Relative contributions of bleeding scores and iron status on health-related quality of life in von Willebrand disease: a cross-sectional study.
BACKGROUND
von Willebrand disease (VWD) is the most common inherited bleeding disorder known in humans. Currently, studies investigating the health-related quality of life (HR-QoL) in VWD using standardized tools are limited, particularly among patients with mild decreases in von Willebrand factor or activity.


OBJECTIVE
To determine HR-QoL and its predictors among patients with mild, moderate and severe forms of VWD.


METHODS
Patients with clinical diagnosis of VWD were recruited from a tertiary Inherited Bleeding Disorder Clinic. Upon informed consent, bleeding scores were obtained via a standardized, self-administered tool. Each participant also completed a HR-QoL questionnaire (SF-36). Analyses included paired t-test, independent t-test, one-way anova and multivariate regression.


RESULTS
A total of 102 patients were recruited (consent rate = 95%). Participants were 38 years on average (SD 14.8), 78% were female and 80% were diagnosed with VWD Type 1. Compared to age- and sex-matched normative data, VWD patients had clinically and statistically significant reductions in seven of eight HR-QoL domains and the physical and mental component summaries. Adjusted for age, sex, socioeconomic status and rurality, there was a trend towards lower physical component summary with increasing bleeding score, and lower mental domains with iron deficiency status (P = 0.07 and P = 0.08 respectively).


CONCLUSIONS
This study is the first to examine the impact of VWD on HR-QoL across disease severity while incorporating socioeconomic status and rurality. Significant reductions in HR-QoL among VWD patients, especially the relationship between iron status and mental HR-QoL, strengthen the rationale for prospective studies to evaluate the efficacy of iron replacement in this setting.
School of Medicine, Queen's University, Kingston, Canada.
Y
Y
Xu
NCIC Clinical Trials Group, Queen's University, Kingston, Canada.
M
M
Deforest
Department of Pathology and Molecular Medicine, Queen's University, Kingston, Canada.
J
J
Grabell
Clinical Research Centre, Kingston General Hospital, Queen's University, Kingston, Canada.|Department of Public Health Sciences, Queen's University, Kingston, Canada.
W
W
Hopman
Department of Pathology and Molecular Medicine, Queen's University, Kingston, Canada.|Department of Medicine, Queen's University, Kingston, Canada.
P
P
James
D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006470:Hemorrhage; D006801:Humans; D007501:Iron / Q000378:metabolism*; D008297:Male; D011788:Quality of Life; D014842:von Willebrand Diseases / Q000175:diagnosis*
D007501:Iron
haemorrhage; quality of life; rural population; social class; von Willebrand disease