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Treatment of acute pain in individuals with sickle cell disease presenting to the emergency department or day hospital

Treatment of acute pain in individuals with sickle cell disease presenting to the emergency department or day hospital
Triage rapidly (<15 minutes)
  • The individual has likely been having pain at home that has exceeded what they can manage with oral opioids
  • Follow individualized care plan if available
Assess pain clinically
  • Believe the individual's and/or family's self-report of pain
  • Rapidly assess pain location(s), quality, duration, severity
  • Assess potential comorbidities that cause pain (eg, avascular necrosis of a joint, compression fracture)
  • Assess potential comorbidities that require other treatments (eg, stroke, infection, acute chest syndrome, splenic sequestration)
Initiate analgesia as soon as possible (<30 minutes)
  • Most individuals will require opioids
  • Initial dose is based on intensity of pain and previous effective doses
  • If initial dose is unknown, use one of the following:
    • Intravenous morphine (0.1 to 0.15 mg/kg; maximum single dose 10 mg)
    • Intravenous hydromorphone (0.02 to 0.05 mg/kg; maximum single dose 1.5 mg)
    • Intranasal fentanyl (1.5 mcg/kg, give up to two doses, 5 to 10 minutes apart; maximum single dose 100 mcg)*
  • Rapidly reassess for efficacy and repeat dose if needed at appropriate interval (20 minutes for morphine, 30 minutes for hydromorphone)
  • Provide adjunctive therapies if appropriate (refer to the section below)
  • Avoid ineffective therapies (refer to the section below)
  • Admit to hospital if pain control is inadequate with three or more doses of an opioid analgesic (or two doses of intranasal fentanyl)
  • Plan for around-the-clock analgesia with additional doses for breakthrough pain; do not rely exclusively on as-needed (PRN) dosing
Adjunctive therapies
  • Adjunctive therapies are used in addition to (not instead of) analgesia
  • Ensure adequate hydration (encourage oral fluids, give intravenous fluids if hypovolemic)
  • Enlist family and other psychosocial supports
  • Use heat packs if helpful
  • Possible use of ketamine
  • Treat insomnia and other conditions that interfere with adequate sleep
Therapies to avoid or use sparingly
  • Never use placebo
  • Avoid ice and cold compresses (may precipitate sickling)
  • Avoid meperidine
  • Avoid ketorolac in adults; if ketorolac is given, no more than one dose should be used
  • Use anxiolytics only if indicated for anxiety, not for pain
  • Use NSAIDs cautiously as they may cause renal failure or bleeding and may be ineffective in many cases
  • Use oxygen only for hypoxia, not for routine pain
  • Use transfusion only for indicated complications, not for uncomplicated vaso-occlusive pain
Other therapies may be used if the individual is admitted to the hospital and after discharge (eg, relaxation/breathing exercises, hydroxyurea, L-glutamine, treatment for depression, treatments for opioid side effects). Special considerations may apply to individuals with renal or hepatic dysfunction and during pregnancy. Refer to UpToDate for details of pain management in SCD.

NSAID: nonsteroidal anti-inflammatory drug; SCD: sickle cell disease.

* Refer to the pediatric drug monograph of Lexicomp for additional details of intranasal fentanyl administration in children.
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