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Health and Quality of Life Outcomes
Published in: Health and Quality of Life Outcomes 1/2018

Open Access 01-12-2018 | Research

The impact of severe haemophilia and the presence of target joints on health-related quality-of-life

Authors: Jamie O’Hara, Shaun Walsh, Charlotte Camp, Giuseppe Mazza, Liz Carroll, Christina Hoxer, Lars Wilkinson

Published in: Health and Quality of Life Outcomes | Issue 1/2018

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Abstract

Background

Joint damage remains a major complication associated with haemophilia and is widely accepted as one of the most debilitating symptoms for persons with severe haemophilia. The aim of this study is to describe how complications of haemophilia such as target joints influence health-related quality of life (HRQOL).

Methods

Data on hemophilia patients without inhibitors were drawn from the ‘Cost of Haemophilia across Europe – a Socioeconomic Survey’ (CHESS) study, a cost-of-illness assessment in severe haemophilia A and B across five European countries (France, Germany, Italy, Spain, and the UK). Physicians provided clinical and sociodemographic information for 1285 adult patients, 551 of whom completed corresponding questionnaires, including EQ-5D.
A generalised linear model was developed to investigate the relationship between EQ-5D index score and target joint status (defined in the CHESS study as areas of chronic synovitis), adjusted for patient covariates including socio-demographic characteristics and comorbidities.

Results

Five hundred and fifteen patients (42% of the sample) provided an EQ-5D response; a total of 692 target joints were recorded across the sample. Mean EQ-5D index score for patients with no target joints was 0.875 (standard deviation [SD] 0.179); for patients with one or more target joints, mean index score was 0.731 (SD 0.285). Compared to having no target joints, having one or more target joints was associated with lower index scores (average marginal effect (AME) -0.120; SD 0.0262; p < 0.000).

Conclusions

This study found that the presence of chronic synovitis has a significant negative impact on HRQOL for adults with severe haemophilia. Prevention, early diagnosis and treatment of target joints should be an important consideration for clinicians and patients when managing haemophilia.
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Metadata
Title
The impact of severe haemophilia and the presence of target joints on health-related quality-of-life
Authors
Jamie O’Hara
Shaun Walsh
Charlotte Camp
Giuseppe Mazza
Liz Carroll
Christina Hoxer
Lars Wilkinson
Publication date
01-12-2018
Publisher
BioMed Central
Published in
Health and Quality of Life Outcomes / Issue 1/2018
Electronic ISSN: 1477-7525
DOI
https://doi.org/10.1186/s12955-018-0908-9

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