Study recommends tailoring VWD care to improve quality of life
Women, those with severe disease see particularly 'profound impact'
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Von Willebrand disease (VWD) harms physical and mental health-related quality of life, with an increased burden for women and those with severe disease types, according to a study in France.
The study “demonstrated the profound impact of VWD across physical, mental, and social health domains, highlighting the need for tailored care—especially for patients with severe disease and women,” the researchers wrote.
The study, “Health-related quality of life in Adults with von Willebrand disease: results of the French real-life Willebrand Study on Health-related Quality of Life (WiSH-QoL),” was published in Research and Practice in Thrombosis and Haemostasis.
VWD is a bleeding disorder in which the body cannot properly form blood clots, leading to abnormally heavy and prolonged bleeding. Recurrent bleeding episodes have significant physical, mental, and social consequences.
This WiSH-QoL (Willebrand Study Health-related Quality of Life) study assessed health-related quality of life at 27 treatment centers in France, using generic and VWD-specific patient-reported outcome measures.
Questionnaire responses show disease’s impact
The researchers followed 224 adults, 71% of whom were women, with a median age of 42 and a mean follow-up of 20.6 months. The majority had VWD type 2 (67.9%), followed by 21.8% with type 1 and 6.7% with type 3. Half had severe disease (VWF activity lower than 15 IU/dL).
Most participants (79%) had abnormal Tosetto Bleeding scores, a clinical tool measuring bleeding severity. Clinical events were reported in 60% of patients during the study; most were minor bleeds following surgeries or invasive procedures.
Health-related quality of life was assessed using the generic questionnaire SF-36V2 and the VWD-specific questionnaire VWD-QoL. Treatment satisfaction was evaluated using the global treatment satisfaction score (VWD-SAT).
VWD patients had significantly lower scores on the 36-item SF-36 — indicating lower quality of life — than the general French population in the role-physical, role-emotional, and social functioning domains. The researchers said this highlights “the negative impact of VWD on patients’ physical and social activities as well as mental well-being.”
The scores in the four physical domains of SF-36 — physical functioning, role-physical, bodily pain, and general health — were lowest in people with VWD type 3, the most severe disease type.
SF-36 scores showed no significant changes during follow-up. “This stability may reflect either a slow physical decline due to the underlying disease or the efficacy of therapeutic management,” the researchers wrote.
When analyzing only those patients with type 3 disease, those receiving long-term preventive treatment had significantly poorer physical domain scores than those treated on demand. No differences were seen in mental health scores.
On the VWD-QoL, the most affected dimension was feelings about the future, regardless of disease type. The mean total score was lower in patients with type 3 disease, as they were the most affected across physical, mental, and social health domains. The social impact of the disease was found to increase with disease severity, affecting patients’ self-perception, relationships with family or others, and work.
Patients with VWD type 1 or 2 had similar health-related quality of life scores, although those with more severe disease reported more issues related to leisure and sport activities.
Support could help, researchers say
The use of VWD-specific outcomes also revealed the impact of VWD on women, as men of the same age appeared to be less affected in most areas, particularly in the physical domains.
“These insights highlight the potential value of complementary interventions—such as physiotherapy, adapted physical activity, and psychosocial support—to improve well-being, sustain social relationships, and facilitate professional participation,” the researchers wrote.
The highest treatment satisfaction was found in the centers and specialist categories, and the lowest in the burden and efficacy domains. While there were no significant differences across VWD types, patients with type 2 disease generally reported slightly higher satisfaction levels, particularly related to ease of treatment use.
“While implementing full [patient-reported outcome] questionnaires in routine practice remains challenging, selecting key items could help clinicians address not only physical symptoms but also mental health and social challenges,” the researchers wrote.
Study limitations the investigators noted included a high proportion of adults with severe disease, which may limit the generalizability of the findings to milder forms.
