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

1. History:

• Nature, location, duration, and severity of pain
• Character of pain similar to previous sickle pain?
• Analgesics already used for this episode
• Associated symptoms - especially fever or evidence of dehydration
• Consider etiologies other than sickling (e.g. cholecystitis, appendicitis, trauma)
• Previous experience with analgesics (efficacy and side effects). What does patient/family feel best alleviates pain?

2. Physical Exam: Complete with emphasis on:

• vital signs
• hydration status
• degree of pallor
• evidence of infection
• cardiopulmonary status
• spleen size (compare with baseline exam)
• penis (priapism)
• neurologic

3. Lab:

• CBC, diff, platelet, and reticulocyte count (compare with patient's baseline values)
• Blood cultures if febrile (see fever care path, p 16)
• Type and crossmatch if extreme pallor, respiratory or neurologic symptoms, or acute splenic enlargement present. Consider requesting, if available, minor-antigen-matched, sickle-negative, and leukocyte-depleted RBC.
• CXR and pulse ox (or blood gas) if:

• Fever
• chest pain
• tachypnea
• respiratory symptoms

• Consider abdominal ultrasound and liver function tests for RUQ, epigastric pain (R/O choletithiasis/cholecystitis).

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

5. Treatment (discuss with patient, family, and hematologist or primary physician on-call)

a. Mild to moderate pain:

• Acetaminophen with codeine 1 mg/kg po (and then q 4 hr) and oral fluids
• If inadequate relief within 30 min, follow b, below
• Consider starting ibuprofen 10 mg/kg po q 6-8 h or other anti-inflammatory agent if no contraindication present (i.e. gastritis, ulcer, coagulopathy, or renal impairment). Limit more frequent dosing to 72 hr maximum duration.
• If adequate relief and no other acute complications present, discharge on oral analgesics (acetaminophen with codeine and/or ibuprofen).

b. Moderate to severe pain:

• Morphine 0.1-0.15mg/kg IV. Reassess pain q 15-30 min. Patients with severe pain may require repeated doses of morphine 0.02-0.05 mg/kg IV q 15-30 min to achieve pain relief. Alternative analgesics, such as hydromorphone (Dilaudid) 0.015-0.02 mg/kg IV, may be appropriate in individual cases. Ketorolac (Toradol) 0.5 mg/kg (30 mg maximum dose) IV may be used in addition to opioid analgesia if no contraindication (i.e., gastritis, ulcer, coagulopathy, dehydration or renal impairment). Do not use ibuprofen with ketorolac. Repeated doses of meperidine (Demerol) should be avoided because of the risk of seizures.
• IV fluids: 10 cc/kg bolus over 1 hr then maintenance rate. Excessive fluids should be avoided unless patient is judged dehydrated.
• Monitor pulse ox. Use 0₂ by nasal cannula or face mask if needed to keep 0₂ saturation ³ 92% or ³ patient's baseline value, if >92%. The etiology of a supplemental 0₂ requirement should be investigated.
• If adequate pain relief with one or two doses of morphine, consider giving acetaminophen with codeine (1 mg/kg) as outpatient therapy.
• Consider hospitalization for around-the-clock parenteral analgesics if more than one or two doses of morphine required.

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References

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Last modified: November 01, 2002