Sickle Cell Disease with Fever (<18 years old)

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Sickle Cell Disease with Fever (<18 years old)
Inclusion Criteria
  • Children >2 months of age
  • HbSS, HbSC, or HbS-β-thalassemia type sickle cell disease
  • Fever >38.5°C
Exclusion Criteria
  • None

Complete Blood Count, Serum
Blood Culture (Aerobic and Anaerobic), Serum
Reticulocyte Count, Serum

Respiratory symptoms?

Urinalysis with micro, Urine
Urine Culture, Urine

Vancomycin: If the patient is ill appearing or there is concern for meningitis, add vancomycin 15 mg/kg intravenously (maximum dose 1 g).

X-ray Chest, 2 view

Concern for acute chest syndrome?

Reason for admission?

Localized or multifocal bone tenderness?

Evaluate for osteomyelitis.

Drop in Hgb of 2 g/dL or more below baseline?

Evaluate for associated conditions: acute chest syndrome, splenic sequestration, or aplastic crisis.

Ceftriaxone injection
Admit to Floor
Azithromycin tabs

Admit to Floor

History and physical exam including:

  • Vital signs including pulse oximetry
  • Signs of focal infection
  • Respiratory exam
  • Pain in long bones including sternum
  • Abdominal exam including splenic size
  • Neurologic exam to assess for stroke
  • Skin exam for rash or petechiae

Any of the following present:

  • Pale?
  • Tachycardic?
  • Splenomegaly?
  • Ill appearing?
  • Hgb < 5 gm/dL or Hgb drop > 2 gm/dL from baseline Hgb?

Type & Screen, Serum

<2-years-old OR symptoms of UTI

Administer within 60 minutes of triage.

Ceftriaxone injection

Discharge criteria

  • >12 months
  • Tolerating PO
  • Well-appearing
  • Reassuring vital signs including oxygen saturation
  • No central line access
  • No concern for ACS, sequestration, or pain crisis
  • WBC <30,000/microL and >5000/microL
  • Hgb has not dropped 2 g/dL or more below baseline
  • No history of bacteremia
  • No social/family concerns
  • Reliable follow up of blood culture in 24 hours
  • Parents comfortable and understand discharge plan
  • Provider comfortable with outpatient therapy

  • Obtain working phone number for patient or family, and place in chart
  • Schedule follow up for 24 hours, in person or via phone
  • Discharge home

Risk of Infection by Type of SCD:

  • HbSS: Most susceptible to serious bacterial infections.
  • HbSC: Less likely than patients with HbSS to develop serious bacterial infections because they do have some splenic function. Less likely to develop sepsis and septic shock, even when bacteremic.
  • HbS-β thalassemia: Patients with HbS-β0 thalassemia are functionally asplenic in early childhood and thus have a similar risk of infection as patients with HgSS. Patients with HbS-β+ thalassemia have variable production of HbA and thus variable outcomes, however, their risk of infection is most similar to HbSC.

Diagnosis of acute chest syndrome:

  • A new infiltrate on chest x-ray
  • AND one of the following:
  • Fever
  • Hypoxemia
  • Respiratory symptoms: shortness of breath, tachypnea, cough, rales, rhonchi, wheezes, or retractions
  • Chest wall pain

Admit if patient has one or more of the following:

  • <2 years old with HbSS or HbS-β0 thalassemia
  • Temperature of 40°C or greater
  • White blood cell count (WBC) >30,000/microL or <5000/microL
  • Hgb is 2 g/dL or more below baseline or hemoglobin level ≤5 g/dL
  • History of bacteremia
  • Central line
  • Toxic appearing or hemodynamic instability
  • Acute chest syndrome, pain crisis, aplastic crisis
  • Inability to be reached by phone or without means to return to hospital

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