Approach to patients with Sickle Cell Disease and Thalassemia in Obstetric Anaesthesia

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Approach to patients with Sickle Cell Disease and Thalassemia in Obstetric Anaesthesia – ATOTW 554

18 August 2026

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1) Haemoglobinopathies are most common in populations with ancestral origins in Africa, Asia, and the Middle East.

True False

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Haemoglobinopathies occur most commonly in populations whose ancestral origins come from Africa, Asia, and the Middle East.

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2) Sickle cell disease is caused by a mutation in the beta-globin gene, producing abnormal haemoglobin S (HbS).

True False

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Sickle cell disease (SCD) is an inherited blood disorder caused by a mutation in the beta-globin gene. This mutation produces haemoglobin S (HbS), an abnormal form of haemoglobin that is prone to distorting its shape when stressed by low oxygen concentrations.

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3) The pain from a Sickle Cell Crisis is very different from labour pain.

True False

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The crises are intensely painful to the patient. In parturients, these may mimic the pain of labor.

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4) There are only two types of beta-thalassaemia: major and minor.

True False

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Beta-thalassaemia can be classified into minor (carrier), intermedia, and major.

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5) All parturients with sickle cell disease should be screened antenatally for pulmonary hypertension with a right heart catheterization.

True False

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Parturients with SCD should have a complete cardiovascular and respiratory assessment. They should be screened for pulmonary hypertension with echocardiography and electrocardiography (ECG).

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6) All parturients with thalassaemia should have a cardiac MRI carried out antenatally.

True False

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Patients with haemoglobinopathies should also have a T2-weighted cardiac MRI to assess for iron-related cardiomyopathy secondary to repeated blood transfusions.

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7) Patients with thalassaemia can have rare red cell antibodies, making transfusion difficult.

True False

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Patients who receive recurrent blood transfusions can develop multiple red cell autoantibodies, making it difficult to obtain fully cross-matched blood.

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8) Patients with haemoglobinopathies are at a reduced risk of venous thromboembolism.

True False

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Patients with haemoglobinopathies are at a significantly increased risk of venous thromboembolism due to platelet dysfunction and sequestered red blood cells.

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9) All parturients with sickle cell disease should be delivered by Caesarean section.

True False

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Sickle cell disease is not an indication for Caesarean section. These parturients can deliver via vaginal delivery provided there are no other contraindications.

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10) Haemoglobinopathies are an absolute contraindication to neuraxial anaesthesia.

True False

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Haemoglobinopathies are not a contraindication to regional anaesthesia. Patients are encouraged to receive an early epidural in labour provided there are no other contraindications. Regional anaesthesia is preferred to general anaesthesia in caesarean section.

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11) Parturients with sickle cell disease are at high risk of developing a sickle cell crisis in labour.

True False

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Parturients are at high risk of developing a sickle cell crisis in labour because of increased pain and increased catecholamine surge. Exposure can reduce the patient’s temperature, further predisposing a patient to a sickle cell crisis.

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12) Desferrioxamine can be used throughout labour for parturients with thalassaemia.

True False

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Desferrioxamine, an iron chelating agent, can be used throughout labour and in the early postpartum period. It is secreted in breast milk but not teratogenic to the foetus as it is not absorbed from the GI tract.

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13) All uterotonics can be used without concern in patients with haemoglobinopathies.

True False

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Not all uterotonics can be used. Caution needs to be exercised with ergometrine and prostaglandins as many patients with haemoglobinopathies can have co-existing cardiac disease and/or pulmonary hypertension.

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14) General anaesthesia is preferable to neuraxial anaesthesia for Caesarean section in patients with haemoglobinopathies.

True False

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Provided there are no contraindications, regional anaesthesia is preferred to general anaesthesia in patients with haemoglobinopathies. Maternal morbidity is higher with general anaesthesia.

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15) Patients with haemoglobinopathies are pre-disposed to infections.

True False

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Patients with haemoglobinopathies are predisposed to infections as they may have bone marrow suppression or may have had a splenectomy.

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16) Cell salvage is relatively contraindicated in patients with haemoglobinopathies.

True False

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It is theorised that the more fragile cells may sickle or haemolyse during the wash process resulting in haemoglobin precipitation. This may reduce the red cell efficacy after re-transfusion and potentially result in a sickle cell crisis.

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17) Senior haematology input is essential in major haemorrhage situations if a patient has a haemoglobinopathy.

True False

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Patients may have multiple red cell antibodies, making complete blood crossmatch impossible. Senior haematology input is required to guide which blood can be administered in these situations.

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18) Parturients with haemoglobinopathies should be monitored postnatally in the high dependency unit.

True False

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Parturients with haemoglobinopathies are pre-disposed to many complications in the early postpartum period such as haemorrhage, VTE, and sickle cell crisis. They thus must be monitored closely in a high-dependency environment.

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19) Patients with sickle cell disease cannot have opioids post-operatively.

True False

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Patients with sickle cell disease must receive significant analgesia in the postoperative period to prevent a sickle cell crisis. The only opioid that is contraindicated is pethidine, which can provoke seizures in those with sickle cell disease.

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20) Patients with haemoglobinopathies should not receive prophylactic anticoagulation in the postpartum period.

True False

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Patients with haemoglobinopathies are at an increased risk of VTE and as such receive prophylactic LMWH throughout their inpatient stay and up to 6 weeks postpartum, provided there is no separate contraindication to anticoagulation.

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