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Center for Health + Technology [CHeT]

URMC / Center for Health + Technology / Our Expertise / CHeT Outcomes / HD-HI: The Huntington’s Disease Health Index

 

HD-HI: The Huntington’s Disease Health Index

  • Type of Clinical Outcome Assessment (COA): Patient-reported outcome measure (PRO)
  • Administration Mode: Self-administered
  • Therapeutic Area: Huntington’s disease
  • Approximate Completion Time: 17-22 minutes
  • Required Supervision: None
  • Recall Period: Immediate point in time

Instrument Variations

  • The Huntington’s Disease Health Index Short Form (HD-HI-SF)
    • HD-HI Short From Completion Time: Under 1 minute

Symptom Subscales

  • Number of Independently Validated Symptom Subscales: 13

Emotional Health; Cognition; Mobility & Ambulation; Activity Participation; Social Performance; Hand & Arm Function; Fatigue; Abnormal Movements; Social Satisfaction; Daytime Sleepiness; Pain; Communication; Gastrointestinal Health

Instrument Attributes

  • The HD-HI is a patient-reported outcome (PRO) measure designed to assess symptoms and health-related quality-of-life from the perspective of patients with Huntington’s disease.
  • Designed and validated to fully satisfy regulatory and published FDA guidance for use in drug-labeling claims and measuring changes in how a patient feels and functions.
  • The HD-HI is a fully valid, reliable, responsive, and disease-specific instrument capable of measuring changes in patient-reported health in patients with Huntington’s disease.
  • The instrument was developed using extensive patient input, including an analysis of 2,082 patient quotes and a large cross-sectional study involving 389 patients and caregivers.
  • The HD-HI is highly relevant to patients, has low patient burden, correlates with markers of disease severity, and demonstrates low floor and no ceiling effects.
  • The HD-HI has been shown, through known groups analysis with 201 patients, to accurately differentiate between patients with high vs. low total functional capacity (TFC) score, prodromal vs. manifest HD, and normal ambulation vs. mobility impairment, and it has high sensitivity and specificity, as revealed by Area Under the Curve (AUC) analysis.
  • Used in industry and foundation sponsored longitudinal trials.
  • The HD-HI was integral in the FDA approval of Valbenazine for the treatment of chorea during Neurocrine Biosciences’ KINECT-HD Trial.
  • English

Additional translations available upon request.

  • “The Huntington Disease Health Index Study (HD-HI).”  A. Glidden, E. Luebbe, M. Elson, S. Goldenthal, C. Snyder, C. Zizzi, O. Brumfield; R. Dorsey, C. Heatwole.  The Huntington Study Group Meeting.  Sacramento, CA.  11/8/19
  • “The Huntington’s Disease-Health Index (HD-HI): A Novel, Clinically Relevant, Disease-Specific Patient-Reported Outcome Measure for HD.” A. Varma, J. Seabury, S. Rosero, J. Weinstein, O. Brumfield, C. Zizzi, N. Dilek, D. Alexandrou, A. Glidden, A. Kaat, M. McDermott, R. Dorsey, C. Heatwole. Huntington Study Group (HSG) 2022 Annual Meeting. Tampa, FL. 11/03/22.
  • “Valbenazine Decreases Disease Burden in Patients with Chorea Associated with Huntington Disease: Patient-Reported Huntington Disease Health Index (HD-HI) Results From the KINECT-HD Trial.” C. Heatwole, E. Furr Stiming, D. Claassen, E. Kayson, J. Goldstein, O. Klepitskaya, G. Liang, D. Haubenberger. American Academy of Neurology (AAN) Annual Meeting. Boston, MA. 4/22/23.
  • “The Huntington Disease-Health Index (HD-HI): Measuring Changes in Disease Burden in Response to Valbenazine During the KINECT-HD Trial.” C. Heatwole, E. R. Stimming, D. O. Claassen, E. Kayson, J. Goldstein, H. Zhang, O. Klepitskaya, G. Liang, D. Haubenberger, on behalf of the Huntington Study Group KINECT-HD Investigators and Coordinators. International Association of Parkinsonism and Related Disorders (IAPRD) XXVIII World Congress. Chicago, IL. 5/14/2023.

Instrument Scoring

All subscales are scored on a scale of 0 to 100 with 0 representing no disease burden and 100 representing the maximum level of disease burden. Symptom questions within each subscale are weighted based on participant-reported prevalence and average impact as identified through the cross-sectional study. Subscale scores are also weighted to generate a total HD-HI score (0-100) representing overall disease burden.

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