Vaso-occlusive pain crisis

Revision as of 17:20, 15 August 2019 by Gregtong (talk | contribs) (Management)


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
    • Controversial to bolus IVF, rehydration can lead to atelectasis, pulmonary edema, and acute chest syndrome. Normal saline may also promote hyperchloremic metabolic acidosis, further precipitating sickling [2] Recent studies in children has shown potential worse pain control and increased admission associated with normal saline boluses [3] [4]
    • oral rehydration, consider D5 1/2 NS at maintenance rate
    • There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.[5]
  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. Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20
  3. 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)
  4. 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.
  5. 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