Managing pregnancy in patients with sickle cell disease from a transfusion perspective

Advances in the management of sickle cell disease (SCD) have made it possible for most female patients (whether homozygous or compound heterozygous) to reach childbearing age and become pregnant. However, even in the less symptomatic forms of SCD a high risk of complications during pregnancy and the postpartum period can occur for both the mother (1% to 2% mortality) and the fetus. Coordinated care from the obstetrician and the sickle cell disease expert is essential, together with the active participation of the patient. Vaso-occlusive complications, such as vaso-occlusive crisis and acute chest syndrome, often increase in frequency when hydroxyurea treatment is interrupted. Obstetric complications, such as pre-eclampsia, fetal growth restriction, and preterm delivery, are more common in women with SCD. Recent meta-analysis-based studies support prophylactic transfusion. However, there have been no randomized trials assessing the benefits of prophylactic transfusion. Given the known risk of transfusion complications, including delayed hemolytic transfusion reaction and hyperhemolysis, transfusion is not systematically performed in pregnant women with SCD. We describe here a case-by-case approach to the management of pregnancy in women with SCD based on the medical and transfusion history of each patient.