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

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Abstract

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.

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Acknowledgements

Work on this manuscript was supported by the National Institutes of Health (NIH), National Institute of Neurological Disorders and Stroke (R01NS077946) and the National Center for Advancing Translational Sciences (UL1TR000433). In addition, a portion of this study sample was collected in conjunction with the Predict-HD study. The Predict-HD study was supported by the NIH, National Institute of Neurological Disorders and Stroke (R01NS040068), the NIH, Center for Inherited Disease Research (provided supported for sample phenotyping), and the CHDI Foundation (award to the University of Iowa). We thank the University of Iowa, the Investigators and Coordinators of this study, the study participants, the National Research Roster for Huntington Disease Patients and Families, the Huntington Study Group, and the Huntington’s Disease Society of America. We acknowledge the assistance of Jeffrey D. Long, Hans J. Johnson, Jeremy H. Bockholt, Roland Zschiegner, and Jane S. Paulsen. We also acknowledge Roger Albin, Kelvin Chou, and Henry Paulsen for the assistance with participant recruitment. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH. HDQLIFE Site Investigators and Coordinators: Noelle Carlozzi, Praveen Dayalu, Stephen Schilling, Amy Austin, Matthew Canter, Siera Goodnight, Jennifer Miner, Nicholas Migliore (University of Michigan, Ann Arbor, MI); Jane Paulsen, Nancy Downing, Isabella DeSoriano, Courtney Shadrick, Amanda Miller (University of Iowa, Iowa City, IA); Kimberly Quaid, Melissa Wesson (Indiana University, Indianapolis, IN); Christopher Ross, Gregory Churchill, Mary Jane Ong (Johns Hopkins University, Baltimore, MD); Susan Perlman, Brian Clemente, Aaron Fisher, Gloria Obialisi, Michael Rosco (University of California Los Angeles, Los Angeles, CA); Michael McCormack, Humberto Marin, Allison Dicke, judy Rokeach (Rutgers University, Piscataway, NJ); Joel S. Perlmutter, Stacey Barton, Shineeka Smith (Washington University, St. Louis, MO); Martha Nance, Pat Ede (Struthers Parkinson’s Center); Stephen Rao, Anwar Ahmed, Michael Lengen, Lyla Mourany, Christine Reece (Cleveland Clinic Foundation, Cleveland, OH); Michael Geschwind, Joseph Winer (University of California – San Francisco, San Francisco, CA), David Cella, Richard Gershon, Elizabeth Hahn, Jin-Shei Lai (Northwestern University, Chicago, IL).

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Authors and Affiliations

  1. Department of Physical Medicine and Rehabilitation, University of Michigan, North Campus Research Complex, 2800 Plymouth Road, Building NCRC B14, Room G216, Ann Arbor, MI, 8109-2800, USA

    Noelle E. Carlozzi & Nicholas R. Boileau

  2. Department of Psychiatry, Carver College of Medicine, The University of Iowa, Iowa City, IA, USA

    Jane S. Paulsen

  3. Department of Neurology, Carver College of Medicine, The University of Iowa, Iowa City, IA, USA

    Jane S. Paulsen

  4. Department of Psychological and Brain Sciences, The University of Iowa, Iowa City, IA, USA

    Jane S. Paulsen

  5. Neurology, Radiology, Neuroscience, Physical Therapy, Occupational Therapy, Washington University in St. Louis, St. Louis, MO, USA

    Joel S. Perlmutter & Stacey K. Barton

  6. Department of Medical Social Sciences, Northwestern University, Chicago, IL, USA

    Jin-Shei Lai, Elizabeth A. Hahn & David Cella

  7. Department of Pathology, Rowan University—SOM, Stratford, NJ, USA

    Michael K. McCormack

  8. Department of Psychiatry, Rutgers University, RWJMS, Piscataway, NJ, USA

    Michael K. McCormack

  9. Struthers Parkinson’s Center, Golden Valley, MN, USA

    Martha A. Nance

  10. Hennepin County Medical Center, Minneapolis, MN, USA

    Martha A. Nance

  11. Department of Preventive Medicine, Northwestern University, Chicago, IL, USA

    David Cella

  12. Northwestern University, Evanston, IL, USA

    David Cella

  13. College of Nursing, Texas A&M University, Bryan, TX, USA

    Nancy R. Downing

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  1. Noelle E. Carlozzi
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  2. Nicholas R. Boileau
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  3. Jane S. Paulsen
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  9. David Cella
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Corresponding author

Correspondence to Noelle E. Carlozzi.

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Conflicts of interest

Carlozzi, N.E. is supported by grant funding from the NIH, the Neilsen Foundation, and CHDI, as well as a government contract from the Center for Medicaid and Medicare Services. She has previously provided patient-reported outcome measurement selection and application consultation for Teva Pharmaceuticals. She declares no conflicts of interest. Boileau, N.R. is supported by grant funding from the NIH, the Neilsen Foundation and CHDI. He declares no conflicts of interest. Paulsen, J.S. currently has research grants from the NIH; she is also supported by grant funding from NIH, NINDS, and CHDI; she declares no conflicts 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. Lukes Hospital in St Louis, Emory U, 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. Lai J.-S. currently has research grants from the NIH; 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. McCormack, M.K. currently has grants from the NJ Department of Health; he declares no conflicts of interest. Nance, M.A. declares no conflicts of interest. Cella, D. receives grant funding from the National Institutes of Health and reports that he has 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. Downing, N.R. is supported by grant funding from the Health Resources and Services Administration and declares no conflicts of interest.

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All data has been collected in accordance with local institutional review boards.

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Study participants provided informed consent prior to the participation in study activities.

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Carlozzi, N.E., Boileau, N.R., Paulsen, J.S. et al. End-of-life measures in Huntington disease: HDQLIFE Meaning and Purpose, Concern with Death and Dying, and End of Life Planning. J Neurol 266, 2406–2422 (2019). https://doi.org/10.1007/s00415-019-09417-7

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  • DOI: https://doi.org/10.1007/s00415-019-09417-7

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