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URMC / Center for Health + Technology / Our Expertise / CHeT Outcomes / FA-HI: The Friedreich’s Ataxia Health Index

 

FA-HI: The Friedreich’s Ataxia Health Index

  • Type of Clinical Outcome Assessment (COA): Patient-reported outcome measure (PRO) and Caregiver or observer-reported outcome (CR/ObsRO)
  • Administration Mode: Self-administered
  • Therapeutic Area: Friedreich’s ataxia
  • Approximate Completion Time: 15 minutes
  • Required Supervision: None
  • Recall Period: Immediate point in time

Instrument Variations

  • The Friedreich’s Ataxia Health Index Short Form (FA-HI-SF)
    • FA-HI Short Form Completion Time: Under 1 minute
  • The Friedreich’s Ataxia Caregiver Reported Health Index (FACR-HI)  
    • FACR-HI Completion Time: 8 minutes (completed by a caregiver on behalf of a patient)
  • The Friedreich’s Ataxia Caregiver Reported Health Index Short Form (FACR-HI-SF)
    • FACR-HI Short Form Completion Time: Under 1 minute (completed by a caregiver on behalf of a patient)

Symptom Subscales

  • FA-HI & FACR-HI Number of Independently Validated Symptom Subscales: 18

Mobility & Ambulation; Coordination; Fatigue; Lower Extremity Strength; Social Performance; Activity Participation; Emotional Health; Shoulder & Arm Function; Hand & Finger Function; Communication; Sleep & Daytime Sleepiness; Pain; Sensation; Vision; Gastrointestinal Function; Swallowing Function; Heart Health; Breathing Function

Instrument Attributes

  • The FA-HI is a patient-reported outcome (PRO) measure designed to assess symptoms and health-related quality-of-life from the perspective of patients with Friedreich’s ataxia.
  • Designed and validated to fully satisfy published FDA guidance for use in drug-labeling claims and measure how a patient feels and functions.
  • The FA-HI is a fully valid, reliable, responsive, and disease-specific instrument capable of measuring changes in patient-reported health in patients with Friedreich’s ataxia in response to therapeutic intervention during clinical trials or clinical monitoring.
  • The FA-HI was developed using extensive patient input, including an analysis of 1,152 adult and 571 minor patient quotes and a large cross-sectional study involving 153 patients.
  • The FA-HI is highly relevant to patients, has low patient burden, correlates with markers of disease severity, and demonstrates no floor or ceiling effects.
  • Utilized by academic and industry groups in multiple longitudinal trials.
  • The FACR-HI is a caregiver-reported outcome (CRO/ObsRO) measures designed to assess the disease burden of a patient from the perspective of the caregiver.
  • The FACR-HI was developed using extensive caregiver and patient input, including an analysis of 805 caregiver quotes and a large cross-sectional study involving 49 caregivers.
  • The FACR-HI is highly relevant to patients using the perspective of the caregiver, has low patient burden, correlates with markers of disease severity, and demonstrates no floor or ceiling effects.
  • English
  • English (NZ)
  • English (UK)
  • Czech
  • Dutch (NL)
  • French (CA)
  • French (FR) 
  • German (GE)
  • Greek
  • Hindi
  • Italian
  • Portuguese (BR)
  • Slovak
  • Spanish (Latin America)
  • Spanish (SP)

Additional translations available upon request.

  • Seabury J, Alexandrou D, Dilek N, Cohen B, Heatwole J, Larkindale J, Lynch DR, Park C, Rosero S, Subramony SH, Varma A, Wagner E, Walther S, Weinstein J, Wells M, Zizzi C, Heatwole C. Patient-Reported Impact of Symptoms in Friedreich Ataxia. Neurology. 2023 Feb 21;100(8):e808-e821. doi: 10.1212/WNL.0000000000201598. Epub 2022 Nov 28. PMID: 36443012; PMCID: PMC9984224.
  • Seabury J, Rosero S, Varma A, Weinstein J, Engebrecht C, Dilek N, Heatwole J, Alexandrou D, Cohen B, Larkindale J, Lynch DR, Park C, Subramony SH, Wagner E, Walther S, Wells M, Zizzi C, Heatwole C. Friedreich's Ataxia-Health Index: Development and Validation of a Novel Disease-Specific Patient-Reported Outcome Measure. Neurol Clin Pract. 2023 Oct;13(5):e200180. doi: 10.1212/CPJ.0000000000200180. Epub 2023 Aug 28. PMID: 37646046; PMCID: PMC10462051.
  • “The Identification of the Most Important Friedreich’s Ataxia Symptoms:  Results form a Patient and Caregiver Study.”  C. Zizzi, E. Wagner, B. Greco, J. Larkindale, L. Corben, S. Subramony, C. Heatwole.  International Ataxia Research Conference.  Washington DC.  11/15/19.
  • “Identification of the Most Important Friedreich’s Ataxia Symptoms: The First Step in Developing the Friedreich’s Ataxia Health Index (FA-HI).”  FARA 2020 Biomarker and Clinical Endpoint Meeting.  9/24/20.
  • “Development of Novel Outcome Measures for Use in Friedreich’s Ataxia Clinical Trials”. J. Seabury, J. Weinstein, S. Rosero, C. Zizzi, C. Heatwole. Friedreich’s Ataxia Research Alliance Flash Talk Series. Virtual. 5/27/21.
  • “Development & Validation of the Patient-Reported and Caregiver-Reported FA-Health Indices.” A. Varma, J. Seabury, J. Weinstein, S. Rosero, J. Larkindale, S. Walther, E. Wagner, B. Greco, N. Dilek, J. Heatwole, Z. Rose, C. Zizzi, D. Lynch, C. Park, M. Wells, S. Subramony, C. Heatwole. 2022 Friedreich’s Ataxia Research Alliance (FARA) Flash Talk Series. Virtual. 5/19/22.
  • “The FA-HI & FACR-HI: Development and Validation of Two Novel Friedreich’s Ataxia Outcome Measures.” S. Rosero, J. Larkindale, S. Walther, J. Seabury, A. Varma, E. Wagner, J. Weinstein, N. Dilek, J. Heatwole, Z. Rose, C. Zizzi, D. Lynch, C. Park, M. Wells, C. Heatwole. 2022 International Congress for Ataxia Research. Dallas, TX. 11/01/22.
  • “Development & Validation of Two Novel Disease-Specific Outcome Measures: The FA-HI and FACR-HI”.  C. Engebrecht, A. Varma, J. Seabury, J. Weinstein, S. Rosero, J. Larkindale, S. Walther, E. Wagner, B. Greco, N. Dilek, J. Heatwole, S. Khosa, Z. Rose, C. Zizzi, D. Lynch, C. Park, M. Wells, S. Subramony, C. Heatwole. Oral session presented at: 2023 Friedreich’s Ataxia Research Alliance (FARA) Flash Talk Series; May 25, 2023; virtual.

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 FA-HI or FACR-HI score (0-100) representing overall disease burden.

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