Sickle Cell Disease in Pregnancy

Pregnancy in women with SCD is considered high risk due to increased maternal and fetal complications, including vaso-occlusive crises, acute chest syndrome, severe anemia, pre-eclampsia, venous thromboembolism (VTE), fetal growth restriction, preterm birth, and perinatal mortality. Management requires close multidisciplinary care involving obstetricians, hematologists, anesthetists, and pediatricians.

Preconception Care

• Discontinue hydroxyurea at least 3 months before conception.

• Counsel the patient regarding maternal and fetal risks associated with pregnancy in SCD.

• Determine the haemoglobinopathy status of the partner and offer genetic counselling where appropriate.

• Document pre-pregnancy baseline investigations, including haemoglobin level, renal function, liver function, blood pressure, and previous complications.

• Optimize nutritional status and ensure routine vaccinations are up to date.

Antenatal Care

• Refer all pregnant women with SCD to a high-risk antenatal clinic for multidisciplinary follow-up.

• Prescribe routine prenatal vitamins according to obstetric guidelines.

• Monitor maternal haemoglobin, blood pressure, renal function, fetal growth, and signs of vaso-occlusive crisis throughout pregnancy.

• Maintain adequate hydration, oxygenation, and prompt treatment of infections.

• Routine prophylactic blood transfusion is not recommended, but transfusion should be considered when clinically indicated.

Pharmacological Treatment

• Acetylsalicylic acid (PO) 75 mg once daily, beginning from the second trimester, to reduce the risk of pre-eclampsia.

• Prescribe venous thromboembolism (VTE) prophylaxis in women with additional VTE risk factors (e.g., previous VTE), according to the coagulation disorders guideline.

Intrapartum Care

• Vaginal delivery is preferred unless there is an obstetric indication for Caesarean section.

• Maintain adequate hydration, oxygenation, normothermia, and effective analgesia during labour.

• Avoid hypoxia, dehydration, acidosis, and hypothermia, as these may precipitate vaso-occlusive crises.

Postpartum Care

• Continue adequate hydration and pain management.

• Encourage early mobilization and continue VTE prophylaxis where indicated.

• Assess and manage neonatal opioid dependence and withdrawal in infants with a history of in utero opioid exposure.

• Hydroxyurea should remain discontinued during breastfeeding.