A 30-year-old African man presents with painful priapism and right hypochondrial pain during a febrile illness. Peripheral smear shows sickle cells. Haematocrit is 22%. He is on hydroxyurea. Which is the MOST appropriate ACUTE management for his vaso-occlusive crisis?

• A Exchange transfusion immediately
• B Increase hydroxyurea dose urgently
• C Prophylactic antibiotics and G-CSF
• D IV fluids, adequate analgesia (opioids), oxygen if hypoxic, incentive spirometry ✓

Explanation

Acute vaso-occlusive crisis (VOC) in sickle cell disease is managed with IV hydration (to reduce sickling), aggressive multimodal analgesia (IV opioids are first-line for severe pain — NICE guidelines recommend PCA morphine), incentive spirometry to prevent ACS, and supplemental oxygen only if SpO2 <95%. Hydroxyurea works prophylactically (increases HbF) but dose escalation during an acute crisis is not appropriate. Exchange transfusion is reserved for stroke, ACS, multi-organ failure, or priapism lasting >4 hours unresponsive to conservative therapy. G-CSF is contraindicated in SCD (risk of splenic sequestration).

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.