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. 2018 May 2;16(1):84.
doi: 10.1186/s12955-018-0908-9.

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

Jamie O'Hara  1   2 , Shaun Walsh  2 , Charlotte Camp  3   4 , Giuseppe Mazza  5 , Liz Carroll  6 , Christina Hoxer  7 , Lars Wilkinson  7
Affiliations

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

Jamie O'Hara et al. Health Qual Life Outcomes. .

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.

Keywords: Disease burden; Haemophilia; Health-related quality of life; Joint disease; Patient-reported outcome measures.

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Conflict of interest statement

Ethics approval and consent to participate

The CHESS study was reviewed by the Research Ethics Sub Committee of the Faculty of Health and Social Care within the University of Chester. Patient consent for use of clinical data was not required (as per European Pharmaceutical Market Research Association (EPhMRA) guidelines). Patient consent was obtained via tick box selection for the PSC element of the study.

Competing interests

LW and CH are employees of Novo Nordisk.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Distribution of study cohort by count of target joints (N = 515)
Fig. 2
Fig. 2
EQ-5D-3 L index score by number of target joints (N = 515)
Fig. 3
Fig. 3
EQ-5D-3 L index score by location of target joint
Fig. 4
Fig. 4
EQ-5D VAS score by count of target joints
See this image and copyright information in PMC

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