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URMC / Center for Health + Technology / Our Expertise / CHeT Outcomes / ALS-HI: The Amyotrophic Lateral Sclerosis Health Index

 

ALS-HI: The Amyotrophic Lateral Sclerosis Health Index

  • Type of Clinical Outcome Assessment (COA): Patient-reported outcome measure (PRO)
  • Administration Mode: Self-administered
  • Therapeutic Area: Amyotrophic lateral sclerosis
  • Approximate Completion Time: <15 minutes
  • Required Supervision: None
  • Recall Period: Immediate point in time

Instrument Variations

  • The Amyotrophic Lateral Sclerosis Health Index Short Form (ALS-HI-SF)
    • ALS Short From Completion Time: Under 1 minute

Symptom Subscales

  • Number of Independently Validated Symptom Subscales: 15

Fatigue; Mobility & Ambulation; Lower Extremity Function; Upper Extremity Function; Social Performance; Hand & Finger Function; Activity Participation; Emotional Health; Pain; Communication; Sleep & Daytime Sleepiness; Swallowing Function; Breathing Function; Gastrointestinal Health; Cognitive Health

Instrument Attributes

  • The ALS-HI is a patient-reported outcome (PRO) measure designed to assess symptoms and health-related quality-of-life from the perspective of patients with amyotrophic lateral sclerosis.
  • 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 ALS-HI is a fully valid, reliable, responsive, and disease-specific instrument capable of measuring changes in patient-reported health in patients with amyotrophic lateral sclerosis in response to therapeutic intervention during clinical trials or clinical monitoring.
  • The instrument was developed using extensive patient input, including an analysis of 732 patient quotes and a large cross-sectional study involving 497 patients.
  • The ALS-HI is highly relevant to patients, has low patient burden, correlates with markers of disease severity, and demonstrates no floor or ceiling effects.
  • The ALS-HI was developed in partnership with the ALS Association (ALSA), CReATe network, and the Centers for Disease Control and Prevention (CDC). 
  • The ALS-HI and its subscales demonstrated a high internal consistency (Cronbach’s α = 0.981 for full instrument) and an ability to differentiate between groups with dissimilar disease severity.
  • The ALS-HI is currently utilized in studies conducted by Industry, Academic Groups, and Foundations.
  • English

Additional translations available upon request.

  • Zizzi C, Seabury J, Rosero S, Alexandrou D, Wagner E, Weinstein JS, Varma A, Dilek N, Heatwole J, Wuu J, Caress J, Bedlack R, Granit V, Statland JM, Mehta P, Benatar M, Heatwole C. Patient reported impact of symptoms in amyotrophic lateral sclerosis (PRISM-ALS): A national, cross-sectional study. EClinicalMedicine. 2022 Dec 13;55:101768. doi: 10.1016/j.eclinm.2022.101768. PMID: 36531982; PMCID: PMC9755057.
  • Varma A, Weinstein J, Seabury J, Rosero S, Zizzi C, Alexandrou D, Wagner E, Dilek N, Heatwole J, Wuu J, Caress J, Bedlack R, Granit V, Statland J, Mehta P, Benatar M, Kaat A, Heatwole C. The amyotrophic lateral sclerosis-health index (ALS-HI): development and evaluation of a novel outcome measure. Amyotroph Lateral Scler Frontotemporal Degener. 2023 Aug;24(5-6):514-522. doi: 10.1080/21678421.2023.2204871. Epub 2023 May 15. PMID: 37190795.
  • “Development of the ALS-Health Index: Interface Between Academia, Government, and CReATe Connect.”  CReATe Therapies for ALS and Related Disorders Meeting.  Miami.  3/14/17.
  • “Development and Validation of the ALS-Health Index: A Disease-Specific, Clinically Relevant, Multifaceted, Patient Reported Outcome Measure for ALS Clinical Trials.”  CReATe Therapies for ALS Meeting.  Miami.  2/4/18.
  • “Development and Validation of the ALS-Health Index: A Disease-Specific, Clinically Relevant, Multifaceted, Patient Reported Outcome Measure for ALS Clinical Trials.”  Create Annual Meeting.  Miami, FL.  2/5/18.
  • “Patient-Reported Disease Burden in ALS:  A Summary of Qualitative Interviews.”  ALS-HI Study Update.  CREATE Annual Meeting.  Miami, FL.  3/11/19.
  • “Initial Validation of the ALS Health Index (ALS-HI), a Multi-Faceted Patient Reported Outcome Measure: Results from Qualitative Patient Interviews.” C. Zizzi, E. Wagner, S. Hussain, M. Benatar, C. Heatwole. The Muscle Study Group. Snowbird, Utah. September 2019. Muscle and Nerve. Vol 60, Number S2.
  • “What Matters Most to Patients with ALS: Initial validation of the ALS Health Index (ALS-HI), a Multi-Faceted Patient Reported Outcome Measure.” C. Zizzi, E. Wagner, S. Hussain, M. Benatar, C. Heatwole. 30th International Symposium on ALS/MND. December 2019.
  • “Results from a National Cross-Sectional Study of Disease-Burden in Amyotrophic Lateral Sclerosis (ALS): Results from a National Cross-Sectional Study.” C. Zizzi, E. Wagner, J. Weinstein, J. Seabury, N. Dilek, M. McDermott, S. Hussain, J. Wuu, J. Caress, R. Bedlack, V. Granit, J. Statland, P. Mehta, M. Benatar, C. Heatwole.  Muscle Study Group Annual Meeting.  September 2020. 
  • “The Amyotrophic Lateral Sclerosis-Health Index (ALS-HI): A Novel Outcome for Measuring How a Patient Feels and Functions in Clinical Trials.”  C. Zizzi, J. Weinstein, J. Seabury, S. Rosero, E. Wagner, N. Dilek, M. McDermott, S. Hussain,  J. Wuu, J. Caress, R. Bedlack, V. Granit,  J. Statland,  P. Mehta, M. Benatar, C. Heatwole.  NEALS Annual Meeting. September 2020. 
  • “The Amyotrophic Lateral Sclerosis Health Index (ALS-HI): A Measure of Prioritized Symptomatic Themes in ALS.” C. Zizzi; J. Weinstein, J. Seabury, S. Rosero, E. Wagner, N. Dilek, M. McDermott, S. Hussain, J. Wuu, J. Caress, R. Bedlack, V. Granit, J. Statland, P. Mehta, M. Benatar, C. Heatwole.  MNDA 31st International Symposium on ALS/MND.  12/9/20.
  •  “ALS-Health Index: A Valid and Responsive Instrument for us in ALS Clinical Trials”. CReATe Consortium Annual Meeting. 3/11/21.
  • “The Amyotrophic Lateral Sclerosis-Health Index (ALS-HI): A Novel Outcome Measure for Measuring How Patients with ALS Feel and Function During Clinical Trials.” J. Weinstein, J. Seabury, S. Rosero, A. Varma, C, Zizzi, E. Wagner, D. Alexandrou, N. Dilek, M. McDermott, J. Wuu, J. Caress, R. Bedlack, V. Granit, J. Statland, P. Mehta, J. Heatwole, M. Benatar, C. Heatwole. Northeast Amyotrophic Lateral Sclerosis (NEALS) Annual Meeting. Clearwater, Fl. 11/1/22.

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 ALS-HI score (0-100) representing overall disease burden.

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