Proactive management to improve outcomes of high-risk pregnancy in people with sickle cell disease

Pregnancy is high risk for individuals with sickle cell disease (SCD), and maternal morbidity and mortality are unchanged in middle- and high-income settings. As many as 30% of SCD pregnancies may be uncomplicated, but most are associated with complications. The odds of severe maternal morbidity are much higher in Black women with SCD than in Black women without SCD or non-Black women. Prophylactic red cell transfusions are considered low risk according to American Society of Hematology guidelines and are the only disease-modifying therapy available for use in pregnancy. When initiated in the preconception and/or prenatal period, treatment is associated with reduced maternal and fetal mortality, painful crises, and pulmonary complications in pregnancy. Furthermore, pain occurs in as many as 75% of SCD pregnancies; in meta-analyses and randomized controlled data, transfusions reduce pain events during SCD pregnancy. British, American, and French guidelines make recommendations regarding transfusion indications in SCD pregnancy. Many people with SCD, and most with hemoglobin SS or hemoglobin Sβ0-thalassemia, have an indication for prophylactic transfusion during pregnancy. Transfusion may meaningfully improve outcomes for pregnant people with SCD. We discuss our approach to managing SCD pregnancy using the case of a patient cared for in the mid-Atlantic region of the United States.