Higher Rates of Bleeding and Use of Treatment Products Found Among Young Boys Compared to Girls with von Willebrand Disease

Racially diverse group of boys and girls smiling for picture

Von Willebrand disease (VWD) is a blood disorder in which the blood does not clot properly. Blood contains many proteins that help the body stop bleeding. One of these proteins is called von Willebrand factor (VWF). People with VWD either have a low level of or no VWF in their blood or the VWF protein doesn’t work the way it should.  Normally when a person is injured and starts to bleed, the VWF in the blood attaches to small blood cells called platelets. This helps the platelets stick together, like glue, to form a clot at the site of injury and stop the bleeding. When a person has VWD, the clot might take longer to form or not form the way it should, and bleeding might take longer to stop. This can lead to heavy, hard-to-stop bleeding. Although rare, the bleeding can be serious enough to damage joints or internal organs, or even be life-threatening. There are several different types of VWD, with type 1 being the mildest and most common form of VWD and type 3 being the rarest and most severe form.

There is little information about VWD diagnosed during childhood, in particular whether there are differences in bleeding symptoms between boys and girls with the various types of VWD.  Early identification of bleeding characteristics may aid physicians in efforts to educate families about seeking care for their child’s bleeding symptoms.  Awareness of how bleeding symptoms differ by type of VWD, sex, and other demographic characteristics may improve outcomes for children with VWD.

About This Study

Researchers analyzed information from 2,712 children with VWD. The children were 2–12 years old and enrolled in the Universal Data Collection (UDC) system during 1998 to 2011.

The UDC is a public health surveillance (health monitoring) system that tracked the prevalence (total number of existing cases) and trends in complications linked to bleeding disorders, such as VWD.  People with bleeding disorders who received care at federally funded hemophilia treatment centers (HTCs) across the United States were eligible to enroll in UDC.

We invite you to read the full reportexternal icon.

Main Findings

  • Children with type 3 VWD (the most severe form)
    • Children with type 3 VWD were diagnosed and had their first bleed 1–2 years earlier compared to children with other VWD types.
    • Children with type 3 VWD reported higher proportions of ever having a bleed compared to children with other VWD types and used HTCs more frequently.
    • Higher treatment product use was seen among children with type 3 VWD.
    • Children with type 3 VWD showed a greater need for mobility assistance (such as the use of a cane, crutches, walker, or wheelchair) compared to children with other types of VWD.
  • Boys versus girls
    • On average, among all VWD types, boys were younger at their first bleed and at diagnosis of VWD than girls.
    • There were no differences in life quality or well-being status in boys versus girls.
    • Among children with type 1 and type 2 VWD, more boys than girls were reported to have had a previous bleeding episode.
    • Also, among children with type 1 and type 2 VWD, more boys than girls had been given a treatment for VWD.
  • Other
    • Some minority children with VWD (Hispanic or Asian/Pacific Islander/American Indian/American Native) were at higher risk of bleeding, compared to children who are non-Hispanic white.

Critical Gaps & Future Directions

Past studies of VWD in children grouped children 18 years of age and younger, despite known age and sex-related differences in growth and puberty.  Girls with VWD often show heavy menstrual bleeding when they go through puberty, but it was not known what may be happening in earlier stages of life.  This study was able to show differences by sex before puberty because surveillance (health monitoring) work at the HTCs allowed for the collection of the largest sample of children with diagnosed VWD.  Interestingly, it showed that boys had more bleeds and higher use of treatment products compared to girls.  Future studies, specifically during adolescence (the period from puberty to legal adulthood usually associated with the teenage years) and into early adulthood are needed to examine whether bleeding symptoms and treatment needs differ by sex during these critical time periods in a person’s development.

More Information

For more information about VWD, please visit our VWD webpages.

Paper Reference

Abe K, Dupervil B, O’Brien SH, et al. Higher rates of bleeding and use of treatment products among young boys compared to girls with von Willebrand disease. Am J Hematol. 2020;95:10–17. https://doi.org/10.1002/ajh.25656