End-of-life measures in Huntington disease: HDQLIFE Meaning and Purpose, Concern with Death and Dying, and End of Life Planning

• Published in: Journal of neurology Vol. 266; no. 10; pp. 2406 - 2422
• Main Authors: Carlozzi, Noelle E., Boileau, Nicholas R., Paulsen, Jane S., Perlmutter, Joel S., Lai, Jin-Shei, Hahn, Elizabeth A., McCormack, Michael K., Nance, Martha A., Cella, David, Barton, Stacey K., Downing, Nancy R.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.10.2019; Springer Nature B.V
• Subjects: Adult; Advance Care Planning; Aged; Attitude to Death; Death & dying; Female; Follow-Up Studies; Humans; Huntington Disease - psychology; Huntington Disease - therapy; Huntington's disease; Huntingtons disease; Male; Medicine; Medicine & Public Health; Middle Aged; Neurodegenerative diseases; Neurology; Neuroradiology; Neurosciences; Original Communication; Psychometrics - instrumentation; Psychometrics - standards; Quality of Life; Reproducibility of Results; Terminal Care; Validation studies; Validity; Validity; Huntingon disease; HDQLIFE; End of life; Reliability
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-019-09417-7

Background and purpose Huntington disease (HD) is a progressive neurodegenerative disorder. There are no HD-specific measures to assess for end-of-life (EOL) preferences that have been validated for clinical use. The purpose of this study is to demonstrate reliability and validity of three HD-specific EOL measures for use in and clinical research settings. Methods We examined internal reliability, test–retest reliability, floor and ceiling effects, convergent and discriminant validity, known groups’ validity, measurement error, and change over time to systematically examine reliability and validity of the HDQLIFE EOL measures. Results Internal consistency and test–retest reliability were > 0.70. The measures were generally free of floor and ceiling effects and measurement error was minimal. Convergent and discriminant validity were consistent with well-known constructs in the field. Hypotheses for known groups validity were partially supported (there were generally group differences for the EOL planning measures, but not for meaning and purpose or concern with death and dying). Measurement error was acceptable and there were minimal changes over time across the EOL measures. Conclusions Results support the clinical utility of the HDQLIFE EOL measures in persons with HD.