Revisions: 251; 175; 252
Pregnancy should be managed by a multidisciplinary team of specialists, including a coagulation
disorders specialist, an obstetrician/gynecologist and an anesthesiologist, all of whom are knowledgeable in the management of women with bleeding disorders. Early pregnancy care should include anticipatory guidance about bleeding because spontaneous early pregnancy loss is associated with need for interventions in approximately 50%. (5) Women with bleeding disorders are not generally believed to be at increased risk of miscarriage unless they have either fibrinogen or factor XIII deficiency. (4,6, 7) Women who have fibrinogen deficiency with a bleeding phenotype are at a high risk of miscarriage if their fibrinogen levels are below 100 mg/dL. They should receive replacement therapy during pregnancy to maintain their levels above 100 mg/dL. (6) Women with severe factor XIII deficiency are also at high risk of miscarriage and should also receive replacement therapy monthly during pregnancy. (4)
Any early pregnancy procedures should be performed in settings where hemorrhage could be managed, including uterine evacuation for early pregnancy loss and procedural abortion. Bleeding prophylaxis for uterine evacuation can include uterotonics and anti-fibrinolytics (e.g. tranexamic acid). Prophylaxis with VWF concentrate, desmopressin, and/or anti-fibrinolytics is indicated prior to uterine evacuation and other invasive procedures during pregnancy, such as chorionic villous sampling, amniocentesis, and cerclage.
Hemophilia carriers and women with VWD should receive factor replacement prophylaxis at the time of delivery if their factor levels are less than 50% (50 IU/dL). (2-4) When available, recombinant or virally inactivated clotting factor concentrate should be used as opposed to fresh frozen plasma or cryoprecipitate. (2-4) Women with factor levels above 50% (50 IU/dL) should be given the option of neuraxial anesthesia. (8) Targeting a VWF activity level of 0.50 to 1.50 IU/mL over targeting an activity level of 1.50 IU/mL is advisable. (9) VWF activity levels should be maintained at 0.50 IU/mL while the epidural is in place and for at least 6 hours after
removal. The assessment of whether neuraxial anesthesia is appropriate for an individual patient is a complex decision that includes assessment of factors outside the scope of these guidelines. The ultimate decision about whether it is appropriate for an individual patient to undergo these procedures lies with the obstetric anesthesiologist or other clinician performing the procedure. Decisions regarding anesthesia and delivery should be made in the context of a multidisciplinary discussion with input from anesthesia, hematology, and obstetrics and shared decision making with the patient. These discussions should take place well in advance of the patient’s due date. Patients should also be assessed for thrombotic risk post-delivery, and prophylaxis (e.g., compression stockings or low-molecular-weight heparin) should be provided when needed.
(See MASAC Document #250 for specific treatment product recommendations for each of these bleeding disorders.)
Obstetricians and midwives routinely take precautions to prevent postpartum hemorrhage, and this is especially important in women with bleeding disorders. The third stage of labor should be actively managed to reduce blood loss and reduce the incidence of postpartum hemorrhage. (7, 15) Anti-fibrinolytics can be used to prevent or treat postpartum hemorrhage. (4, 7) Specifically, tranexamic acid is advised in women with type 1 VWD or low VWF levels (and this may also apply to types 2 and 3 VWD) during the postpartum period.(9) Tranexamic acid may be given systemically via the oral or IV route. The oral dose is 25 mg/kg (typically 1000-1300 mg) 3 times per day for 10 to 14 days or longer if blood loss remains heavy. (9)
Factor levels may drop precipitously postpartum and should be monitored. In women who require clotting factor replacement therapy, prophylaxis should be continued for at least five days postpartum or longer, since delayed postpartum hemorrhage is not uncommon and may occur more than two weeks after delivery.(3, 7, 12) Clotting factor replacement initially should be targeted closer to the physiological level of 150% then 50%. (9)
- Srivastava A, Santagostino E, Dougall, et al. Haemophilia 2020. Suppl 6: 1-158.
- Lee CA, Abdul-Kadir R. von Willebrand disease and women's health. Semin Hematol.
2005 Jan; 42(1):42-48.
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