Developing neuropalliative care for sporadic Creutzfeldt-Jakob Disease

ABSTRACT

We aimed to identify targets for neuropalliative care interventions in sporadic Creutzfeldt-Jakob disease by examining characteristics of patients and sources of distress and support among former caregivers. We identified caregivers of decedents with sporadic Creutzfeldt-Jakob disease from the University of California San Francisco Rapidly Progressive Dementia research database. We purposively recruited 12 caregivers for in-depth interviews and extracted associated patient data. We analysed interviews using the constant comparison method and chart data using descriptive statistics. Patients had a median age of 70 (range: 60–86) years and disease duration of 14.5 months (range 4–41 months). Caregivers were interviewed a median of 22  (range 11–39) months after patient death and had a median age of 59 (range 45–73) years. Three major sources of distress included (1) the unique nature of sporadic Creutzfeldt-Jakob disease; (2) clinical care issues such as difficult diagnostic process, lack of expertise in sporadic Creutzfeldt-Jakob disease, gaps in clinical systems, and difficulties with end-of-life care; and (3) caregiving issues, including escalating responsibilities, intensifying stress, declining caregiver well-being, and care needs surpassing resources. Two sources of support were (1) clinical care, including guidance from providers about what to expect and supportive relationships; and (2) caregiving supports, including connection to persons with experience managing Creutzfeldt-Jakob disease, instrumental support, and social/emotional support. The challenges and supports described by caregivers align with neuropalliative approaches and can be used to develop interventions to address needs of persons with sporadic Creutzfeldt-Jakob disease and their caregivers.

KEYWORDS:

• Sporadic Creutzfeldt-Jakob
• palliative
• qualitative
• mixed methods
• caregiver

Acknowledgments

The authors would like to thank the patients, caregivers, and their families for participating in our research; referring physicians; the U.S. National Prion Disease Pathology Surveillance Center (NPDPSC) for PRNP analyses, prion typing and pathological analyses; the U.S. CJD Foundation (for supporting our patients and families). The authors thank Aili Golubjatnikov and Megan Casey for their assistance in identifying potential caregivers, and Nicole Boyd and Madina Halim for administrative help. All analyses, interpretations and conclusions reached through this project are the sole responsibility of the authors.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Author contributions

• KLH: study conceptualization/design, data analysis and interpretation, drafting/revising manuscript for content, obtaining funding

• SBG: study conceptualization/design, data acquisition, data interpretation and analysis, drafting/revising manuscript for content

• ABS: study conceptualization/design, data interpretation and analysis, drafting/revising manuscript for content

• JG: data acquisition, data interpretation/analysis, drafting/revising manuscript for content

• MJT: data acquisition, data interpretation/analysis, revising manuscript for content

• CSR: study conceptualization/design, data interpretation and analysis, drafting/revising manuscript for content

• MDG: study conceptualization/design, data acquisition, data interpretation and analysis, drafting/revising manuscript for content

Sources of Support

Research reported in this publication was the Global Brain Health Institute (GBHI), Alzheimer’s Association, and Alzheimer’s Society Pilot Awards for Global Brain Health Leaders, as well as the National Institutes of Health (NIH), National Institute on Aging (NIA) grants R01-AG031189 (MDG), R56 AG055619 (MDG) and R01 AG062562 (MDG) and the Michael J. Homer Family Fund (MDG).

Additional funding supporting investigators’ time included the Atlantic Fellowship for Equity in Brain Health (KLH, ABS, JG), AHRQ T32HS022241(SBG), Maurange Fund, King Baudouin Foundation (JG), NIH/NIA K01AG059831 (KLH) and K01AG059840 (ABS), and California State Alzheimer’s Disease Program Grant 19-10,615 (ABS).

Supplementary material

Supplemental data for this article can be accessed here

Additional information

Funding

This work was supported by the Global Brain Health Institute, the Alzheimer's Association, and Alzheimer’s Society [no award number], the National Institute on Aging [R01-AG031189]; National Institute on Aging [R01 AG062562]; National Institute on Aging [R56 AG055619]; and Michael J. Homer Family Fund [no award/grant number].