SMFM Consult Series #68 : Sickle Cell Disease in Pregnancy

This document has been endorsed by the American College of Obstetricians and Gynecologists.

Abstract : Pregnant individuals with sickle cell disease have an increased risk of maternal and perinatal morbidity and mortality. However, pre-pregnancy counseling and multidisciplinary care can lead to favorable maternal and neonatal outcomes. In this Consult, we summarize what is known about sickle cell disease and provide guidance for sickle cell disease management during pregnancy. The following are Society for Maternal-Fetal Medicine recommendations: (1) we recommend that patients with sickle cell disease be managed by a multidisciplinary team that includes maternal-fetal medicine, hematology (ideally a hematologist specializing in sickle cell disease), genetics, pain management, and social work or behavioral health (as appropriate) starting in early pregnancy (Best Practice); (2) we recommend that pregnant patients with sickle cell disease receive all routinely recommended antenatal vaccinations in addition to meningococcal and pneumococcal vaccination if due (GRADE 1A); (3) we recommend prenatal vitamins without iron unless iron deficiency is confirmed and initiation of 4 mg of folic acid for pregnant patients with sickle cell disease (GRADE 1B); (4) we recommend fetal growth surveillance every 4 weeks beginning in the 28th week of gestation (GRADE 1C); (5) for patients with uncomplicated sickle cell disease and a normally-grown fetus, we suggest weekly or twice-weekly antenatal testing beginning at 32 to 34 weeks of gestation. For patients with complicated sickle cell disease (i.e., maternal hypertension, vaso-occlusive crises, fetal growth restriction, or other coexisting complication), we suggest initiating individualized antenatal testing at diagnosis or at a gestational age when delivery would be considered if results are abnormal (GRADE 2B); (6) we recommend following evidence-based guidelines for the management of chronic and acute pain during pregnancy (Best Practice); (7) we suggest the use of prophylactic transfusions be individualized for high-risk patients with sickle cell disease in accordance with American Society of Hematology guidelines and directed by a hematologist and maternal-fetal medicine subspecialist in shared decision-making with the patient (GRADE 2B); (8) we recommend shared decision-making occur regarding the use of hydroxyurea in pregnancy, in conjunction with a sickle cell disease specialist and maternal-fetal medicine subspecialist, accounting for the timing of use and individual disease severity (GRADE 1C); (9) we recommend that reliable contraception be offered to patients with sickle cell disease to decrease the risk of an unintended pregnancy and associated maternal and perinatal risks (GRADE 1B).