosis, or treatment. Although Connecticut Children’s makes all efforts to ensure the accuracy of the posted content, Connecticut Children’s makes no warranty of any kind as to the accuracy or completeness of the information or its fitness for use at any particular facility or in any individual case.

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