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HbSS sickle cell disease: elective laparoscopic cholecystectomy24-year-old man, 66 kg, for elective laparoscopic cholecystectomy for gallstones

Sickle cell disease: preoperative transfusion, avoiding triggers, acute crisis

From memory: the diagnosis and the notes are hidden. Answer each question aloud, then press Show.

Know this first

  • Before general anaesthesia, transfuse to Hb 10 g/dL; on hydroxyurea, a sickle expert picks the method.
  • Transfused red cells are C, E and K antigen-matched.
  • Sickling is triggered by cold, dehydration, acidosis, hypercarbia and hypoxia.
  • Watch for acute chest syndrome 2–3 days after surgery; incentive spirometry helps prevent it.

The patient

24-year-old man, 66 kg, for elective laparoscopic cholecystectomy for gallstones. HbSS sickle cell disease: four painful crises a year, one past acute chest syndrome, many transfusions, one red-cell antibody. Drugs: hydroxycarbamide, folic acid. Vitals: BP 118/70, HR 86, SpO2 96% on air, RR 14. Hb 8.2 g/dL.

The examiner asks

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  1. Consider

    What are your concerns?

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    • A 24-year-old with HbSS disease, frequent crises and past acute chest syndrome, for elective laparoscopic cholecystectomy. Patient
    • His gallstones are pigment stones from chronic haemolysis; abdominal surgery is intermediate sickling risk. Surgery
    • Frequent severe crises and past acute chest syndrome raise his perioperative risk. Disease
    • Many transfusions and an antibody mean finding compatible blood may take time.
    • Pneumoperitoneum adds CO2, and hypercarbia and acidosis promote sickling. Surgery

  2. Prepare

    How will you prepare him?

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    • A joint plan with his haematologist, timed away from any crisis. Disease
    • Transfuse to Hb 10 g/dL; he takes hydroxycarbamide, so the haematologist decides simple or exchange.
    • Red cells matched for C, E and K, with his antibody declared to the blood bank early.
    • Hydroxycarbamide and folic acid continue.
    • IV fluids from the start of his fast, so he is never dry. Stem trigger

  3. Plan

    How will you anaesthetise him?

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    • I keep him warm, well filled, well oxygenated, normocarbic and free of acidosis. Stem trigger
    • Oxygen to SpO2 100%, PaO2 at least 90 mmHg. Disease
    • Minute ventilation raised during insufflation to keep his CO2 normal. Surgery
    • Forced-air and fluid warmers, and a warm theatre. Stem trigger
    • Multimodal analgesia: paracetamol, port-site local anaesthetic, titrated opioid. After surgery
    • Afterwards, incentive spirometry, early mobilisation, Hb and renal checks, and thromboprophylaxis.

  4. Crisis

    During a unit of red cells he becomes febrile, BP falls to 78/42, HR 128, and his urine turns dark. What do you do?

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    • Acute haemolytic transfusion reaction until proven otherwise. (Crisis)
    • Stop the transfusion; recheck his identity against the unit's labels.
    • Bag back to the blood bank, with new crossmatch and direct antiglobulin samples.
    • Haemolysis screen: haptoglobin, free haemoglobin, bilirubin and clotting.
    • Fluids and vasopressor for his pressure, aiming for more than 1 mL/kg/h of urine.
    • Watch for DIC, and give components if it appears.

  5. Crisis

    In recovery he reports severe pain in his back and legs, 9 out of 10. SpO2 95%, temperature 37.9°C. What now?

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    • A vaso-occlusive crisis, while I exclude a surgical cause for his pain. Crisis
    • Parenteral opioid within 30 minutes, titrated and reassessed often.
    • Opioid tolerance is common in sickle cell disease, so he may need more.
    • Oxygen, warmth and IV fluids, avoiding overload.
    • Watch for acute chest syndrome, which may appear 2–3 days after surgery. After surgery
    • Incentive spirometry while awake and early walking.

Fatal mistakes

  • Continuing the transfusion during a haemolytic reaction.
  • Under-treating his crisis pain.
  • Letting him get cold, dry, hypoxic or hypercarbic.

Checked against: Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014 · Faust · Miller 10e

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