Table 7.

Obstetric management for hemophilia carriers²,⁴⁰,⁵⁹,⁷²–⁸¹

Item	Hemophilia A	Hemophilia B
Factor levels	FVIII level increased 2- to 3-fold during pregnancy Median FVIII/FIX levels are 55–68 IU/dL but may range from < 10 IU/dL to > 100 IU/dL	FIX level increased only slightly As for hemophilia A
Obstetric clinical area
Second- and third-trimester activity	Multidisciplinary consultations and labour and delivery planning are required	As for hemophilia A
Second stage before delivery	Tranexamic acid, 1000 mg orally
Anesthesia	FVIII level > 0.5 IU/dL is required Regional anesthesia risk with coagulation factor levels < 0.5 IU/dL, as prevalence of neuraxial hematoma is 2% with factor levels 1% of normal76	FIX level > 0.5 IU/dL is required As for hemophilia A
Low level of coagulation factor (VIII/IX)	FVIII level < 0.8 IU/dL: FVIII coagulation factor replacement to achieve > 100–150 IU/dL at delivery, or desmopressin, 0.3 μg/kg (prepregnancy weight) nasally or subcutaneously after cord clamping*	FIX level < 1%–30% of normal: FIX coagulation factor replacement at 80 U/kg FIX level > 30%–60% of normal: FIX coagulation factor replacement at 50 U/kg
Delivery	Use of ventouse, midcavity forceps, fetal scalp blood sampling, fetal scalp electrode should be avoided in fetuses at risk Active third-stage management with evaluation of vaginal blood loss	As for hemophilia A As for hemophilia A
Postpartum hemorrhage	Prophylaxis therapy: coagulation factor replacement is most common therapy; desmopressin, plasma or tranexamic acid rarely used	FIX level < 1%–30% of normal: tranexamic acid, FIX coagulation factor replacement at 50 U/kg FIX level > 30%–60% of normal: tranexamic acid, FIX coagulation factor replacement at 15 U/kg
Postpartum period	Maintain FVIII level > 0.5 IU/dL for 3 d with vaginal delivery and 5 d with cesarean delivery Tranexamic acid, 1000 mg 3–4 times per day for 7 d, including until lochia is minimal Hospital- and home-based monitoring of blood loss should be reported to obstetric provider	Maintain FIX level > 0.5 IU/dL for 3 d with vaginal delivery and 5 d with cesarean delivery As for hemophilia A As for hemophilia A

FIX = factor IX; FVIII = factor VIII.

^*Desmopressin is safe in the first and second trimesters, but safety at delivery has not been shown (risk of fluid overload and hyponatremia/fluid restriction). Saline use and sodium evaluation are required.