Meaningful and Measurable Health Domains in Huntington's Disease: Large-Scale Validation of the Huntington's Disease Health-Related Quality of Life Questionnaire Across Severity Stages.

Ho, Aileen K; Horton, Mike C; Landwehrmeyer, G Bernhard; Burgunder, Jean-Marc; Tennant, Alan (2019). Meaningful and Measurable Health Domains in Huntington's Disease: Large-Scale Validation of the Huntington's Disease Health-Related Quality of Life Questionnaire Across Severity Stages. Value in health, 22(6), pp. 712-720. Elsevier 10.1016/j.jval.2019.01.016

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BACKGROUND Although health-related quality of life is key for patients with long-term neurodegenerative conditions, measuring this is less straightforward and complex in Huntington's disease (HD). OBJECTIVES To refine and validate a fully patient-derived instrument, the Huntington's Disease health-related Quality of Life questionnaire (HDQoL), and to elucidate health domains that are meaningful to patients' lived experience. METHODS Five-hundred forty-one participants, from premanifest to end-stage disease, completed the HDQoL, together with generic quality-of-life measures and in-person motor, cognitive, and behavioral assessments. The psychometric properties of the HDQoL were examined using factor analysis and Rasch analysis. RESULTS Four HDQoL domains emerged, reflecting the classical triad of HD features; they were Physical-Functional, Cognitive, and 2 different behavioral aspects, that is, the Mood-Self domain and a distinct Worries domain. These domains clarify the behavioral sequelae as experienced by patients, and all showed good to excellent internal consistency. Known-groups analyses illustrated significant and graded changes in clinical assessments and corresponding HDQoL domains across disease severity levels. Convergent and discriminant validity was demonstrated by the expected pattern of correlations between specific HDQoL domains and corresponding domain-relevant clinical assessments as well as patient-reported measures. The data demonstrate robust support for the refined HDQoL across disease stages. CONCLUSIONS The HDQoL, with its 2 distinct behavioral domains of Mood-Self and Worries as well as the Physical-Functional and Cognitive domains, is a relevant, reliable, and valid patient-derived instrument to measure the impact of HD across all severity stages.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology

UniBE Contributor:

Burgunder, Jean-Marc

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1098-3015

Publisher:

Elsevier

Language:

English

Submitter:

Chantal Kottler

Date Deposited:

14 Nov 2019 12:37

Last Modified:

14 Nov 2019 12:37

Publisher DOI:

10.1016/j.jval.2019.01.016

PubMed ID:

31198189

Uncontrolled Keywords:

Huntington’s disease patient-reported outcome presymptomatic/preclinical Huntington’s disease quality of life questionnaire

BORIS DOI:

10.7892/boris.134820

URI:

https://boris.unibe.ch/id/eprint/134820

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