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

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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.

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Acknowledgments

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 data were supported by the NIH, National Institute of Neurological Disorders and Stroke (R01NS040068), the NIH, Center for Inherited Disease Research (provided support 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 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 (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).

Author information

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, 48109-2800, USA

    Noelle E. Carlozzi

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

    E. A. Hahn

  3. Beth Israel Deaconess Medical Center, Boston, MA, USA

    S. A. Frank

  4. Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA

    J. S. Perlmutter & S. Barton

  5. Departments of Radiology and Neuroscience, Program in Occupational Therapy and Program in Physical Therapy, Washington University, St. Louis, MO, USA

    J. S. Perlmutter

  6. Forensic Health Care College of Nursing, Texas A&M University, College Station, TX, USA

    N. D. Downing

  7. Department of Psychiatry, Rutgers University-Robert Wood Johnson Medical School, Brunswick, NJ, USA

    M. K. McCormack

  8. Piscataway and Department of Pathology, Rowan University-SOM, Stratford, NJ, USA

    M. K. McCormack

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

    M. A. Nance

  10. Institute for Social Research, University of Michigan, Ann Arbor, MI, USA

    S. G. Schilling

Authors
  1. Noelle E. Carlozzi
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  2. E. A. Hahn
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  3. S. A. Frank
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  4. J. S. Perlmutter
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  5. N. D. Downing
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  6. M. K. McCormack
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  7. S. Barton
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  8. M. A. Nance
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  9. S. G. Schilling
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Consortia

HDQLIFE Site Investigators and Coordinators

  • Noelle Carlozzi
  • , Praveen Dayalu
  • , Stephen Schilling
  • , Amy Austin
  • , Matthew Canter
  • , Siera Goodnight
  • , Jennifer Miner
  • , Nicholas Migliore
  • , Jane Paulsen
  • , Nancy Downing
  • , Isabella DeSoriano
  • , Courtney Shadrick
  • , Amanda Miller
  • , Kimberly Quaid
  • , Melissa Wesson
  • , Christopher Ross
  • , Gregory Churchill
  • , Mary Jane Ong
  • , Susan Perlman
  • , Brian Clemente
  • , Aaron Fisher
  • , Gloria Obialisi
  • , Michael Rosco
  • , Michael McCormack
  • , Humberto Marin
  • , Allison Dicke
  • , Joel S. Perlmutter
  • , Stacey Barton
  • , Shineeka Smith
  • , Martha Nance
  • , Pat Ede
  • , Stephen Rao
  • , Anwar Ahmed
  • , Michael Lengen
  • , Lyla Mourany
  • , Christine Reece
  • , Michael Geschwind
  • , Joseph Winer
  • , David Cella
  • , Richard Gershon
  • , Elizabeth Hahn
  •  & Jin-Shei Lai

Corresponding author

Correspondence to Noelle E. Carlozzi.

Ethics declarations

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 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.

Appendix

Appendix

HDQLIFE end of life planning

1. Advance directive

0 = I have not thought about getting an advance directive

1 = I have thought about getting an advance directive

2 = I have taken steps to obtain an advance directive

3 = I have an advance directive

2. Health care power of attorney

0 = I have not thought to getting a health care power of attorney

1 = I thought about getting a health care power of attorney

2 = I have taken steps to identify a health care power of attorney

3 = I have a health care power of attorney

3. Nursing home care

0 = I have not thought about living in a nursing home

1 = I have thought about the type of nursing home I would like to go to

2 = I have taken steps to arrange nursing home care

3 = I have established nursing home care

4. Location of death preference

0 = I have not thought about where I would like to die (i.e., at home, in the hospital)

1 = I have thought about where I would like to die (i.e., at home, in the hospital)

2 = I have taken steps to arrange where I would like to die (i.e., at home, in the hospital)

3 = I have identified a location where I would like to die (i.e., at home, in the hospital)

5. Conversations about death and dying

0 = I have not thought about starting a conversation about death with my friends, family or members in the community (e.g., church/synagogue)

1 = I have thought about starting a conversation about death with my friends, family or members in the community (e.g., church/synagogue)

2 = I have taken steps to start a conversation about death with my friends, family or members in the community (e.g., church/synagogue)

3 = I have has a conversation about death with my friends, family or members in the community (e.g., church/synagogue)

6. Living will

0 = I not thought about getting a living will

1 = I have thought about getting a living will

2 = I have taken steps to prepare a living will

3 = I have a living will

7. Life insurance

0 = I have not thought about getting life insurance

1 = I have thought about getting life insurance

2 = I have taken steps to get life insurance

3 = I have life insurance

8. Palliative care (management of pain, symptoms and stress)

0 = I have not thought about palliative care

1 = I have thought about palliative care

2 = I have taken steps to arrange palliative care

3 = I am receiving palliative care

9. Child care planning

Not applicable

0 = I have not thought about the care of my children if I become unable to care for them

1 = I have thought about the care of my children if I become unable to care for them

2 = I have taken steps to arrange care for my children if I become unable to care for them

3 = My children are receiving care from others because I am no longer able to care for them

10. Finances

0 = I not thought about the necessary financial resources for my long-term care

1 = I have thought about the necessary financial resources for my long-term care

2 = I have taken steps to arrange for the necessary financial resources for my long-term care

3 = I have the necessary financial resources for my long-term care

11. Estate planning

0 = I have not thought about what will happen to my estate after my death

1 = I have thought about what will happen to my estate after my death

2 = I have made plans for my estate after my death

12. Support to make decisions

0 = I have not thought about who will help me make decisions (e.g., financial, health/medical) if I am unable to

1 = I have thought about who will help me make decisions (e.g., financial, health/medical) if I am unable to

2 = I have arranged for someone to help me make decisions (e.g., financial, health/medical) if I am unable to

13. Hospice care

0 = I have not thought about hospice care

1 = I have thought about hospice care

2 = I have taken steps to arrange hospice care

3 = I am receiving hospice care

14. Rescusitation preference

0 = I have not thought about my preference for resuscitation if I stop breathing

1 = I have thought about my desires for resuscitation if I stop breathing

2 = I have made my desires about resuscitation clear to others if I stop breathing

15. Funeral arrangements

0 = I have not thought about what others should do with my body after I die (e.g., burial, cremation)

1 = I have thought about what others should do with my body after I die (e.g., burial, cremation)

2 = I have made arrangements about what others should do with my body after I die (e.g., burial, cremation)

16. Preference about death

0 = I have not thought about how I would like to die

1 = I have thought about how I would like to die

2 = I have told others of my preferences regarding how I would like to die

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Carlozzi, N.E., Hahn, E.A., Frank, S.A. et al. A new measure for end of life planning, preparation, and preferences in Huntington disease: HDQLIFE end of life planning. J Neurol 265, 98–107 (2018). https://doi.org/10.1007/s00415-017-8677-7

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