Vaso-occlusive pain crisis

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Precipitating Factors

  • Stress
  • Cold weather
  • Dehydration
  • Hypoxia
  • Infection
  • Acidosis
  • Alcohol intoxication
  • Pregnancy
  • Exertional stress

Clinical Features[1]

  • Pain
    • Most common manifestation of SCA (79-91% of ED visits)
    • Lower back, long bones most commonly affected

Differential Diagnosis

Sickle cell crisis


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


  1. Analgesia
    • IV opiods prefered (morphine or hydromorphone)
      • Avoid IM route (use SQ if necessary)
      • Avoid meperidine
      • Use PCA pump if available
      • Redose in 30min if inadequate
      • Normally admit if needs more than three doses
    • Use of concurrent acetaminophen encouraged, unless contraindicated
  2. Hydration
    • Initial bolus of 0.5-1 L of normal saline
    • Caution if underlying renal and cardiac dysfunction
    • There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.[2]
  3. O2 if hypoxic; otherwise may inhibit erythopoesis


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


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


  1. Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
  2. 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