Sickle cell disease

Sickle cell disease (SCD) refers to a group of autosomal recessive disorders involving abnormal hemoglobin. Crisis is associated with increased maternal and perinatal morbidity and mortality. Caution must be used when interpreting theresults ofthesetestsduring anacute painepisode, because they may not be predictive of increased perinatal morbidity and mortality in the absence of other findings. Controversy exists regarding the role of prophylactic blood transfusion in the management of SCD in pregnancy. The exact mechanism of hemolytic transfusion reaction is not well understood, and the pathophysiology seems to be far more complex because it involves the destruction of both patient and donor RBCs. The same pitfalls of management applied during the antepartum period are also prudent after birth. Overall, pregnant women with SCD appear to be more likely to experience antepartum, intrapartum, and postpartum complications when compared with unaffected women. Access to high-risk obstetric care, patient education, and close follow-up are important to minimize maternal morbidity and mortality.