Journal of Thrombosis and Haemostasis. 8(7):1492–1499, JULY 2010
DOI: 10.1111/j.1538-7836.2010.03864.x
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PMID: 20345712
Issn Print: 1538-7933
Publication Date: July 2010
Health-related quality of life among adult patients with moderate and severe von Willebrand disease
E. De Wee;E. Mauser-Bunschoten;J. Van Der Bom;M. Degenaar-Dujardin;H. Eikenboom;K. Fijnvandraat;A. De Goede-Bolder;B. Laros-Van Gorkom;K. Meijer;H. Raat;F. Leebeek;
+ Author Information
*Hematology, Erasmus University Medical Center Rotterdam†Van Creveldkliniek and Hematology, University Medical Center Utrecht‡Department of Clinical Epidemiology, Leiden University Medical Center§Netherlands Hemophilia Society, Badhoevedorp¶Thrombosis and Hemostasis, Leiden University Medical Center**Pediatric Hematology, Emma Children’s Hospital AMC, Amsterdam††Pediatrics, Erasmus MC, Sophia Children’s Hospital, Rotterdam‡‡Hematology, Radboud University Nijmegen Medical Centre§§Hematology, University Medical Center Groningen¶¶Public Health, Erasmus University Medical Center Rotterdam, the Netherlands1See Appendix.
Abstract
von Willebrand Disease (VWD) is the most frequent inherited bleeding disorder. It is unknown how this disorder affects quality of life.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.HR-QoL was assessed using the Short Form (SF)-36, and bleeding severity was measured using the Bleeding Score (BS).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, co-morbidity 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.HR-QoL is lower in VWD patients compared with the general population. HR-QoL is strongly associated with bleeding phenotype.
