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. 2018 Jan;265(1):98-107.
doi: 10.1007/s00415-017-8677-7. Epub 2017 Nov 15.

A new measure for end of life planning, preparation, and preferences in Huntington disease: HDQLIFE end of life planning

Noelle E Carlozzi  1 , E A Hahn  2 , S A Frank  3 , J S Perlmutter  4   5 , N D Downing  6 , M K McCormack  7   8 , S Barton  4 , M A Nance  9 , S G Schilling  10 ; HDQLIFE Site Investigators and Coordinators
Collaborators, Affiliations

A new measure for end of life planning, preparation, and preferences in Huntington disease: HDQLIFE end of life planning

Noelle E Carlozzi et al. J Neurol. 2018 Jan.

Abstract

Background: Huntington disease is a fatal inherited neurodegenerative disease. Because the end result of Huntington disease is death due to Huntington disease-related causes, there is a need for better understanding and caring for individuals at their end of life.

Aim: The purpose of this study was to develop a new measure to evaluate end of life planning.

Design: We conducted qualitative focus groups, solicited expert input, and completed a literature review to develop a 16-item measure to evaluate important aspects of end of life planning for Huntington disease. Item response theory and differential item functioning analyses were utilized to examine the psychometric properties of items; exploratory factor analysis was used to establish meaningful subscales.

Participants: Participants included 508 individuals with pre-manifest or manifest Huntington disease.

Results: Item response theory supported the retention of all 16 items on the huntington disease quality of life ("HDQLIFE") end of life planning measure. Exploratory factor analysis supported a four-factor structure: legal planning, financial planning, preferences for hospice care, and preferences for conditions (locations, surroundings, etc.) at the time of death. Although a handful of items exhibited some evidence of differential item functioning, these items were retained due to their relevant clinical content. The final 16-item scale includes an overall total score and four subscale scores that reflect the different end of life planning constructs.

Conclusions: The 16-item HDQLIFE end of life planning measure demonstrates adequate psychometric properties; it may be a useful tool for clinicians to clarify patients' preferences about end of life care.

Keywords: End of life; HDQLIFE; HDQLIFE Site Investigators and Coordinators; Health-related quality of life; Huntington disease; Patient-reported outcome (PRO).

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Conflict of interest statement

CONFLICTS OF INTEREST:

Carlozzi, N.E. currently has research grants from the NIH; she is also supported by grant funding from the NIH and CHDI. She provides patient reported outcome measurement selection and application consultation for Teva Pharmaceuticals. She declares no conflicts of interest.

Hahn, E.A. currently has research grants from the NIH; she is also supported by grant funding from the NIH and PCORI, and by research contracts from Merck and EMMES; she declares no conflicts of interest.

Frank, S. receives salary support from the Huntington Study Group for a study sponsored by Auspex Pharmaceuticals. There is no conflict of interest.

Perlmutter, J.S. currently has funding from the NIH, HDSA, CHDI, and APDA. He has received honoraria from the University of Rochester, American Academy of Neurology, Movement Disorders Society, Toronto Western Hospital, St. Luke’s Hospital in St Louis, Emory University, Penn State, Alberta innovates, Indiana Neurological Society, Parkinson Disease Foundation, Columbia University, St. Louis University, Harvard University and the University of Michigan; he declares no conflicts of interest.

Downing, N.R. declares no conflicts of interest.

McCormack, M.K. currently has grants from the NJ Department of Health; he declares no conflicts of interest.

Barton, S.K. is supported by grant funding from the Huntington’s Disease Society of America, CHDI Foundation and the NIH. She declares no conflicts of interest.

Nance, M.A. declares no conflicts of interest.

Schilling, S.G. has a research grant from NSF. He also is supported by grant funding from NIH. He declares no conflicts of interest.

Figures

Figure 1
Figure 1. HDQLIFE End of Life Planning and Preparation Short Form
Caption: In general, we want total information to be > 9.0 and standard error to be < 0.33 (this provides a reliability of 0.9). This figure shows excellent total information and standard error for End of Life Planning and Preparation Short Form scale scores between −1.5 and 1.0.
See this image and copyright information in PMC

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