Sickle Cell Crisis

Sickle Cell Crisis – Michael J. Neuss

Background

  • Present with severe pain in bone, joints, chest, abdomen
  • Causes: (HIDISC) Hypoxia, Ischemia, Dehydration, Infection, Stress, Cold
  • Cant Miss:
    • Acute chest: hypoxia + fever + chest pain + new infiltrate on CXR
    • 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 1/2 NS @ 150-250 cc/hr
  • Transfuse hgb to >10
  • PCA w/ dilaudid
  • Abx for CAP (vs HAP if risk factors) ± bronchodilators