The PACE-DLB Study is for people with at least moderate dementia with Lewy bodies (DLB) and their primary caregiver. The study's goals are to learn more about the experiences of people with DLB and their caregivers as DLB progresses, and what happens as the end of life of the person with DLB approaches. Participants are enrolled through one of five Lewy Body Dementia Association (LBDA) Research Center of Excellence clinics, or directly via the LBDA.

People with DLB will enroll into the study along with their primary informal caregiver, such as the family member or friend who is involved in most of their care and attends the majority of their medical appointments. A legally authorized representative (LAR) for the person with DLB will consent to participate in the study on the person's behalf. If the person with DLB resists taking part in the study, the pair will not be enrolled.

The study consists of questionnaires about the progression of the person's dementia and the experiences of the caregiver. The person with DLB will complete one questionnaire themselves if they are able to. If the pair is enrolled at a LBDA Research Center of Excellence clinical site, and completes study visits in-person, a brief physical exam of the person with DLB will be completed by a clinician.

Study participation will last for three years, or until the person with DLB dies. Study visits take place every six months, either virtually or in person at a clinical study site. If the person with DLB dies during the study, the caregiver will complete one final virtual study visit three months afterward.

Specific Aims of the PACE-DLB Study:

1. To determine predictors of the end of life (death within 6 months) in the DLB population.

   Hypotheses: Predictors of death within 6 months for individuals with DLB will include symptoms (e.g. worsening hallucinations, fluctuations) not captured in existing dementia end-of-life prediction tools.

2. To identify whether change in caregiver measures can predict the end of life in the DLB.

   Hypotheses: The investigators hypothesize that there will be worsening caregiver burden, depression, grief, and quality of life preceding the last 6 months of life, controlling for variables such as resilience, support, and living situation.

3. To identify the impact of demographics, health care factors, and specific DLB symptoms on patient quality of life and caregiver experiences at the end of life of the person living with DLB.

Hypotheses: Location (rural, urban, suburban), provider experience (e.g. specialist vs not), patient residence (e.g. home, skilled nursing), degree of behavioral symptoms, caregiver type (spouse vs child vs other), and caregiver characteristics (self-efficacy, perceived social support, resilience, and coping) will affect patient and informal caregiver experiences at the patient's end of life, including patient and caregiver quality of life and caregiver well-being, depression, burden, grief, and satisfaction with end-of-life care.

Dementia with Lewy bodies (DLB) is part of Lewy body dementia, the 2nd-most-common degenerative dementia in the U.S. It is also one of the Alzheimer's disease-related dementias (ADRDs). Evidence-based models of end-of-life care for ADRDs are insufficient; families face difficult medical decisions with little information for guidance. This is particularly relevant for DLB, where >70% of individuals die of the dementia itself or failure to thrive. Caregivers describe lack of knowledge of what to expect at the end of life as an important driver of negative end-of-life experiences. The proposed study addresses this unmet need. It also addresses the National Institute on Aging's priority to improve quality of care and quality of life for persons with ADRDs at the end of life and their caregivers.

Based on published clinical series, over half of enrolled individuals with DLB are expected to die over the course of the study. To achieve diverse recruitment, the study includes an in-person cohort where dyads are recruited from Lewy Body Dementia Association Research Centers of Excellence and a novel virtual cohort where dyads are recruited through the Lewy Body Dementia Association and study visits are conducted by telephone. Having a virtual cohort will capture the experiences of individuals with DLB and caregivers not receiving care at specialty centers.

Because the majority of study measures are caregiver-reported, the in-person and virtual groups have identical study measures, except for the Unified Parkinson Disease Rating Scale, completed only in person. Collected measures will include demographics, DLB characteristics (e.g. cognitive, motor, behavioral, sleep, and autonomic symptoms), health care providers and settings, caregiver considerations (e.g. resilience, support, grief, burden), quality of life (for the patient and caregiver), and satisfaction with end of life experiences.

The caregiver will complete a final study visit 3 months after the death of the person with DLB to assess grief, recovery, and quality of the end of life experience. By following dyads through the end of life and death of the person with DLB and the initial bereavement period for the caregiver, the study will identify DLB-specific predictors of the last 6 months of life, changes in caregiver measures that identify the last 6 months of life, and factors which influence quality end of life experiences. These results will inform the content and timing of end-of-life counseling in DLB, help establish quality metrics for end of life care, and identify areas where interventions could potentially improve end of life experiences for individuals with DLB and their caregivers.

Inclusion Criteria:

• Patient and caregiver willing to participate as a dyad (patients will need a legally authorized representative to consent on their behalf given the requirement for at least moderate dementia, but patients who resists participating in the study will not be enrolled)
• U.S. residents (i.e., individuals accessing the LBDA from outside the U.S. will be excluded)
• Patient with a clinical diagnosis of DLB
• Patient with moderate severity dementia as assessed by the Quick Dementia Rating System (QDRS, with a score of >12 OR ≥2 in at least 3 domains suggestive of moderate dementia)
• Caregiver telephone interview for cognitive status (TICS) score of >31 to ensure that the caregiver is able to reliably complete study visits
• Patient participant expected to live at least 6 months (so that at least a baseline visit and 1 follow up visits is expected to be completed)

Exclusion Criteria:

• Patient participant diagnosed with dementia of less than moderate severity
• Patient with dementia resists participation
• Non-U.S. residents
• Patient participant with dementia not expected to live at least 6 months
• Virtual cohort ONLY: Patient participant receives medical care from a LBDA Research Centers of Excellence clinic

Patients at five LBDA Research Centers of Excellence clinics may enroll into the study if they meet inclusion criteria. Clinics participating in the study are located at the University of Florida, Gainesville, FL; University of Miami, Miami, FL; University of Virginia, Charlottesville, VA; University of Michigan, Ann Arbor, MI; and the Mayo Clinic, Rochester, MN.

People who have indicated interest in research opportunities through the LBDA mailing listserv will be emailed study information, including how to enroll.