Vaso-occlusive pain crisis

Background

  • Common complication of sickle cell anemia, constituting 79-91% of ED visits[1]
  • Sickled red blood cells obstruct vasculature, causing ischemic injury and pain

Precipitating Factors

Clinical Features[2]

  • Pain
    • Lower back, long bones most commonly affected

Differential Diagnosis

Sickle cell crisis

Evaluation

  • CBC
    • Because anemia can precipitate a crisis, must check for acute hemoglobin drop
  • Retic count (if aplastic crisis considered - rare in adults)
    • Should be >0.5%
  • Consider:
    • Chemistry / LFTs / lipase
      • If abdominal pain, may be cholecystitis, mesenteric ischemia, or perforation
    • T&S/T&C
    • Urine pregnancy
    • CXR
      • If cough, shortness of breath, or febrile

Management

  • Analgesia
  • Hydration
    • Controversial
      • Dehydration promotes sickling
      • IVF bolus may lead to atelectasis, pulmonary edema, and acute chest syndrome. Hyperchloremic metabolic acidosis due to normal saline may further precipitate sickling. [5] [6] [7]
      • There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.[8]
  • O2 if hypoxia; otherwise may inhibit erythropoiesis

Anemia

Pediatric Pain Crisis

Ideally should have institutional protocols to initiate pain management at triage

  • Consider early intranasal fentanyl even prior to establishing IV
    • Dosage 2 mcg/kg intraNASAL
  • IV pain medication options
    • Morphine 0.1 mg/kg
    • Hydromorphone 0.02 mg/kg
    • Toradol 0.5 mg/kg
  • PO options
    • Oxycodone 0.1 mg/kg
    • Morphine 0.5 mg/kg
    • Hydromorphone 0.05 mg/kg
    • Ibuprofen 10 mg/kg


Repeat pain medications q20 minutes at 50% of initial loading dose until adequate pain control achieved

Disposition

Consider admission

Consider discharge

  • Pain is under control and patient can take oral fluids and medications
  • Ensure appropriate oral analgesics are available
  • Provide home care instructions
  • Ensure resource for follow-up

See Also

External Links

References

  1. ↑ Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
  2. ↑ Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
  3. ↑ *Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics
  4. ↑ *Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics
  5. ↑ Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20
  6. ↑ Carden, M.A., et al., Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso-occlusive pain: A single institution experience.Pediatr Blood Cancer, 2018. 65(1)
  7. ↑ Carden, M.A., et al., Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.Am J Hematol, 2019.
  8. ↑ Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database of Systematic Reviews 2015, Issue 3. Art. No.: CD005406. DOI: 10.1002/14651858.CD005406.pub4
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