Sickle Cell Crisis

Pt > 6 months old with h/o sickle cell anemia presents with severe acute-onset pain. Reports recent cold exposure, dehydration, alcohol consumption, and current menses. ROS positive for headache, chest pain, dyspnea, abdominal pain, flank pain, dysuria, change in urine color/appearance, hip pain, hand/foot pain. Tachycardia, tachypnea, SPO2 < 92%, jaundice, abdominal pain, hepatomegaly, costovertebral angle tenderness, facial droop, slurring of speech, unilateral weakness on exam.

  • Initial Labs

    • CBC, reticulocyte count, type/cross, CMP, U/A, EKG

    • Reproductive age female not currently menstruating: Urine beta-hCG

  • Start pain control within 30 minutes of initial evaluation and titrate until pain is controlled or patient displays somnolence

    • Starting dose for opioid tolerant adult: Oxycodone 4 mg q4 hours PRN

    • Home dose > starting dose (above): Start dose that appropriately controlled patient’s pain during most recent crisis

  • Potential Complications

    • Chest pain and/or dyspnea

      • DDX: Pulmonary embolism vs. acute coronary syndrome vs. acute chest syndrome (see below)

      • Overall increased index of suspicion if tachycardia, tachypnea, decreased SPO2, thrombocytopenia

      • Obtain troponin, EKG, CXR +/- DVT U/S, CT angiogram

    • Renal involvement

      • CVA tenderness → concern for pyelonephritis → renal ultrasound

      • U/A with leukocyte esterase +/- nitrites → concern for UTI

      • U/A with hematuria → concern for renal infarction

    • Jaundice

      • DDX: Hemolytic transfusion reaction vs. hepatobiliary disease (e.g. biliary complications including surgical abdomen)

      • Obtain PT/INR, LDH, RUQ U/S

    • Extremity pain

      • Fingers/toe pain: Dactylitis vs. gout → uric acid level, x-ray of affected extremity

      • Calf pain → concern for DVT → DVT U/S of affected extremity

      • Hip pain → concern for avascular necrosis → hip x-ray +/- MRI

    • Focal neurologic deficit: R/o stroke

  • Treatment

    • Oxygen by nasal cannula to maintain SPO2 > 92%

    • Adult patient: Heparin 5,000 units SQ q12 hours for VTE prophylaxis

    • Acute chest

      • Incentive spirometry at bedside

      • Administer D5 1/2 NS at 1.5 x maintenance for 24 to 48 hours

      • Start cefotaxime 1g IV q8h + azithromycin 500 mg PO qd x 7 days

      • Heparin for VTE prophylaxis regardless of age (reference dosing)

      • Initiate transfusion therapy per hematology/oncology

  • Consults

    • Notify hematology/oncology

      • Discuss potential need for exchange transfusion

      • Discuss starting hydroxyurea 15 mg/kg/day at discharge

    • Cholecystitis: Consult general surgery

    • Renal infarction: Consult nephrology

Notes

  • A sickle cell crisis is PAINFUL - adjust acute pain medication based on patient’s reported pain

  • Risk factors for sickle cell crisis include cold exposure, dehydration, alcohol consumption, and current menses

  • Increased suspicion for

    • Acute chest in patient with leukopenia or leukocytosis

    • PE in patients with h/o obesity, decreased mobility, splenectomy