DIAGNOSIS, GUIDELINES FOR COMPREHENSIVE CARE, AND CARE PATHS AND PROTOCOLS FOR MANAGEMENT OF ACUTE AND CHRONIC COMPLICATIONS  the Sickle Cell Disease Care Consortium (Arizona, Colorado, Georgia, Missouri, New Mexico, Tennessee, Texas, and Utah)

1. Rapid triage - immediately upon presentation. Place immediately into exam room.

2. Brief history and physical exam with emphasis on:

• vital signs
• degree of pallor
• evidence of systemic or localized infection
• cardiopulmonary status
• spleen size (compare with baseline exam)
• neurologic exam

3. Laboratory:

• Stat CBC, diff, platelet, reticulocyte count, and blood culture (use butterfly or angiocath and follow immediately with IV antibiotic).
• Type and crossmatch if extreme pallor, respiratory or neurologic symptoms, or acute splenic enlargement are present. Consider requesting, if available, minor-antigen-matched, sickle-negative, and leukocyte-depleted RBC.
• Urinalysis and urine, CSF, other cultures if clinically indicated.

4. Prompt administration of IV ceftriaxone (50-100 mg/kg, 2.0 grams maximum single dose) through butterfly or IV catheter used for phlebotomy. Relatively high doses (75-100 mg/kg) are sometimes recommended in regions with high prevalence of antibiotic resistent S. pneumoniae. For patients with known or suspected cephalosporin allergy, substitute clindamycin 10-15 mg/kg, 600 mg maximum single dose.

• Strongly consider adding vancomycin (10-15 mg/kg IV) for severe illness or if CNS infection is suspected.
• Parenteral antibiotics should be given before other procedures, such as CXR, etc.
• The presence of a focus of infection (e.g. otitis) does not alter the urgency of giving parenteral antibiotics.

5. Acetaminophen 15 mg/kg po (if not given in the last 4 hr) and/or ibuprofen 10 mg/kg po. Avoid ibuprofen if contraindication present (i.e. gastritis, ulcer disease, coagulopathy, or renal impairment).

6. Review summary of patient's last comprehensive evaluation or seek baseline information by phone.

7. Contact pediatric hematologist or patient's primary physician with expertise in sickle cell disease.

8. CXR and pulse ox (or blood gas), particularly if:

• toxic appearance
• any respiratory symptoms
• chest and/or abdominal pain

May use 0₂ by nasal cannula or face mask if signs of respiratory illness present. The etiology of a supplemental 0₂ requirement should be investigated.

9. Observation:

a) Admission should be strongly considered if one or more of the following criteria are present:

1. Age <1 yr with HbSS or Sb ^o-thalassemia

2. History of previous episodes of bacteremia or sepsis

3. T >40⁰C, WBC >30,000/mm³ or <5,000/mm³, and/or platelet count <100,000/mm³

4. Signs of systemic toxicity

5. Patient who received clindamycin or vancomycin

6. Evidence of other acute complications including severe pain, aplastic crisis, splenic sequestration, acute chest syndrome, stroke, or priapism (see other Clinical Care Paths).

7. Concerns about compliance / follow-up

b) Outpatient management for patients who are not admitted:

Observe with repeat vital signs and assessment ³ 2 hr post ceftriaxone. If non-toxic and clinically stable with reliable family and hematologist/PCP approval, discharge with a specific plan for outpatient follow-up. Minimum follow-up includes phone contact the next day. Repeat exam and 2nd dose of ceftriaxone (with or without repeat CBC and reticulocyte count) 24 hr later may be advisable in some cases.

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References

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Last modified: November 01, 2002