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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: new infiltrate on CXR + another clinical symptom (fever, chest pain, hypoxia). Consult Benign Hematology immediately. Do not wait until the next day.
    • PE (ACS less likely in these pts), avascular necrosis of hip, priapism, stroke

Evaluation

  • Labs: ↑ LDH, Hgb/Hct (low; check versus baseline), retic, smear, WBC
  • If febrile: UA + blood cultures
  • Hgb S level only in certain circumstances (e.g. guides treatment in acute chest)
  • Imaging: CXR, MRI for hip pain, abdominal U/S or CT abdomen
  • Maintain active type and cross given probability of alloimmunization

Management

  • General
    • Look for a care coordination yellow note in the Summary Tab
      • Heme clinic will have specific management preferences for individual pts
    • 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 QD
    • 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, often initiation of PCA
    • All SS pts should have pain plans. Inpt pain plans are in the problem list under sickle cell disease or in the care coordination section of Epic
    • Outpt 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
    • Obtain HbS level
    • Avoid dehydration. Consider LR @ 125-200 cc/hr but caution with overhydration (may worsen pulmonary edema)
    • Incentive spirometry: atelectasis and hypoxia drive V/Q mismatching and further sickling
    • Transfuse hgb to goal of 10 (do not exceed to avoid hyperviscosity)
    • Pain control per pain plan
    • Consider Abx for CAP (vs HAP if risk factors)
    • Plasma exchange for moderate-severe cases (driven by hematology)