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. 2023 Mar;29(2):505-512.
doi: 10.1111/hae.14741. Epub 2023 Jan 14.

Cross-sectional study evaluating the association of haemophilia-related distress and clinically relevant outcomes

Sara Guasch  1 , Lia C Scott  2   3 , Janet Figueroa  4 , Tyler W Buckner  5 , Shanna Mattis  2   3 , Duc Q Tran Jr  2   3 , Christine L Kempton  2   3
Affiliations

Cross-sectional study evaluating the association of haemophilia-related distress and clinically relevant outcomes

Sara Guasch et al. Haemophilia. 2023 Mar.

Abstract

Introduction: In chronic diseases, disease-related distress can impact disease outcomes. Distress and haemophilia-related distress has been demonstrated in people with haemophilia (PwH). The association of haemophilia-related distress on disease outcomes among PwH is unknown.

Aim: To study the association of haemophilia-related distress with haemophilia specific outcomes, including adherence to prophylactic therapy, the presence of a target joint, healthcare utilization and work-impairment.

Methods: In a cross-sectional study, adults with haemophilia A or B were enrolled in a study to validate the haemophilia-related distress questionnaire (HRDq). In this planned analysis, univariate and multivariate associations between the HRDq total score and disease outcomes were explored.

Results: The 114 participants in this analysis were male, mostly with haemophilia A (92%) and severe disease (52%) with a median age of 31.9 years. On univariate analysis, HRDq total score (5-point change) was associated with the presence of a target joint (P = .002), high healthcare utilization (P = .011), poor adherence (P = .033) and work-impairment (P ≤ .001). After adjustment for age, race, severity and other potential confounders, adherence (aβ 0.33, 95% CI .17, .49) and work-impairment (aβ 4.69, 95% CI 3.27-6.1) remained statistically significantly associated with HRDq total score.

Conclusion: Haemophilia-related distress is associated with poor adherence to factor prophylaxis and work-impairment. The direction of the association (causation) is yet to be determined and requires future study.

Keywords: haemophilia; hemarthrosis; medication adherence; psychological distress.

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References

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