RESEARCH PAPER
Developing neuropalliative care for sporadic Creutzfeldt-Jakob Disease
Krista L. Harrison a,b,c, Sarah B. Garrettb, Joni Gilissenc,d, Michael J. Terranovae , Alissa Bernstein Sidemanb,c,f, Christine S. Ritchiea,c,g, and Michael D. Geschwindc,e a Division of Geriatrics, University of California, San Francisco, USA; b Philip R. University of California, San Francisco, USA; c Global Brain Health Institute, University of California, San Francisco, California, USA; d End-of-Life Care Research Group, Department of Family Medicine & Chronic Care, Vrije Universiteit Brussel (Vub), Belgium; e Memory and Aging Center, Department of Neurology, Weill Institute for Neurosciences, University of California San Francisco, San Francisco, CA, USA; f Department of Humanities and Social Sciences, University of California San Francisco, San Francisco, California, USA; g The Mongan Institute and the Division of Palliative Care and Geriatric Medicine, Massachusetts General Hospital, Boston, USA
ARTICLE HISTORY
Received 17 December 2021
Accepted 10 February 2022
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.
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Sources of support and amelioration
We identified 2 major categories of support – clinical care and caregiving.
Clinical care
Despite challenges, many respondents also experienced clinicians and clinical care as sources of support (Table 5). Caregivers appreciated clinician guidance about diagnosis, prognosis, or referrals. Anticipatory guidance was particularly helpful: to understand the patient’s disease trajectory, ‘[the doctor] told me to focus on the rate of change of various phases, . . . [if] it’s accelerating or he’s moving to another function loss that is your sign that he’s moving forward. But if he stabilizes somehow . . . that may not be sign that’s [he’s] ready to go. That really helped me’ (c4). Guidance about when to seek additional help and the expected disease course, including how to recognize signs of imminent death, helped caregivers feel prepared, take breaks, and feel less guilt.
Caregivers valued clinicians’ expertise in sCJD, particularly early in the disease journey, when detailed explanations of the disease were helpful. Respondents appreciated expert management of medications and advice about behavioural adaptations to manage sCJD symptoms. Confirming the CJD as sporadic (not genetic) was a source of relief for nearly all.
Sensitive and supportive relationships with clinicians and researchers stood out, such as when clinicians responded quickly and thoroughly: ‘I could call her or text her anytime and she would be answering questions for me’ (c3). Clinicians and others with prion expertise helped caregivers prioritize self-care: ‘[Dr. III] himself . . .. advised me strongly to back off from [caregiving], that I would potentially cause harm to myself that could be damaging. So I really appreciated that advice’ (c4). All caregivers reported engaging hospice, sometimes before the diagnosis of CJD. Though some caregivers expressed frustrations with hospice clinicians or the care model (especially lack of continuity), most found hospice helpful for providing hands-on ADL support, breaks, and comfort. They benefited from fast implementation and hospice staff’s expertise in recognizing imminent death and appreciated when hospice made effort to learn about sCJD and inquire about respondents’ knowledge as sCJD caregivers.
Caregiving
Finally, respondents identified sources that facilitated being a caregiver (Table 6). They emphasized the benefit of reading or hearing stories from, or connecting with other sCJD caregivers, via YouTube, Facebook, and the CJD Foundation: ‘all you want to do is talk to people that have been through it. Because you don’t know what to expect’ (c7).
Caregivers benefited from instrumental support (e.g. paid caregivers or facilities relieved caregiving burden): ‘I did all the heavy lifting at our house . . . When we transferred to the care facility, I felt lighter’ (c7). Friends or family also provided ADL, legal or financial help, such as documenting preferences or decision-makers while the patient was still able to make decisions. Many narratives indicated that socioeconomic resources were essential, such as being able to pay for caregiving, having access to state-funded care, and/or having jobs that permitted reduced schedules and lengthy leaves of absence.
Social and emotional support was beneficial. Much of this came from friends and family, e.g., keeping the patient company or reminding (and helping) the caregiver to take a break: ‘There wasn’t anything to do except support her and everybody was ready, willing and able to sign up’ (c5). Some caregivers benefitted resilience-bolstering activities, such as religious practice or exercise. Others found comfort in maintaining close connection to the person with sCJD; one caregiver did ‘spa days’ for his wife after she was bedbound (c12).
Caregiver recommendations or wishes
Caregivers also identified items that they thought would have been helpful to them or future sCJD caregivers. Though hypothetical, these insights may be useful for intervention development.
Regarding clinical care, some caregivers felt earlier accurate diagnosis and familiarity with sCJD among clinicians, hospice staff, and funeral home directors would have made the experience less difficult. Some recommended that clinics provide resources about expected symptoms and prognosis, what to take care of (e.g., advance care planning), resources (e.g., CJD Foundation, support groups, local hospice organizations), and contact information for expert advice about sCJD management to give facilities, hospices, and funeral homes.
Regarding caregiving, some respondents thought they would have benefited from more self-care and time connecting with the patient. When asked for recommendations for future sCJD caregivers, respondents echoed these themes: spend more meaningful time with the patient, have more patience with themselves and the patient, connect with other sCJD caregivers, and engage hospice care.
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Discussion
This novel study provides an expanded understanding of challenges experienced by caregivers and persons with sCJD and identifies opportunities for improvement. Challenges primarily related to clinical care and caregiving and were exacerbated by the unique nature of sCJD. To our knowledge, this is the first in-depth description of palliative care needs of persons with sCJD. Ford et al. (2018) previously studied caregivers’ struggles to manage symptoms of patients with sCJD and found the most problematic to be mobility and coordination, mood and behaviour, personal care and continence, eating and swallowing, communication, and cognition and memory [20 – 23, 7]. Caregivers in our study voiced similar challenges with symptoms, and described broader sources of distress and challenges. Caregivers framed changes in patient function within the larger context of major losses and changes to relationships, life plans, and family roles. We additionally asked about supports to identify factors that ameliorated caregivers’ difficulties. Supports were often the inverse of challenges, such as sensitive versus insensitive disclosure of diagnosis and prognosis.
Data on sources of distress and support in sCJD facilitates the development of neuropalliative tools and interventions. Table 7 demonstrates how palliative care approaches might be integrated into neurology practice for sCJD and slower-progressing dementia syndromes. For example, neurology trainees can be taught to use serious illness communication strategies [24] for sensitively disclosing a diagnosis of sCJD and asking if patients and caregivers want prognostic information or anticipatory guidance at this time [25,26]. Findings from this study can facilitate improving sCJD-specific care among hospice and palliative care clinicians. Neuropalliative-infused interventions for improving sCJD care will need to be refined with interdisciplinary multi-stakeholder input and tested for utility and effectiveness.
Evidence regarding neuropalliative care needs in sCJD may be applicable to other rare and rapidly progressive diseases with no cure, as well as longer-course neurodegenerative diseases. A recent systematic review of factors influencing the provision of palliative care to persons with advanced dementia report similar problems: difficulty managing symptoms, lack of continuity of care, and lack of clinician skill in palliative care (such as sensitive disclosure of information or providing anticipatory guidance) [27]. A systematic review of integration of palliative care into dementia management highlights the importance of discussing disease trajectory and expectations and challenges from suboptimal symptom and medication management [28]. These challenges appeared in our study as well. We are adapting the analytic approach of this sCJD study to our parallel efforts to identify neuropalliative intervention targets for longer-course dementia syndromes [29–31].
Limitations of the study include a relatively small sample at one institution, albeit one that recruits study participants nationally (and even globally) for this rare disease. Demographics of participating caregivers suggest that they are well-resourced. Caregivers with fewer resources may encounter more, or more severe, challenges than documented here. Future research should engage larger, more socioeconomically- and globallydiverse populations, and other RPDs that may raise different caregiving challenges. Nevertheless, these novel findings provide foundational data for further research and intervention development.
In summary, this study drew on palliative care frameworks and mixed methods to yield a comprehensive description of challenges, supports, and opportunities to improve care for people with sCJD and their caregivers. Though sCJD is rare and rapidly progressing, the themes uncovered provide a framework for ongoing efforts to improve neuropalliative care for dementia care more broadly.
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