Health status and health‐related quality of life associated with von Willebrand disease

Barr, Ronald D.; Sek, J.; Horsman, John; Furlong, William; Saleh, Mahassen; Pai, Mohan; Walker, Irwin

doi:10.1002/ajh.10327

Cited by 69 publications

(92 citation statements)

References 32 publications

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“…However, the differences in median SF-36 scores were not statistically significant. It is possible that the lack of statistical significance was due to the small sample size of the study population, since several earlier studies demonstrated that women with VWD had lower health-associated quality of life compared with women in the general population (14,28). The studies by Solovieva (33) and de Wee et al (34) reported that the 'vitality' SF-36 dimension was negatively affected in women with VWD.…”

Section: Discussionmentioning

confidence: 90%

Heavy menstrual bleeding and health-associated quality of life in women with von Willebrand's disease

Govorov

Ekelund

Chaireti

et al. 2016

Experimental and Therapeutic Medicine

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Abstract. Women with the inherited bleeding disorder von Willebrand's disease (VWD) face gender-specific hemostatic challenges during menstruation. Heavy menstrual bleeding (HMB) can negatively affect their overall life activities and the health-associated quality of life. The purpose of the present study was to investigate whether women with VWD experienced HMB and an impaired health-associated quality of life. The study subjects were recruited from the Coagulation Unit of Karolinska University Hospital. Information was retrieved from various self-administered forms and medical records. Of the 30 women (18-52 years) that were included in the present study, 50% suffered from HMB, although the majority received treatment for HMB. In addition, almost all the included women perceived limitations in the overall life activities due to menstruation. The health-associated quality of life for women with HMB was significantly lower (P<0.10) with regards to 'bodily pain' compared with Swedish women of the general population. In conclusion, women with VWD experienced reduced health-associated quality of life as a result of HMB. Therefore, preventing limitations in overall life activities and improving their health-associated quality of life thorough counseling on menstrual bleeding is important for women with VWD. IntroductionVon Willebrand's disease (VWD) is the most common inherited bleeding disorder worldwide, with a prevalence varying within 0.1-1% of the general population depending on the method of diagnosis (1-4). The disorder is caused by a quantitative or qualitative deficiency in the von Willebrand factor (VWF). Since VWD is an autosomal inherited disorder, men and women are equally affected. However, more women experience hemostatic challenges through menstrual bleeding and childbirth (5).Heavy menstrual bleeding (HMB), which is defined as >80 ml per menstruation (6), is common in women with VWD. Previous studies have shown that 78-92% of women with VWD suffer from HMB, compared with a 10% prevalence of HMB in all Swedish women of reproductive age (7-9).HMB can be managed through a range of pharmacological treatments, such as contraceptive pills, levonorgestrel-releasing intrauterine systems (LNG-IUS), and tranexamic acid (TA) (10). For women with VWD, treatment with D-arginine vasopressin and VWF/factor VIII concentrates is also an option (11). Despite the availability of extensive pharmacological options, surgical treatment may be necessary (10). Kirtava et al (12) demonstrated that a higher proportion of women with VWD undergo hysterectomy and at a younger age compared with women in the general population.HMB affects numerous aspects of health-associated quality of life, both physical and mental. It can cause iron-deficiency anemia in severe cases, and there are psychological, social and employment consequences associated with HMB (13). Barr et al (14) demonstrated that women with VWD participate less in post-secondary education than the general population, and suggested that this may be due to iron defici...

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Section: Discussionmentioning

confidence: 90%

Heavy menstrual bleeding and health-associated quality of life in women with von Willebrand's disease

Govorov

Ekelund

Chaireti

et al. 2016

Experimental and Therapeutic Medicine

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“…This is the first study on HR-QoL in a large cohort of VWD patients. So far only two articles have been published on VWD and HR-QoL [16,17]. A study by Barr et al used the Health Utility Index 2 and 3 and included only a limited number of type 1 VWD patients (n = 28) [16].…”

Section: Discussionmentioning

confidence: 99%

“…Despite the frequency and severity of bleeding, especially in patients with low VWF levels [11][12][13][14][15], there are only two small studies about the impact of VWD on HR-QoL [16,17]. A study by Barr et al used the Health Utility Index 2 and 3 and included only a limited number of patients (n = 28), most of whom had type 1 disease [16]. Solovieva et al reported HR-QoL of 47 type 2 and type 3 VWD patients [17].…”

Section: Introductionmentioning

confidence: 99%

Health‐related quality of life among adult patients with moderate and severe von Willebrand disease

Wee

Mauser‐Bunschoten

Bom

et al. 2010

Journal of Thrombosis and Haemostasis

112

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Summary. Background: von Willebrand Disease (VWD) is the most frequent inherited bleeding disorder. It is unknown how this disorder affects quality of life. Objectives: This nationwide multicenter cross-sectional study determined health-related quality of life (HR-QoL) in adult patients with moderate or severe VWD, and assessed whether bleeding severity and type of VWD are associated with HR-QoL. Methods: HR-QoL was assessed using the Short Form (SF)-36, and bleeding severity was measured using the Bleeding Score (BS). Results: Five hundred and nine patients participated; 192 males and 317 females, median age and range 45 (16-87) and 47 (16-84) years, respectively. Compared with the general population, HR-QoL in VWD patients was lower in the vitality domain (61 vs. 66 P < 0.001 for females, 67 vs. 72 P < 0.001 for males). Patients with the most severe bleeding phenotype (highest quartile BS, BS > 17) had a lower HR-QoL in eight domains than patients with a less severe bleeding type (lowest quartile BS, BS < 7) in the univariate analysis. After adjustment for age, gender, comorbidity and employment/educational status, a more severe bleeding phenotype was associated with lower scores on the domains of physical functioning, role limitations due to physical functioning, bodily pain, general health, social functioning and physical component summary. Conclusions: HRQoL is lower in VWD patients compared with the general population. HR-QoL is strongly associated with bleeding phenotype.

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“…Even when they do seek help, diagnosis of a bleeding disorder is often overlooked and appropriate treatment is not provided because of the lack of awareness among caregivers. Women with bleeding disorders are therefore more likely to have unnecessary surgical intervention, including hysterectomy, at an early age [8].…”

Section: Introductionmentioning

confidence: 99%

“…Severe PPH is less common, but is a significant contributor to maternal morbidity and accounts for approximately 150 000 deaths per year [9] with a huge impact on the motherless child. PPH can be prevented or its severity can be moderated by educating women and their providers [8]. While delayed or secondary PPH is rare, occurring after <1% of deliveries [10,11], it has been reported in 20-25% of women with VWD [12,13], 2-11% of haemophilia carriers [14,15] and 24% of women with factor XI deficiency [14].…”

Section: Introductionmentioning

confidence: 99%

Building our global family – achieving treatment for all

Skinner

2010

Haemophilia

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Summary. Building our global family by reaching out to women, children and youth and those in sub‐Saharan Africa to achieve Treatment for All. The World Federation of Hemophilia (WFH) has committed to recognizing and incorporating the critical and important challenges that are faced by women with bleeding disorders within our global family. The next crucial steps include the development of outreach and registry programmes which can be adapted globally to accelerate the identification of such women, and to educate and guide them to the appropriate clinical care setting. Equally important, awareness must be raised within the broader medical community where women would typically first present with clinical symptoms. Family practitioners, nurse‐midwives, obstetricians, gynaecologists and community health clinics will increasingly be strategic and central to WFH outreach efforts, in addition to serving as new care partners essential to the multidisciplinary model of care. Adapting and implementing the WFH development model regionally within Africa is proving to be a successful approach both for the introduction as well as the development of sustainable national care programmes for patients with bleeding disorders. The targeted development of solid national programmes such as in South Africa, Senegal and Kenya has expanded the training capacity of the WFH, as well as providing key regional examples. Local medical professionals are now responsible for providing the training in many regional programmes. Children with bleeding disorders in low‐income countries are at great risk of dying young. WFH data demonstrate that among such patients, as the economic capacity of a country decreases so does the ratio of adults to children. The organization of care, training of a multi‐disciplinary healthcare team, and education of patients and their families lead to improved mortality independent of economic capacity or increased clotting factor concentrate availability. Additionally, through enhanced youth education, awareness and engagement, we will assure continuity within WFH national member organizations, build greater unity within our global family and capture the innovation and creativity of their ideas to improve Treatment for All.

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Health status and health‐related quality of life associated with von Willebrand disease

Cited by 69 publications

References 32 publications

Heavy menstrual bleeding and health-associated quality of life in women with von Willebrand's disease

Heavy menstrual bleeding and health-associated quality of life in women with von Willebrand's disease

Health‐related quality of life among adult patients with moderate and severe von Willebrand disease

Building our global family – achieving treatment for all

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