A 22-year-old man with sickle cell disease presents with acute severe chest pain, fever, tachypnoea, and new bilateral infiltrates on chest X-ray with SpO₂ 88% on room air. Which is the single most important immediate intervention for this acute chest syndrome episode?
- A Exchange transfusion to reduce HbS to < 30%
- B Hydroxyurea administration
- C Oxygen supplementation to maintain SpO₂ > 95% and simple blood transfusion ✓
- D IV ceftriaxone alone as infection is the precipitant
Correct answer: C. Oxygen supplementation to maintain SpO₂ > 95% and simple blood transfusion
Explanation
Acute chest syndrome (ACS) in sickle cell disease is managed with oxygen therapy (to maintain SpO₂ >95%), simple blood transfusion (if Hb declines or hypoxia worsens) to dilute HbS, empirical broad-spectrum antibiotics (covering atypicals), analgesia, and incentive spirometry. Simple transfusion to achieve Hb 10 g/dL is appropriate for mild-moderate ACS. Exchange transfusion (reducing HbS <30%) is reserved for severe/rapidly progressive ACS or neurological crisis. Hydroxyurea is for chronic prevention, not acute management.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.