Social participation is reduced in type 3 Von Willebrand disease patients and in patients with a severe bleeding phenotype

• Published in: Haemophilia : the official journal of the World Federation of Hemophilia Vol. 28; no. 2; pp. 278 - 285
• Main Authors: Kempers, Eva K., Kwawegen, Calvin B., Meris, Joke, Schols, Saskia E. M., Galen, Karin P. M., Meijer, Karina, Cnossen, Marjon H., Bom, Johanna G., Fijnvandraat, Karin, Eikenboom, Jeroen, Atiq, Ferdows, Leebeek, Frank W. G.
• Format: Journal Article
• Language: English
• Published: England Wiley Subscription Services, Inc 01.03.2022; John Wiley and Sons Inc
• Subjects: absenteeism; Adolescent; Adult; Bleeding; Cross-sectional studies; educational status; Female; Hemophilia; Hemorrhage - complications; Humans; Male; Original; ORIGINAL ARTICLES; Participation; Patients; Phenotype; Phenotypes; Regression analysis; Social Participation; von Willebrand Disease, Type 1 - complications; von Willebrand Disease, Type 3 - complications; von Willebrand Diseases; von Willebrand Diseases - complications; von Willebrand Factor - genetics; Netherlands; von Willebrand Diseases; absenteeism; bleeding; educational status; social participation; cross-sectional studies
• ISSN: 1351-8216; 1365-2516
• DOI: 10.1111/hae.14475

Introduction The negative impact of haemophilia on social participation is well established in previous studies, however, the impact of Von Willebrand disease (VWD) on social participation has not been studied. Aim To compare the social participation of a large cohort of VWD patients in the Netherlands with the general Dutch population. In addition, to identify factors associated with social participation in VWD. Methods Patients participating in the “Willebrand in the Netherlands” study completed an extensive questionnaire on educational level, absenteeism from school or work, and occupational disabilities. Results Seven‐hundred and eighty‐eight VWD patients were included (mean age 38.9 years, 59.5% females), of whom 136 children < 16 years. Adult patients with type 3 VWD more often had a low educational level (52.9%) compared to type 1 (40.2%), type 2 VWD (36.8%) and the general population (36.4%) (p = .005). Moreover, in patients aged ≥16 years the days lost from school and/or work in the year prior to study inclusion differed significantly between the VWD types (p = .011). Using negative binomial regression analysis, the occurrence of bleeding episodes requiring treatment in the year preceding study inclusion was significantly associated with the number of days lost from school and/or work among patients aged ≥16 years. Multivariable logistic regression analysis showed that a higher total bleeding score, older age and presence of at least one comorbidity were significantly associated with occupational disability in patients aged ≥16 years. Conclusion Our study shows that social participation was lower in type 3 VWD and VWD patients with a more severe bleeding phenotype.