Huntington’s Disease Progression and Caregiver Burden

Abstract

Huntington’s disease (HD) is a neurodegenerative, progressive disorder conditioned by a mutation in the <i>HTT</i> gene. Its progression is dependent on the causative mutation extension. Caregivers of individuals affected by HD, most often patients’ relatives, are burdened with the care. This study aims to assess the caregivers’ burden cross-sectionally and longitudinally and look for biological and clinical patients-related burdening factors. In total, 144 caregiver-patient pairs observed annually for up to 8 years were included in the study. In all of the patients, demographic data were collected, Unified Huntington's Disease Rating Scale (UHDRS) assessments were conducted, and disease burden (DB) was calculated when caregivers were assessed in Caregiver Burden Inventory (CBI). Caregivers’ burden measured in CBI at the first visit reached 18.7 ± 18.4 scores. Longitudinal observation showed no evidence for any discrepancy between clinical progression measured in UHDRS, nor biological progression measured in DB and the caregivers’ burden progression measured in CBI. Caregivers were burdened mostly by patients’ dependence and a discrepancy between reality and life expectations. This study indicates factors to be addressed to reduce caregivers’ burden. Strict relation between caregivers’ burden and biological and clinical progression denies conception of overloaded with care tasks or adaptation to the burden.

Caregiver Burden of Patients With Huntington’s Disease in South Korea

“…By carefully reviewing the literature and our data, we found that the caregiver burden of HD patients in Korea was high, which was consistent with previous reports in other countries. [9][10][11][12][13]15 The present study provides useful insight into the factors associated with the caregiver burden in Korea. Previous studies have shown that advanced disease and low functional capacity were associated with the caregiver burden.…”

“…The present study provides useful insight into the factors associated with the caregiver burden in Korea. Previous studies have shown that advanced disease and low functional capacity were associated with the caregiver burden [ 9 , 10 , 12 ]. It makes sense that as patients’ function declines, their dependency on caregivers increases.…”

“…Previous studies have shown that advanced disease and low functional capacity were associated with the caregiver burden. 9,10,12 It makes sense that as patients' function declines, their dependency on caregivers increases. However, functional capacity is not solely determined by chorea or other motor symptoms in HD.…”

“…The caregiver burden of HD seems to be affected by patients' motor disturbance, dependency, and functional capacity as well as by the discrepancy between reality and their expectations of life and patients' nonmotor disabilities, such as irritability, obsessive-compulsive behaviors, depression, anosognosia, and cognitive dysfunction. [9][10][11][12][13][14][15] On the other hand, the burden is relatively low for patients with the apathetic predominant type. 9 In addition, regional and ethnic differences should be considered since the caregiver burden is re-lated to social and cultural factors.…”

Invisible patients in rare diseases: parental experiences with the healthcare and social services for children with rare diseases. A mixed method study

“…While several previous studies on the experiences of caregivers, namely parents, of CRDs have been conducted in Poland, including Huntington disease [32][33][34] or Prader-Willi syndrome 35 , most often they focuses either on the challenges and needs related to caring for such a child, caregivers' burden or social functioning of a family whose member experiences RD. However, few data are available on the experiences of CRD caregivers with the healthcare system in Poland 20 .…”