Skip to content

Sickle Cell Crisis

Michael J. Neuss

Background

  • Present with severe pain in bone, joints, chest, abdomen
  • Causes: (HIDISC) Hypoxia, Ischemia, Dehydration, Infection, Stress, Cold
  • Can’t Miss:
    • Acute chest: hypoxia + fever + chest pain + new infiltrate on CXR (consult benign hematology immediately, do not wait until the next day)
    • PE (ACS less likely in these patients); avascular necrosis of hip, priapism, stroke

Evaluation

  • Labs: LDH (high), Hgb/Hct (low; check vs baseline), retic, smear, WBC
  • If febrile: UA + Blood cultures
  • Send Hgb S level, and compare to baseline w/ other hospital admissions
  • Imaging: CXR, MRI for hip pain, Abdominal U/S or CT abdomen
  • Maintain active type and cross given probability of antibodies

Management

  • General:
    • Look for a care coordination yellow note in the Summary Tab
      • Heme clinic will have specific management preferences for individual patients
    • Maintain hydration, IVF at 150-200 cc/hr (if no contraindication)
    • Oxygen: goal sat ~95% (higher O2 goal will help to prevent further sickling!)
    • Continue folic acid 1 mg qDay
    • Continue hydroxyurea if uncomplicated pain crisis
      • Hold if counts suppressed or concern for infection
    • If in the MICU: consider discussion for plasma exchange (if Hgb SS or SC or S-Thal)
    • Transfuse: Simple transfusion if Hgb lower than baseline and/or complications
      • Avoid transfusions when able, given risk of antibody formation
  • Pain:
    • Will generally require opiates, likely initiation of PCA
    • All SS patients should have pain plans; inpatient pain plans are in the problem list under sickle cell disease or in the care coordination section of Epic
    • Outpatient plans (to which you will transition pts back prior to discharge) are not standardized in location, but can be under Media (with a pain contract) or found in notes
  • Acute chest:
    • Consult Hematology at time of admission
    • D5 ½ NS @ 150-250 cc/hr
    • Transfuse hgb to >10
    • PCA w/ dilaudid
    • Abx for CAP (vs HAP if risk factors) ± bronchodilators
Last update: 2022-06-25 02:05:01