Gynecologic and obstetric management of girls and women with von Willebrand disease

Among the first patients with von Willebrand disease (VWD) described by Eric von Willebrand almost a century ago were young girls and women from a family in the Åland Islands who experienced "genital hemorrhage" so excessive that it led to exsanguination in five members.1 Only recently has attention focused on the risks and challenges that females with VWD face when experiencing heavy menstrual bleeding, conception, pregnancy, and delivery. The persistent monthly losses with heavy menstrual bleeding and increased demands during pregnancy commonly result in iron deficiency, but the lack of consensus regarding screening and treatment leads to underdiagnosis and undertreatment. While the genetics of VWD are known, female members of affected kindreds are infrequently screened and not considered at risk. Among girls and women with VWD in whom reproductive tract bleeding is the most common symptom, a diagnosis of VWD is often delayed up to a decade or more, leading to significant morbidity, fatigue, depression, iron deficiency, and poor quality of life. Furthermore, there is low certainty regarding effectiveness of current therapies and a lack of prospective trials to guide treatment. Novel therapeutics for inherited bleeding disorders are on the horizon, but women are excluded from studies and have poor access to care. While collaborative hematology-gynecology clinics have improved outcomes, females continue to be excluded from decision-making about their own care. Now, 100 years after the first cases of VWD were reported, it is timely to advocate for better care and management of females with reproductive tract bleeding to assure their future well-being.