Sickle cell disease and pregnancy

Sickle cell disease (SCD) encompasses a group of disorders characterized by inheritance of a Β globin gene mutation leading to the formation of sickle haemoglobin (HbS), which when deoxygenated forms a crystalline structure called a tactoid. SCD is characterized by high lifetime morbidity and mortality, as a result of the long-term consequences of vaso-occlusion, which are not always reversible. Women with SCD experience a higher rate of cesarean delivery, which is likely associated with increased rate of fetal compromise observed in this population. Acute chest syndrome (ACS) is a common and severe complication in SCD that can be seen in up to 20 percent of pregnancies. In pregnancy, pulmonary hypertension (PH) is associated with up to 36 percent risk of mortality with significant comorbidity for mother and fetus. Observational studies show increases in pregnancy-related complications such as preeclampsia, abruption, and intrauterine fetal death at the time of delivery in SCD patients.