Supplementary Material for: Caregiver Burden in Prion Disease

Background: Caregiver burden significantly impacts patient and caregiver outcomes and is an important treatment consideration in dementia. Previous research has demonstrated that like behavioral variant frontotemporal dementia, prion disease has higher caregiver burden than other forms of dementia; however, limited prospective research has investigated this specifically. Here we aim to describe caregiver wellbeing and burden in prion disease and determine whether demographic features, support group attendance, or features of the disease process predicted higher caregiver burden.
Methods: 30 patients with prion disease and their caregivers were assessed longitudinally through the Teleneurology Assessment Program for Creutzfeldt-Jakob Disease. Caregivers were administered assessment instruments including the Neuropsychiatric Inventory Questionnaire (NPI-Q), MRC Prion Disease Rating Scale, and Outcome Evaluation of the National Family Caregiver Support Program. We performed descriptive and inferential statistics to examine the progression of caregiver burden and identify features that impacted caregiver burden severity.
Results: 30 caregiver-patient dyads were followed longitudinally. Prion disease duration averaged 7.88 months. Mean initial NPI-Q distress score was 15. Qualitatively, distress increased until peaking on average half-way through disease duration, then declined. Higher burden (4-item Zarit Burden Interview) was associated with younger age at disease onset. Burden was not predicted by disease type, duration, caregiver demographics, MRC Prion Rating Scale scores, NPI-Q, or support group attendance
Conclusion: These findings confirm significant caregiver distress in prion disease and help to describe the course of caregiver burden throughout the disease. Statistical analyses were limited by small sample size and phenotypic heterogeneity and future research would benefit from larger samples.