Peripartum considerations in sickle cell disease

Pregnancy exacerbates sickle cell disease (SCD) and is associated with increased frequency and severity of complications resulting in high levels of maternal and fetal morbidity and mortality. We review recent recommendations for managing SCD in pregnancy.

An updated pathobiological model of SCD now attributes the clinical picture to a vicious cycle of four major cellular disturbances. Management decisions should be guided by an understanding of this upgraded model. Red cell transfusions are a key therapeutic intervention used in managing several acute and chronic complications. Transfusion however has significant drawbacks. The American Society of Hematology recently published transfusion guidelines to support care providers.

Patients should be managed by a multidisciplinary and experienced team. The perioperative episode is a recognized period of disease exacerbation and informed anesthetic management can contribute to improved patient outcomes.