Table 3.

Summary of recommendations for obstetrical care of women with factor XI deficiency.

Multidisciplinary Care Team	Preconception or early pregnancy consultation with hematology Preconception or early pregnancy consultation with maternal-fetal medicine/perinatology Consideration for preconception or early pregnancy consultation with genetic counseling Routine prenatal care with obstetrician and/or midwife Third trimester consultation (28–32 weeks’ gestation) with anesthesiology Multidisciplinary care plan formulated, documented, and communicated in patient’s chart by 32 weeks’ gestation
Laboratory Data	Preconception or early pregnancy FXI level Third trimester (28–32 weeks’ gestation) FXI level
Intrapartum and Delivery Care	Consider delivery at a tertiary care center with specialized care (hematology consult, transfusion capabilities) Consider consultation with hematology and maternal-fetal medicine/perinatology on admission to Labor and Delivery Unit (LDU) Team-based communication to ensure needs for medication, equipment, and personnel are anticipated and met Ongoing assessment of evolving hemorrhage risk and reevaluation of plan with hematology as needed Avoid operative vaginal delivery (forceps or vacuum) and scalp electrode placement Active type and screen in the blood bank Patients with a negative bleeding history (low-to- moderate risk): Prophylactic tranexamic acid administration at the time of delivery (1 g IV) or standard of care treatment Prophylactic oxytocin administration per local standard of care Anticipatory planning with blood bank and/or pharmacy Patients with a positive or unknown bleeding history (high risk): Prophylactic tranexamic acid administration at time of delivery (1 g IV) at time of delivery for all patients if FXI not a consideration Consider prophylactic administration of FXI concentrate (15 U/kg), plasma (15–20 mL/kg) or rFVIIa (15–30 μg/kg) If not given prophylactically, ensure that FXI concentrate, plasma, or rFVIIa is immediately available, if needed.
Anesthesia Care	Consider low-risk pain-control options such as IV medications or inhaled nitrous oxide Consider prophylaxis for regional anesthesia placement with tranexamic acid, plasma, FXI concentrate, or rFVIIa based on patient’s risk
Neonatal Care	Notify pediatric care team of maternal FXI deficiency Hold pressure for 15 min after intramuscular injections Avoid invasive procedures until hematology evaluation