Pregnancy and Childbirth in a Woman with VWD − A Case Study
Pregnancy and childbirth are relatively demanding periods and situations for women with von Willebrand disease (vWD) from the perspective of hemostasis. They can be associated with an increased risk of bleeding for both the mother and the newborn. For this reason, a multidisciplinary approach to every pregnant woman is essential, involving a hematologist (ideally in a specialized center), the obstetric team, and then also a gynecologist who will care for the woman in the postpartum period. The optimal approach is to create a birth plan early, which will be carefully followed during labor and especially in the postpartum period. The following case study clearly illustrates these points.
History and Initial Condition
A 20-year-old woman with severe type 2M vWD arrives in the 10th week of pregnancy. Her baseline factor VIII (FVIII) level is 58%, von Willebrand factor (vWF) antigen level is 0.28%, activity measured using RCo is < 40%, vWF:collagen binding is 18%, and multimer analysis is normal. This is her first pregnancy.
From the hematologist’s perspective, it is very important to know the precise classification of the disease, the degree of vWF deficiency, previous bleeding issues, the type of inheritance, and other factors already during pregnancy. If the family is interested, prenatal diagnosis can still be focused on at this stage of diagnosis.
Changes During Pregnancy
Concerning the physiological changes in a healthy woman accompanying pregnancy, there is a typical tendency towards hypercoagulability during the early stages of pregnancy with a rise in vWF and FVIII levels, reaching a maximum at the time of delivery. Women with vWD also show an increase in vWF and FVIII during pregnancy, but not to the same extent as healthy women. Furthermore, due to the heterogeneity of vWD presentation, each vWD subtype may behave differently.
Women with type 1 vWD with baseline FVIII and vWF levels > 30% can be expected to have levels of these factors within the physiological range by the end of pregnancy. In type 2, both vWF and FVIII levels also increase, but the deficiency of high molecular weight multimers does not change, so the functionality of VWF:RCo remains reduced. In subtype 2B, pregnancy may exacerbate the degree of thrombocytopenia, while in subtype 2N, FVIII levels are lower compared to vWF. Women with type 3 do not show any increase in FVIII or vWF levels.
In the case of the observed woman, changes in FVIII and vWF levels aligned with expected increases; however, vWF:RCo remained unchanged. In the third trimester, the measured levels were FVIII 97%, vWF:Ag 50%, and vWF:RCo < 40%.
Preparation for Childbirth
Women with vWD should be comprehensively evaluated by a multidisciplinary team between the 32nd and 34th gestational weeks. The birth plan should be finalized at this time, including the mode of delivery, hemostatic management, anesthesia management, pain management, and care for the infant during the postpartum period. The plan must be communicated with the mother-to-be.
Women with type 1 vWD and vWF:RCo levels > 50% in the third trimester with a mild bleeding phenotype may give birth in a local facility if they have no other additional risk factors (such as previous postpartum bleeding). Women with types 2 and 3 or with a more severe type 1 should give birth in a facility associated with a hematology specialized center.
One highly debated topic is the provision of neuraxial block in women with bleeding disorders. For example, British guidelines do not recommend its use unless vWF activity is > 50% or the hemostatic defect is corrected. The level > 50% should be maintained throughout the duration of the catheter placement and for at least 6 hours after its removal. In cases of a mild bleeding phenotype, only fibrinolytics may be used.
Course of Delivery and Postpartum Care
Postpartum hemorrhage (PPH) is defined according to recent vWD guidelines as a blood loss ≥ 1000 ml within the first 24 hours after delivery or any blood loss with the potential for hemodynamic instability. Women with vWD have a clearly higher risk of PPH. Thus, careful monitoring of potential complications by the obstetrician and hematologic management is essential. It is highly recommended that FVIII levels and vWF activity be monitored after delivery, especially in cases of prolonged labor or extensive bleeding. In women managed with vWF concentrate replacement therapy, it is crucial that FVIII and vWF levels are maintained > 50% for 3-5 days after an uncomplicated vaginal delivery or 5-7 days after surgery. In more severe cases of vWD, replacement therapy may be administered for several weeks postpartum. Additionally, tranexamic acid is recommended for all women with vWD until the cessation of lochia.
The woman opted for a planned cesarean section due to concerns about delivery and potential complications for the child. The delivery was planned for the 39th week following preparation with a plasma-derived concentrate with a lower FVIII content. The first dose was administered before the insertion of the spinal catheter, along with the first dose of tranexamic acid. Factor concentrates were administered every 8 hours for the first 24 hours, then twice daily the following day, and once daily from the third to the seventh day. The patient used tranexamic acid for 4 weeks postpartum until the cessation of postpartum bleeding. The child weighed just under 3 kg, and estimated blood loss was 450 ml. Examination of the umbilical cord blood revealed that the child also has vWD.
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Source: Turan O., Kadir R. A. Pregnancy in special populations: challenges and solutions practical aspects of managing von Willebrand disease in pregnancy. Hematology Am Soc Hematol Educ Program 2021; 1: 552−558, doi: 10.1182/hematology.2021000321.
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