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
  • Transfusion PRBCs 10 mL/kg over 2hr period
  • Indications:
    • Aplastic crisis
    • Sequestration crisis
    • hemoglobin <6 with inappropriately low retic count

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

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
  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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