II. Pathophysiology
- See Sickle Cell Anemia
-
Functional Asplenia
- Splenic ischemia and infarction occurs with sickling (age >3 months) and results in Asplenia
-
Asplenia predisposes to overwhelming infection (esp. Encapsulated Bacteria)
- Higher risk for pneumonococcus, Haemophilus Influenzae, Salmonella typhi, Neisseria Meningitidis
- Higher risk for complicated Pneumonia, Urinary Tract Infection or Meningitis
III. Precautions
- Evaluate fever >101 F (38.3 C) carefully even without localizing signs or symptoms
- Unvaccinated children have a risk of serious Bacterial Infection in 10-15% (compared with 1-2% in vaccinated)
IV. Labs
- Complete Blood Count (CBC)
- Reticulocyte Count
- Blood Cultures
- Urinalysis and Urine Culture
- Consider Lumbar Puncture (if meningeal signs)
V. Imaging
VI. Differential Diagnosis
- See Sepsis
- Other Causes of Fever in Sickle Cell Disease
VII. Management
-
General
- Obtain labs, cultures, diagnostics and start empiric management (e.g. Ceftriaxone)
- Even when viral source is suspected, consider empiric Antibiotics until follow-up
- Admission criteria for Sickle Cell Anemia Patients (HgbSS or HgbSB0-Thal) with fever
- Ill appearance
- Fever >104 F (40 C)
- White Blood Cell Count >30k or <5k
- Central Line in place
- History of prior bacteremia
- Hemoglobin <5 g/dl OR >2 g/dl below baseline in HgbSS or HgbSB0-thal
- Unreliable follow-up
- Disposition when admission criteria are not met
- Consider admitting even well appearing sickle cell children under age 2 years (highest bacteremia rate)
- Well appearing children over age 2 years with reassuring findings may be closely rechecked in <24 hours
VIII. Prevention
- See Sickle Cell Disease for Prophylactic Antibiotic
- See Asplenia
- Prophylactic Antibiotics (typically with Penicillin V Suspension twice daily)
- Ages 2 months to 5 years old
- Extend course if complications (until Pneumococcal Vaccine series completion)
- Splenectomy
- Invasive pneumococcal infection
- Immunizations (In addition to standard CDC Primary Series)
IX. References
- Lopez, Kleinmann, Chandra and Lopez (2025) Crit Dec Emerg Med 30(3): 4-12
