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Examining Current Practices in the Care of Pregnant Women With Low Von Willebrand Factor / Disease
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Examining Current Practices in the Care of Pregnant Women With Low Von Willebrand Factor / Von Willebrand Disease


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Von Willebrand Factor (VWF) is a plasma coagulation protein essential for normal hemostasis. Quantitative or qualitative deficiencies of VWF result in a bleeding diathesis, Von Willebrand Disease (VWD). Hemorrhagic episodes are usually mucocutaneous in nature. Gynecological bleeding, especially heavy menstrual bleeding (HMB), may be problematic for women with VWD, and often requires medication or intervention to control.1,2 Cohort studies have identified rates of HMB of up to 90% in studies of women with VWD.1,2 However, the rates of postpartum hemorrhage (PPH) in women with VWD are more difficult to define, with data predominantly from small retrospective studies. 2–6


During pregnancy, plasma VWF levels exhibit a physiological increase with peripartum plasma VWF levels 3-4 fold above baseline.7 In women with type 1 VWD and low VWF, plasma VWF levels may increase to within the normal laboratory reference range (50-150 IU/dL). This pregnancy related rise in plasma VWF levels in women with type 1/low VWF mirrors that seen in control pregnancies, however plasma VWF levels remain lower than controls at all timepoints.7 These data raise critical questions regarding the clinical management of pregnant women with VWD. Firstly, whether this pregnancy related “normalization” of plasma VWF in women with type 1 VWD/low VWF is sufficient to prevent bleeding remains unclear. Secondly, the optimal plasma VWF threshold for initiation of replacement therapy in this clinical context (i.e. targeting a trough of 50 IU/dL or 100 IU/dL) is uncertain. Finally, whether anti-fibrinolytics (such as tranexamic acid) should be used prophylactically in the cohort to prevent PPH is controversial, with the evidence base lacking.  


The lack of clarity for these important clinical questions is of direct clinical relevance for both women with VWD and healthcare providers (HCP). The VWF SSC is seeking to answer these issues through our PPH in women with VWD project. This project will seek to engage HCP and patients through a multistep process in order to gain real world clinical data on PPH rates in women with VWD. The first component of this project is the “Examining current practices in the care of pregnant women with low Von Willebrand Factor / Von Willebrand Disease” survey. In collaboration with the Women’s Health SSC we have developed a structured, comprehensive questionnaire to determine current international clinical practice in the management of pregnant women with VWD. Analysis of the survey responses will provide an insight into the current clinical practices, treatment thresholds, definitions of and monitoring strategies for PPH in women with VWD. These data will form the baseline for this project and assist in identification of key areas of variance internationally among HCPs in this field.


  Please click here to complete the survey in Spanish.


Expected Timeline: 

The duration of this survey will be available until March 16, 2019.


Project Investigators:

Dr. Michelle Lavin and the Von Willebrand Factor SSC Subcommittee in collaboration with the Women's Health Issues in T&H SSC Subcommittee.



  1. de Wee EM, Knol HM, Mauser-Bunschoten EP, et al. Gynaecological and obstetric bleeding in moderate and severe von willebrand disease. Thromb Haemost. 2011;106(5):885-892. doi:10.1160/TH11-03-0180.
  2. Lavin M, Aguila S, Dalton N, et al. Significant gynecological bleeding in women with low von Willebrand factor levels. Blood Adv. 2018;2(14):1784-1791. doi:10.1182/bloodadvances.2018017418.
  3. James AH, Jamison MGG. Bleeding events and other complications during pregnancy and childbirth in women with von Willebrand disease. J Thromb Haemost. 2007;5(6):1165-1169. doi:10.1111/j.1538-7836.2007.02563.x.
  4. Hawke L, Grabell J, Sim W, et al. Obstetric bleeding among women with inherited bleeding disorders: a retrospective study. Haemophilia. 2016;22(6):906-911. doi:10.1111/hae.13067.
  5. James AH. More than menorrhagia: A review of the obstetric and gynaecological manifestations of bleeding disorders. Haemophilia. 2005;11(4):295-307. doi:10.1111/j.1365-2516.2005.01108.x.
  6. Reynen E, James P. Von Willebrand Disease and Pregnancy: A Review of Evidence and Expert Opinion. Semin Thromb Hemost. 2016;42(7):717-723. doi:10.1055/s-0036-1587686.
  7. James AH, Konkle BA, Kouides P, et al. Postpartum von Willebrand factor levels in women with and without von Willebrand disease and implications for prophylaxis. Haemophilia. 2015;21(1):81-87. doi:10.1111/hae.12568.
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