Sickle Cell Vaso-Occlusive Crisis Management (ASH 2020)

Algorithm Steps

1. ▶

   Start

   Sickle Cell Patient with Pain

   Patient with known SCD presents with acute pain episode

   1. ●

      Action

      Immediate Triage (ESI-2)

      High acuity - target analgesic within 60 minutes

      • Assign ESI-2 triage level
      • Target door-to-analgesia <60 minutes
      • Obtain IV access
      • Check vital signs including SpO2
      1. ●

         Action

         Initial Assessment

         Evaluate for complications and pain severity

         • Pain assessment (0-10 scale)
         • Respiratory assessment (ACS screening)
         • Fever evaluation
         • Neurological assessment (stroke screening)
         • Hydration status
         1. ◆

            Decision

            Signs of ACS or Stroke?

            Screen for life-threatening complications

            • ACS: Chest pain, fever, hypoxia, new infiltrate
            • Stroke: Focal neuro deficits, altered mental status
            • Priapism >4 hours
            • Splenic sequestration (LUQ pain, falling Hgb)
            1. ⚠

               Warning

               Critical Complication

               Immediate specialist consultation

               • ACS: Transfusion, antibiotics, incentive spirometry
               • Stroke: Exchange transfusion, neurology consult
               • Priapism: Urology consult, aspiration
               • Sequestration: Transfusion, possible splenectomy
               1. ✓

                  Outcome

                  Admit for Ongoing Management

                  Inpatient pain management and monitoring

            2. ●

               Action

               Rapid Opioid Analgesia

               IV opioids within 60 minutes of arrival

               • Use patient's individualized pain protocol if available
               • Morphine 0.1-0.15 mg/kg IV or equivalent
               • Hydromorphone 0.015-0.02 mg/kg IV alternative
               • Reassess pain every 15-30 minutes
               • Re-dose opioid every 15-30 min until controlled
               1. ●

                  Action

                  Adjunct Therapies

                  Supportive care measures

                  • IV fluids: isotonic, avoid overhydration
                  • Supplemental O2 if SpO2 <95%
                  • Incentive spirometry every 2 hours while awake
                  • NSAIDs (ketorolac) if no contraindication
                  • Antiemetics as needed
                  1. ◆

                     Decision

                     Pain Controlled After 2-3 Doses?

                     Assess response to initial therapy

                     1. ●

                        Action

                        Escalate Therapy

                        Consider admission and PCA

                        • Admit to hospital
                        • Consider PCA (patient-controlled analgesia)
                        • Hematology consultation
                        • Consider transfusion if Hgb significantly below baseline
                        • Evaluate for underlying infection
                     2. ●

                        Action

                        Consider Discharge

                        If pain controlled and stable

                        • Transition to oral opioids
                        • Ensure adequate supply of pain medications
                        • Hydroxyurea adherence counseling
                        • Follow-up with hematology within 7 days
                        • Return precautions: fever, worsening pain, SOB
                        1. ✓

                           Outcome

                           Discharge Home

                           With oral analgesics and close follow-up