What is the appropriate management for two patients with sickle cell disease (SCD) presenting to the emergency room (ER) with fever?

Emergency Management of Fever in Patients with Sickle Cell Disease

Patients with sickle cell disease presenting to the emergency room with fever should receive immediate blood cultures followed by prompt administration of broad-spectrum antibiotics within 30-60 minutes of arrival, regardless of clinical appearance, due to their high risk of life-threatening infection. 1

Initial Assessment and Management

• Triage as high priority: Patients with sickle cell disease and fever should be triaged as urgent cases
• Immediate workup:
  • Blood cultures (before antibiotics)
  • Complete blood count with reticulocyte count
  • Basic metabolic panel
  • Chest X-ray if respiratory symptoms present 1

Antibiotic Management

• Initiate broad-spectrum antibiotics immediately after blood cultures are obtained
  • Cover typical and atypical pathogens including encapsulated organisms
  • Appropriate options include ceftriaxone or similar third-generation cephalosporin 1, 2
• Do not delay antibiotic administration as this increases morbidity and mortality 1

Supportive Care

• Hydration: Administer IV crystalloid fluids to maintain adequate hydration 1
• Oxygenation: Provide supplemental oxygen to maintain SpO2 >95% 1
• Temperature management: Avoid hypothermia as it can precipitate sickling 3
• Pain management: If pain is present alongside fever, provide appropriate analgesia using scheduled dosing or patient-controlled analgesia 1

Admission Criteria

While historically all febrile sickle cell patients were admitted, recent evidence suggests selective outpatient management may be appropriate for some patients:

• Criteria for admission:

  • Temperature ≥38.5°C
  • Significant respiratory symptoms or hypoxemia
  • Abnormal vital signs or evidence of sepsis
  • Neurological symptoms
  • Inability to follow up within 24 hours 4, 2

• Potential for outpatient management:

  • Well-appearing patients
  • No signs of sepsis
  • Reliable follow-up within 24 hours
  • Completed vaccination schedule and on antibiotic prophylaxis 5, 2

Special Considerations

• Changing epidemiology: The incidence of bacteremia in febrile children with SCD has decreased significantly (0.33% in recent studies) 5, likely due to pneumococcal vaccination and prophylactic antibiotics
• Emerging pathogens: There is a shift toward gram-negative organisms as causative agents in SCD infections 6
• Risk stratification: Consider using risk scores that incorporate clinical and laboratory parameters (CRP, IL-6, presence of hypoxemia) to guide management decisions 4

Monitoring and Follow-up

• For admitted patients:

  • Daily assessment by a hematologist (or pediatrician if a child) 3
  • Monitor for signs of acute chest syndrome (decreasing hemoglobin, worsening hypoxemia)
  • Inspect IV sites regularly for signs of phlebitis 3

• For outpatient management:

  • Ensure follow-up within 24 hours
  • Clear return precautions (worsening fever, respiratory symptoms, increased pain) 2

Pitfalls to Avoid

• Delaying antibiotics while waiting for laboratory results or imaging
• Underestimating fever in SCD patients (a spike in temperature may be an early sign of sickling) 3
• Inadequate hydration which can precipitate sickling
• Overlooking subtle signs of serious complications like acute chest syndrome
• Failing to consider both typical encapsulated organisms and emerging gram-negative pathogens 6

The approach to fever in SCD has evolved with improved preventive measures, but the potential for serious infection remains. While some centers are exploring risk-stratified approaches to reduce unnecessary admissions and antibiotic exposure 4, 2, the safest approach remains prompt antibiotic administration and close monitoring.