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Sickle Cell Disease and Pregnancy
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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.
Keywords
Sickle Cell Disease, Pregnancy, Vaso-occlusive crises, Anemia, Hydroxyurea, Delivery.
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