Vaso-occlusive pain crisis


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
    • Because anemia can precipitate a crisis, must check for acute hemoglobin drop

Differential Diagnosis

Sickle cell crisis


  • CBC
    • Assess for significant anemia
  • 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%
  • O2
    • If hypoxic; otherwise may inhibit erythopoesis
  • T&S/T&C
  • Urine pregnancy
  • CXR
    • If cough, shortness of breath, or febrile


  • Analgesia
    • IV opiods prefered (Morphine or hydormorphone)
      • 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
  • Hydration
    • Initial bolus should be 1/2NS with goal of increasing MCV of sickled cells[2][3]
    • 1.5 times maintenance with D5 1/2NS
    • Normal Saline boluses for hypovolemic shock
  • O2 is not useful in the nonhypoxic patient


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


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. Guy, R. Treatment of Sickle Cell Crisis with Hypotonic Saline Clinical Research 1971; 19: 420
  3. Pathogenesis and Treatment of Sickle Cell Disease H. Franklin Bunn, M.D. N Engl J Med 1997; 337:762-769