Prion disease provides a paradigm for developing evidence-based dementia palliative care to reduce distress and increase quality of life. Prion diseases such as sporadic Jakob-Creutzfeldt disease (sJCD) are rare but devastating conditions. sJCD progresses rapidly, with cognitive, movement, and functional deficits requiring 24-hour care. Death often occurs within months and caregiver burden is enormous. Palliative care is an approach to serious illness care that reduces distress and optimizes quality of life through expert management of symptoms and aligning care with goals.
While palliative care could benefit people with prion disease and their caregivers, little is currently known about their end-of-life needs. Prion disease is also a promising model for translating palliative care evidence developed in cancer to dementia syndromes. The pilot study characterizes palliative care needs and experiences through interviews with 12 caregivers of people who died from sJCD after enrolling in one of the studies at the UCSF Memory and Aging Center (MAC). We integrate qualitative data with quantitative data on patient symptoms, function, and other sources of distress assessed at the research visit. We are using these findings to develop a prion palliative care toolkit, refined through discussions with interdisciplinary clinicians and those with prion disease experience. This research will contribute to the development of evidence-based palliative care for people with prion disease and other dementia syndromes locally and internationally.