Identifying Motor, Emotional-Behavioral, and Cognitive Deficits that Comprise the Triad of HD Symptoms from Patient, Caregiver, and Provider Perspectives.
BACKGROUND
The objective of this study was to identify important attributes associated with the triad of symptoms (cognition, emotional-behavioral, and motor) of Huntington's disease (HD) from patient, caregiver, and medical provider perspectives to facilitate development of a new disease-specific, health-related quality of life (HRQOL) instrument.


METHODS
We conducted a targeted literature review of HD and HRQOL instruments, expert surveys, and patient and caregiver phone-based interviews to extract information on the symptoms and issues most relevant to the HD symptom triad (HD triad). The data collected from these sources were used to generate themes and subdomains and to develop an integrated schema that highlights the key dimensions of the triad.


RESULTS
THE SEARCH IDENTIFIED THE FOLLOWING AREAS: emotional functioning/behavioral changes (e.g., positive emotions, sadness/depression); cognitive functioning (e.g., memory/learning, attention/comprehension); physical functioning (e.g., motor functioning, medication); social functioning (e.g., leisure, interpersonal relationships); end-of-life concerns/planning; and gene testing. Fifteen individuals diagnosed with HD and 16 HD caregivers, recruited from several Huntington's Disease Society of America support group networks, completed phone interviews. Nineteen US medical providers who specialize in HD completed the online survey. Twenty-six subdomains of the HD symptom triad (seven cognition, 12 emotional-behavioral, and seven motor) emerged relatively consistently across patient, caregiver, and provider samples. These included movements/chorea, memory impairment, depression, and anxiety.


CONCLUSIONS
Based on an integrated, mixed-methods approach, important HD triad symptom were identified and organized into a guiding schema. These patient-, caregiver-, and provider-triangulated data served as the basis for development of a HD-specific HRQOL instrument, the HD-PRO-TRIAD™.
Northwestern University, Evanston, Illinois, United States of America.
David
D
Victorson
University of Michigan, Ann Arbor, Michigan, United States of America.
Noelle E
NE
Carlozzi
Boston University School of Medicine, Boston, Massachusetts, United States of America.
Samuel
S
Frank
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
University of Alabama at Birmingham, Birmingham, Alabama, United States of America.
Victor
V
Sung
HD-PRO-TRIAD™; Huntington's disease; health-related quality of life; patient-reported outcome; psychometric validation; quality-of-life scale