Sickle Cell Disease and Pregnancy

Abstract

Sickle cell disease is caused by a mutation in the beta-globin gene, leading to the formation of abnormal haemoglobin and various complications. Managing this condition during pregnancy requires a collaborative approach, involving close follow-up, patient education, and active patient involvement. The process begins with pre-conception genetic counselling
and assessing the patient’s baseline health. If the disease is uncontrolled or if the patient has kidney disease, hypertension, or frequent vaso-occlusive crises, pregnancy may need to be delayed or alternative reproductive options considered. The use of hydroxyurea during pregnancy is a subject of debate and is determined on a case-by-case basis. High-dose folic
acid supplementation is recommended before and during pregnancy. Medications should be carefully reviewed, and any potentially harmful drugs to the fetus should be discontinued. Low-dose aspirin and adequate hydration are used to prevent hypertensive and vaso-occlusive crises, respectively. Severe anaemia may require a blood transfusion, but haemoglobin
levels are maintained below 10g/dl to prevent increased blood viscosity. Vaginal delivery can be safely performed with appropriate measures such as hydration, warmth, and nasal oxygen. Cesarean sections are reserved for obstetric indications.