Sports participation and physical activity in patients with von Willebrand disease

Atiq, Ferdows; Mauser‐Bunschoten, Eveline P.; Eikenboom, Jeroen; Galen, Karin P M van; Meijer, Karina; Meris, Joke de; Cnossen, Marjon H.; Beckers, E.; Gorkom, Britta A P Laros-van; Nieuwenhuizen, Laurens; Bom, Johanna G. van der; Fijnvandraat, Karin; Leebeek, Frank W.G.

doi:10.1111/hae.13629

Cited by 17 publications

(27 citation statements)

References 27 publications

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“…Similarly, in our current study, in the total group of autosomal inherited bleeding disorders, boys seemed to have an almost two times higher chance of bleeding requiring treatment in the year prior to inclusion than girls. This is probably due to the fact that boys are more physically active and participate more often in sports with a higher chance of bleeding, as previously shown in VWD patients [ [25] , [26] , [27] ]. Women on the other hand, may present more often with HMB as the first symptom of their bleeding disorder.…”

Section: Discussionmentioning

confidence: 80%

Major differences in clinical presentation, diagnosis and management of men and women with autosomal inherited bleeding disorders

et al. 2021

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Background In recent years, more awareness is raised about sex-specific dilemmas in inherited bleeding disorders. However, no large studies have been performed to assess differences in diagnosis, bleeding phenotype and management of men and women with bleeding disorders. Therefore, we investigated sex differences in a large cohort of well-defined patients with autosomal inherited bleeding disorders (von Willebrand disease (VWD), rare bleeding disorders (RBDs) and congenital platelet defects (CPDs)). Methods We included patients from three nationwide cross-sectional studies on VWD, RBDs and CPDs in the Netherlands, respectively the WiN, RBiN and TiN study. In all studies a bleeding score (BS) was obtained, and patients filled in an extensive questionnaire on the management and burden of their disorder. Findings We included 1092 patients (834 VWD; 196 RBD; 62 CPD), of whom 665 (60.9%) were women. Women were more often referred because of a bleeding diathesis than men (47.9% vs 36.6%, p = 0.002). Age of first bleeding was similar between men and women, respectively 8.9 ± 13.6 (mean ±sd) years and 10.6 ± 11.3 years ( p = 0.075). However, the diagnostic delay, which was defined as time from first bleeding to diagnosis, was longer in women (11.6 ± 16.4 years) than men (7.7 ± 16.6 years, p = 0.002). Similar results were found when patients referred for bleeding were analyzed separately. Of women aging 12 years or older, 469 (77.1%) had received treatment because of sex-specific bleeding. Interpretation Women with autosomal inherited bleeding disorders are more often referred for bleeding, have a longer diagnostic delay, and often require treatment because of sex-specific bleeding. Funding The WiN study was supported (in part) by research funding from the Dutch Hemophilia Foundation (Stichting Haemophilia), Shire (Takeda), and CSL Behring (unrestricted grant).

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Section: Discussionmentioning

confidence: 80%

Major differences in clinical presentation, diagnosis and management of men and women with autosomal inherited bleeding disorders

et al. 2021

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“…2,7 Furthermore, all patients also participated in the WiN study. 3,7,17 A self-administered Tosetto bleeding score, BMI, and presence of comorbidities were assessed at inclusion in the WiN study. In the bleeding score assessment, we did not score for bleeding in case of prophylactic treatment.…”

Section: Assessment Methodsmentioning

confidence: 99%

“…All patients participated in the Willebrand in the Netherlands (WiN) study, for which approval was obtained from our institutional review board. 2,3,7,17 No additional approval was required for the retrospective analyses of the data that are presented in the current article.…”

Section: Patientsmentioning

confidence: 99%

von Willebrand factor and factor VIII levels after desmopressin are associated with bleeding phenotype in type 1 VWD

et al. 2019

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Key Points• VWF and FVIII levels after desmopressin, which mimic hemostatic response, are associated with the bleeding phenotype of type 1 VWD patients.• Variability in VWF and FVIII response to hemostatic challenges may partly explain heterogeneity in bleeding phenotype of VWD patients.The bleeding phenotype of patients with type 1 von Willebrand disease (VWD) is very heterogeneous. We hypothesized that this heterogeneity may partly be explained by variability in response of von Willebrand factor (VWF) and factor VIII (FVIII) levels to stress during hemostatic challenges. We therefore investigated whether VWF and FVIII levels after administration of desmopressin, which mimic in vivo hemostatic response during hemostatic challenges, explain the heterogeneity in bleeding phenotype of patients with type 1 VWD. We performed a retrospective cohort study in 122 patients with type 1 VWD. All patients received a test dose of desmopressin shortly after diagnosis. Patients' mean age was 47 6 14 years, and the mean Tosetto bleeding score was 10 6 7. Higher FVIII activity during the complete time course after desmopressin administration (1, 3, and 5-6 hours), and higher VWF and FVIII levels combined at 3 hours after desmopressin administration, were associated with a lower bleeding score: b 5 -0.9 (-1.7; 20.1) and b 5 -1.2 (-1.9; 20.5), respectively, adjusted for age, sex, body mass index (BMI), and comorbidities. Patients with FVIII activity in the highest quartile 3 hours after desmopressin administration had a much lower bleeding score compared with patients in the other 3 quartiles (b 5 -5.1 [-8.2; 22.0]) and also had a lower chance of an abnormal bleeding score (odds ratio 5 0.2 [0.1-0.5]), both adjusted for age, sex, BMI, and comorbidities. In conclusion, VWF and FVIII levels after desmopressin administration, which mimic hemostatic response to hemostatic challenges, are associated with the bleeding phenotype of patients with type 1 VWD. This may partly explain the variability in bleeding phenotype of these patients.

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“…Primary prophylaxis is commonly performed in severe hemophilia patients, but this is less common in VWD, although patients with severe VWD can still experience significant joint damage, 54 which may affect quality of life, because physical activity may be limited as a result of bleeding or fear of bleeding. 55 Conclusions and research needs for this recommendation.…”

Section: Researchersmentioning

confidence: 99%

ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease

Connell

Flood

Brignardello‐Petersen

et al. 2021

Blood Advances

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Background: von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients. Objective: These evidence-based guidelines from the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), and the World Federation of Hemophilia (WFH) are intended to support patients, clinicians, and health care professionals in their decisions about management of VWD. Methods: ASH, ISTH, NHF, and WFH formed a multidisciplinary guideline panel. Three patient representatives were included. The panel was balanced to minimize potential bias from conflicts of interest. The University of Kansas Outcomes and Implementation Research Unit and the McMaster Grading of Recommendations Assessment, Development and Evaluation (GRADE) Centre supported the guideline development process, including performing and updating systematic evidence reviews (through November 2019). The panel prioritized clinical questions and outcomes according to their importance to clinicians and patients. The panel used the GRADE approach, including GRADE Evidence-to-Decision frameworks, to assess evidence and make recommendations, which were subject to public comment. Results: The panel agreed on 12 recommendations and outlined future research priorities. Conclusions: These guidelines make key recommendations regarding prophylaxis for frequent recurrent bleeding, desmopressin trials to determine therapy, use of antiplatelet agents and anticoagulant therapy, target VWF and factor VIII activity levels for major surgery, strategies to reduce bleeding during minor surgery or invasive procedures, management options for heavy menstrual bleeding, management of VWD in the context of neuraxial anesthesia during labor and delivery, and management in the postpartum setting.

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Sports participation and physical activity in patients with von Willebrand disease

Cited by 17 publications

References 27 publications

Major differences in clinical presentation, diagnosis and management of men and women with autosomal inherited bleeding disorders

Major differences in clinical presentation, diagnosis and management of men and women with autosomal inherited bleeding disorders

von Willebrand factor and factor VIII levels after desmopressin are associated with bleeding phenotype in type 1 VWD

ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease

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