HD-PRO-TRIAD™ Validation: A Patient-reported Instrument for the Symptom Triad of Huntington's Disease.
BACKGROUND
Few valid, disease-specific measures of health-related quality of life (HRQOL) capture the spectrum of symptoms associated with Huntington's disease (HD). The HD-PRO-TRIAD™ is a new, HD-specific, patient-reported outcome (PRO) instrument of the HD symptom triad (cognitive decline, emotional/behavioral dyscontrol, and motor dysfunction) designed for clinical research and practice. The objective was to validate the HD-PRO-TRIAD™ through a cross-sectional sample of individuals with HD and caregivers.


METHODS
Development of the HD-PRO-TRIAD™ has been described elsewhere. A total of 132 individuals with HD and 40 HD caregivers, comprising 29 dyads, participated in the cross-sectional psychometric validation of this instrument. Participants provided responses to the HD-PRO-TRIAD™ and other HRQOL and disease severity instruments (EuroQOL 5D, Short Form 12, Neuro-QOL Item Banks, PROMIS Global Health, and self-reported Unified Huntington's Disease Rating Scale Total Functional Capacity and Independence Scales). Internal consistency, construct validity, and patient-caregiver proxy consistency were evaluated.


RESULTS
Internal consistency of the three domains and overall HD-PRO-TRIAD™ instrument was supported by Cronbach's alpha values ≥0.94. Construct validity was supported by significant correlations between HD-PRO-TRIAD™ domain scores and other measures of the same domains (e.g., significant positive correlations between HD-PRO-TRIAD™ Anxiety with Neuro-QOL Anxiety), as well as slightly weaker but still strong correlations with other HRQOL instruments (e.g., HD-PRO-TRIAD™ Anxiety and UHDRS Independence; all p<0.01). Consistency between patient self-report and caregiver proxy report was supported by an intra-class correlation coefficient ≥0.92 for all three domains and the overall instrument.


CONCLUSIONS
These data indicate that HD-PRO-TRIAD™ is a reliable and valid HRQOL instrument that captures the typical triad of HD symptoms.
University of Michigan, Ann Arbor, Michigan, United States of America.
Noelle E
NE
Carlozzi
Northwestern University, Evanston, Illinois, United States of America.
David
D
Victorson
University of Alabama at Birmingham, Birmingham, Alabama, United States of America.
Victor
V
Sung
Northwestern University, Evanston, Illinois, United States of America.
Jennifer L
JL
Beaumont
Analysis Group, Inc., Boston, Massachusetts, United States of America.
Wendy
W
Cheng
Analysis Group, Inc., Boston, Massachusetts, United States of America.
Brian
B
Gorin
Analysis Group, Inc., Boston, Massachusetts, United States of America.
Mei Sheng
MS
Duh
Analysis Group, Inc., Boston, Massachusetts, United States of America.
David
D
Samuelson
New York University Langone Medical Center, New York, New York, United States of America ; Kessler Foundation, West Orange, New Jersey, United States of America.
David
D
Tulsky
Northwestern University, Evanston, Illinois, United States of America.
Sandra
S
Gutierrez
Northwestern University, Evanston, Illinois, United States of America.
Cindy J
CJ
Nowinski
Northwestern University, Evanston, Illinois, United States of America.
Allison
A
Mueller
Lundbeck LLC, Deerfield, Illinois, United States of America.
Vivienne
V
Shen
Boston University School of Medicine, Boston, Massachusetts, United States of America.
Samuel
S
Frank
HD-PRO-TRIAD™; Huntington's disease; health-related quality of life; patient-reported outcome; psychometric validation; quality-of-life scale