Sickle cell disease in labour23-year-old woman, 58 kg, at 39 weeks with HbSS sickle cell disease, in early labour
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Know this first
- Sickling is provoked by hypoxia, dehydration, cold and acidosis.
- Prophylactic LMWH: wait 12 hours or more before any neuraxial needle.
- Acute chest syndrome: a new X-ray infiltrate plus fever, chest pain or breathing symptoms.
- ACS: oxygen, analgesia, fluids, antibiotics, incentive spirometry; exchange transfusion if severe.
- Alloimmunised patients take longer to crossmatch.
The patient
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Consider
What are the issues?
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- A 23-year-old with HbSS at term in labour, past acute chest syndrome and red cell antibodies. Patient
- At term she has the usual airway, aspiration and caval risks, and labour raises her oxygen use sharply.
- Hypoxia, dehydration, cold and acidosis trigger sickling, and labour can bring all four. Disease
- Her antibodies mean matched blood takes time to find.
Prepare
How do you prepare her?
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- Haematology involved now; a transfusion plan agreed before delivery. Patient
- Group and screen early so antibody-matched units are ready if she bleeds. Disease
- Enoxaparin 40 mg was 8 hours ago; no needle until 12 hours have passed.
- Continuous oximetry, oxygen if her saturation drops.
- IV fluids so she doesn't dehydrate, a warm room, and warmed fluids.
Plan
What is your plan for her labour?
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- Epidural as soon as the 12 hours are up: strong analgesia, and its vasodilation may reduce sickling. Disease
- Dilute bupivacaine with fentanyl; she may need more opioid, given her crises.
- If she needs a caesarean, I extend the epidural; any technique is acceptable in sickle cell disease.
- After birth, enoxaparin at least 12 hours after the needle; catheter out 12 hours after a dose. After surgery
Crisis
In active labour, 9 hours after enoxaparin, she has severe back and leg pain like her crises. SpO2 95%, HR 110, temperature 37.2 °C. What do you do?
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- Vaso-occlusive crisis in labour, and the neuraxial window is 3 hours away. (Crisis)
- Remifentanil PCA meanwhile, with one-to-one midwife care and continuous oximetry.
- Nitrous oxide 50% can add some relief.
- Oxygen, warmth and IV fluids to break the sickling cycle.
- Look for a trigger: infection, dehydration or hypoxia.
- Epidural placed as soon as 12 hours have passed.
Crisis
Day 1 after birth: temperature 38.6 °C, chest pain, SpO2 89% on air, a new infiltrate at the left base on X-ray. What do you do?
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- Acute chest syndrome: new infiltrate with fever and chest pain, not just pneumonia. Crisis
- Oxygen to normal saturation, good analgesia, careful fluids, antibiotics covering atypicals.
- Incentive spirometry and early mobilisation.
- Haematology now: simple transfusion if anaemic, exchange transfusion if severe or worsening.
- ICU if she tires; some patients need mechanical ventilation.
- Thromboprophylaxis continues: postpartum clot risk is very high. After surgery
Fatal mistakes
- Tolerating hypoxia, cold or dehydration.
- Neuraxial block inside the 12-hour LMWH window.
- Treating acute chest syndrome as simple pneumonia.
Checked against: Miller 10e · Regional anesthesia in the patient receiving antithrombotic or thrombolytic therapy: ASRA Pain Medicine evidence-based guidelines (fifth edition) (2025) · Barash 9e
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