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Sickle Cell Disease Anaesthesia — FRCA Final MCQ

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ModerateGeneral AnaesthesiaSickle Cell Disease AnaesthesiaFRCA Final

An 18-year-old woman with sickle cell anaemia (HbSS) requires an emergency laparoscopic appendicectomy. She is not currently experiencing a vaso-occlusive crisis, and surgery must proceed after initial resuscitation without delay for planned preoperative transfusion. Which intraoperative strategy most directly reduces her risk of perioperative sickling?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: C — Avoid hypoxaemia, hypovolaemia, hypothermia and acidosis throughout

Explanation lettering: D = shown as A · E = shown as B · B = shown as C · A = shown as D · C = shown as E

The correct answer is B. Deoxygenation drives HbS polymerisation, while hypovolaemia, hypothermia and acidosis impair tissue perfusion and oxygen delivery, promoting sickling. Meticulous maintenance of oxygenation, euvolaemia, normothermia and acid–base status throughout anaesthesia is the fundamental measure, endorsed by the Association of Anaesthetists 2021 guideline, which stresses meticulous peri-operative care and avoidance of unnecessary transfusion. Exchange transfusion to HbS below 30% (A) is reserved for high-risk surgery or severe phenotypes and would inappropriately delay this emergency; the stem states transfusion cannot be arranged pre-induction. If preoperative transfusion is indicated, the usual target is about 100 g/L; transfusing to 120 g/L (D) risks hyperviscosity. Neuraxial anaesthesia alone (C) is unsuitable for laparoscopic surgery requiring pneumoperitoneum. Withholding opioids (E) is wrong: uncontrolled pain increases catecholamine release and hypoventilation risk; multimodal analgesia including opioids is standard.

Reference: Walker I, Trompeter S, Howard J, et al. Guideline on the peri-operative management of patients with sickle cell disease: Guideline from the Association of Anaesthetists. Anaesthesia. 2021;76(6):805-817. https://pubmed.ncbi.nlm.nih.gov/33533039/