Abstract Number: PB0923
Meeting: ISTH 2021 Congress
Background: Patients with VWD experience bleeds that can affect health-related quality of life (HRQoL) and may have long-term consequences. The magnitude of this burden on patients is not yet fully elucidated.
Aims: Estimate the prevalence and burden of clinical complications associated with VWD (types 1, 2, and 3), economic burden, and HRQoL of affected individuals.
Methods: Embase®, MEDLINE®, and the Cochrane Library were searched (database inception–September 1, 2020) for studies on VWD, including conference abstracts (2017–2020). All study designs were considered. References were screened by 2–3 independent reviewers.
Results: Data are reported for patients with VWD from North American, European, and non-European countries. In 16 clinical burden studies (Figure 1) including 72,450 patients of all ages with VWD, the most reported bleeding symptoms were menorrhagia (2–95%, mean [SD] 46.2% [39.1]; n=7 studies), epistaxis (12–80%, 45.0% [33.5]; n=5), and easy bruising (46–65%, 55.5%; n=2). In 25 cost and resource use studies (79,885 patients of all ages with VWD), costs varied widely; higher costs for patients with VWD compared to patients with non-VWD disorders were reported. Patients with bleeding complications accrued higher costs regardless of VWD type. In two US studies, patients with VWD and severe complications (i.e. major bleeding) were more likely to have inpatient admission and emergency room visits. In 17 HRQoL studies (5,730 patients of all ages with VWD), QoL was assessed with the SF-36 (n=6), VWD-QoL (n=1), and EuroQol-5 Dimension (n=2) scales; all studies assessing treatment impact on VWD (except one) reported an improvement in single or multiple components of HRQoL scales.
Conclusions: Despite available data being highly variable and mostly published as conference abstracts, patients with VWD experience a high disease burden, which warrants further systematic investigation including cost consequence analysis, with the ultimate purpose of improving QoL.
To cite this abstract in AMA style:Castaman G, Jansen N, Santos S, Berntorp E. Clinical, Economic, and Quality of Life Burden Associated with von Willebrand Disease (VWD) in Adults and Children: Systematic and Targeted Literature Reviews [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/clinical-economic-and-quality-of-life-burden-associated-with-von-willebrand-disease-vwd-in-adults-and-children-systematic-and-targeted-literature-reviews/. Accessed November 29, 2021.
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