Hemoglobin

Fever in Sickle Cell Disease

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Fever in Sickle Cell Disease, Sepsis in Sickle Cell Anemia, Infectious Disease in Sickle Cell Disease

  • See Also
  1. Functional Asplenia
  2. Sickle Cell Disease
  3. Osteomyelitis in Sickle Cell Anemia
  4. Septic Arthritis in Sickle Cell Anemia
  • Pathophysiology
  1. See Sickle Cell Anemia
  2. Functional Asplenia
    1. Splenic ischemia and infarction occurs with sickling (age >3 months) and results in Asplenia
  3. Asplenia predisposes to overwhelming infection (esp. Encapsulated Bacteria)
    1. Higher risk for pneumonococcus, Haemophilus Influenzae, Salmonella typhi, Neisseria Meningitidis
    2. Higher risk for complicated Pneumonia, Urinary Tract Infection or Meningitis
  • Precautions
  1. Evaluate fever >101 F (38.3 C) carefully even without localizing signs or symptoms
  2. Unvaccinated children have a risk of serious Bacterial Infection in 10-15% (compared with 1-2% in vaccinated)
  • Labs
  1. Complete Blood Count (CBC)
  2. Reticulocyte Count
  3. Blood Cultures
  4. Urinalysis and Urine Culture
  5. Consider Lumbar Puncture (if meningeal signs)
  • Imaging
  1. Chest XRay
  • Differential Diagnosis
  1. See Sepsis
  2. Other Causes of Fever in Sickle Cell Disease
    1. Acute Chest Syndrome
    2. Severe Anemia
    3. Osteomyelitis in Sickle Cell Anemia
    4. Septic Arthritis in Sickle Cell Anemia
  • Management
  1. General
    1. Obtain labs, cultures, diagnostics and start empiric management (e.g. Ceftriaxone)
    2. Even when viral source is suspected, consider empiric Antibiotics until follow-up
  2. Admission criteria for Sickle Cell Anemia Patients (HgbSS or HgbSB0-Thal) with fever
    1. Ill appearance
    2. Fever >104 F (40 C)
    3. White Blood Cell Count >30k or <5k
    4. Central Line in place
    5. History of prior bacteremia
    6. Hemoglobin <5 g/dl OR >2 g/dl below baseline in HgbSS or HgbSB0-thal
    7. Unreliable follow-up
  3. Disposition when admission criteria are not met
    1. Consider admitting even well appearing sickle cell children under age 2 years (highest bacteremia rate)
    2. Well appearing children over age 2 years with reassuring findings may be closely rechecked in <24 hours
  • Prevention
  1. See Sickle Cell Disease for Prophylactic Antibiotic
  2. See Asplenia
  3. Prophylactic Antibiotics (typically with Penicillin V Suspension twice daily)
    1. Ages 2 months to 5 years old
    2. Extend course if complications (until Pneumococcal Vaccine series completion)
      1. Splenectomy
      2. Invasive pneumococcal infection
  4. Immunizations (In addition to standard CDC Primary Series)
    1. Pneumococcal Conjugate Vaccine
    2. Haemophilus influenzae B Vaccine
    3. Hepatitis B Vaccine
    4. Influenza Vaccine annually (6 months or older)
    5. Meningococcal Vaccine
  • References
  1. Lopez, Kleinmann, Chandra and Lopez (2025) Crit Dec Emerg Med 30(3): 4-12