ASSESSING THE RELEVANCE OF HEALTH-RELATED QUALITY OF LIFE INSTRUMENTS AND PREFERENCE-BASED MEASURES FOR UTILITY GENERATION IN EARLY HD

Author(s)

Katharina Buesch, PhD1, Charlie Smith, PhD2, Nicholas Adlard, MA, MBA, MSc2.
1Consultant, KJM Büsch Consulting GmbH, Zug, Switzerland, 2Novartis Pharma, Basel, Switzerland.
OBJECTIVES: Huntington’s disease (HD) is a progressive neurodegenerative disorder with significant impact on health-related quality of life (HRQoL). Preference-based measures (PBMs) such as EQ-5D are commonly recommended by HTA bodies for utility generation, but their ability in measuring the impact of early-stage HD, prior to functional loss (HD-ISS Stage 2) remains unclear. To evaluate the appropriateness of generic PBMs and HRQoL instruments for utility estimation in early-stage HD by mapping their domains to key disease features identified in the literature.
METHODS: Drivers of HRQoL in early-stage HD and evidence on generic PBMs/HRQoL instruments used in HD were identified through targeted literature reviews. Concept mapping was conducted by two independent reviewers to assess whether relevant HRQoL drivers were captured by each instrument’s dimensions. A gap analysis was undertaken to evaluate the coverage of primary signs and symptoms of early-stage HD.
RESULTS: Currently, utilities reported for HD are primarily stratified by Total Functional Capacity (TFC) stages and not for early-stage HD i.e. prior functional loss. While the EQ-5D partially captures anxiety and depression, it omits key aspects such as cognitive or nuanced mood changes and upper limb motor impacts. The SF-6D includes emotional and mental health domains but lacks coverage of cognition and fine motor control. Both the SF-12 and SF-36 have been applied in HD; with the SF-36 offering broader domain coverage and greater sensitivity to subtle changes in physical and mental health. No generic instrument fully captured cognitive and emotional symptoms that are hallmark features of early-stage HD.
CONCLUSIONS: Generic PBMs are unlikely to capture treatment-related HRQoL changes adequately in early-stage HD. The SF-36 offers the most comprehensive domain coverage among the generic instruments, though it still lacks important disease-specific symptoms. Further evidence generation, including vignette-based utility elicitation, is warranted to be able to capture HRQoL impacts in early-stage HD.

Conference/Value in Health Info

2026-05, ISPOR 2026, Philadelphia, PA, USA

Value in Health, Volume 29, Issue S6

Code

PCR192

Topic

Patient-Centered Research

Topic Subcategory

Health State Utilities, Patient-reported Outcomes & Quality of Life Outcomes

Disease

No Additional Disease & Conditions/Specialized Treatment Areas, SDC: Rare & Orphan Diseases

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