Relative contributions of bleeding scores and iron status on health‐related quality of life in von Willebrand disease: a cross‐sectional study

Xu, Yan; Deforest, Meghan; Grabell, Julie; Hopman, Wilma M.; James, Paula

doi:10.1111/hae.13062

Cited by 30 publications

(63 citation statements)

References 36 publications

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“…Compared with normative data, women with VWD reported significant reductions ( P < .05) in all domain components except “role emotional,” including vitality, mental health, and mental component summary; iron deficiency was one of the contributory factors. 40 The mental component score was also reduced compared with controls in women diagnosed as hemophilia carriers and in the subgroup with a bleeding tendency ( P = .05 for both comparisons); however, there were no significant differences in the individual domains. 36 In 355 people with VWD recruited to a French study, the mental component summary score at baseline was lower among women than men (no statistical significance was reported).…”

Section: Resultsmentioning

confidence: 89%

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The lived experience of women with a bleeding disorder: A systematic review

Sanigorska

Chaplin²,

Holland³

et al. 2022

Research and Practice in Thrombosis and Haemostasis

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Background Research studies have described the morbidity associated with inherited bleeding disorders such as hemophilia and von Willebrand disease in women, but their effect on daily living has long been underrecognized. This systematic review sought to document the lived experience of women with a bleeding disorder by assessing research findings on quality of health care, socioeconomic factors, and mental health. Methods A systematic search was carried out in Web of Science, the Cumulative Index to Nursing and Allied Health Literature, and PubMed on July 31, 2020. References were hand searched. Abstracts of the 2019 and 2020 congresses on bleeding disorders were hand searched. Key journals were screened for relevant studies published after the search date until the analysis was completed on December 7, 2020. Results Of 635 potentially eligible publications, 27 published since 1998 were selected for review. Most studies were of moderate to high quality but meta‐analysis of quantitative studies was not possible due to difference in outcomes and assessment. Women with a bleeding disorder experience obstacles to accessing care, difficulties living with their disorder, interference with schooling and work, and poor mental health. Diagnostic delay and lack of recognition of symptoms mean treatment and support may not be available. Where comparisons with controls were made, women’s negative experiences were greater than those of men. Conclusions Women with bleeding disorders experience major negative impacts of their disorders on daily life and mental well‐being. Many of the challenges identified in earlier research are evident in more recent studies.

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Section: Resultsmentioning

confidence: 89%

“…Thirteen studies reported mental health outcomes. 19 , 20 , 22 , 23 , 24 , 26 , 30 , 31 , 32 , 35 , 36 , 37 , 40 …”

Section: Resultsmentioning

confidence: 99%

The lived experience of women with a bleeding disorder: A systematic review

Sanigorska

Chaplin²,

Holland³

et al. 2022

Research and Practice in Thrombosis and Haemostasis

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“…17 Smaller studies performed in Canada (102 patients, SF-36 questionnaire), Finland (47 patients, Medical Outcomes Study-36 questionnaire), Sweden (30 female patients, SF-36 questionnaire), Canada again (28 patients, Health Utility Index Mark-2 and Mark-3) or United Kingdom (57 female patients, general questionnaire) not only confirmed a lower quality of life for VWD patients compared to the general population, but for studies in which both men and women were included, most reported a difference between both genders. [18][19][20][21][22] Studies focusing primarily on women clearly indicate how the burden of VWD is borne disproportionately by women. Heavy menstrual bleeding and its associated pain (observed in 32-100% of patients), 23 post-partum hemorrhage (in 15-60% of patients), 13 and hysterectomy (performed in 10-26% of patients), 24 are common in VWD women.…”

Section: Vwd and Quality Of Lifementioning

confidence: 99%

von Willebrand disease: what does the future hold?

Denis

Sophie

Lenting

2021

Blood

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Von Willebrand disease (VWD) is characterized by its heterogeneous clinical manifestation, which complicates its diagnosis and management. The clinical management of VWD has remained essentially unchanged over the last 30 years or so, using von Willebrand factor (VWF) concentrates, desmopressin and anti-fibrinolytic agents as main tools to control bleeding. This is in contrast to hemophilia A, for which a continuous innovative path has led to novel treatment modalities. Despite current VWD management being considered effective, quality-of-life studies consistently reveal a higher than anticipated burden of VWD on patients, which is particularly true for women. Apparently, despite our perceived notion of current therapeutic efficiency, there is space for innovation with the goal to reach superior efficacy. Developing innovative treatments for VWD is complex, especially given the heterogeneity of the disease and the multifunctional nature of VWF. In this perspective article, we describe several potential strategies that could provide the basis for future VWD treatments. These include genetic approaches such as gene therapy using dual-vector adeno-associated virus and transcriptional silencing of mutant alleles. Furthermore, protein-based approaches to increase FVIII levels in VWD-type 3 or 2N patients will be discussed. Finally, antibody-based options to interfere with VWF degradation (for congenital VWD-type 2A or acquired Von Willebrand syndrome-type 2A) or increase endogenous VWF levels (for VWD-type 1) will be presented. By highlighting these potential strategies, we hope to initiate an innovative path, which ultimately would allow us to better serve VWD patients and their specific needs.

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“…Clinical manifestations of VWD include mucocutaneous bleeding such as mouth and nose bleeds, gastrointestinal bleeds, joint bleeds, and menorrhagia [16], and bleeding severity can vary depending on the disease type. Patients with severe VWD (mostly type 2 and 3) may suffer from recurrent bleeding, with a potential negative impact on quality of life [15,[17][18][19][20]. In addition, in patients with recurrent joint bleeding, the detrimental effect on long-term joint outcomes may be comparable with moderate/severe hemophilia [19].…”

Section: Introductionmentioning

confidence: 99%