Background Few data is available regarding most advanced stages of HD.
Aims To describe clinical manifestations, medication use and caregivers’ burden in late-stage HD (LS-HD).
Patients and methods LS-HD patients were extracted from the database of the REGISTRY study. Criteria for late-stage were a score in the Total Functional Capacity (TFC) ≤3. Data on demographics, Huntington's Disease Rating Scale (HDRS) scores, QoL (SF-36), resource utilization (Client Service Receipt Inventory-CSRI), caregiver burden (Care Giver Questionnaire-CARE), and medication use were obtained.
Results 420 patients were included (54% females), a mean TFC score of 1.97 (SD±1.0). Mean age 54 years (SD±12.1). Mean HDRS motor score 62.2 (SD±19.2), mean HDRS cognitive score 67.9 (SD±54.8), mean HDRS behaviour score 18.4 (SD±12.6), and mean HDRS functional score 7.1 (SD±4.7). QoL was poor and domains most affected concerned physical functioning, emotional health and energy/fatigue. Admission to neurology ward (15%) or residential home (12.8%) occurred in few. 75% needed informal care and 33% a wheelchair. QoL was mildly correlated with the HDRS motor, behaviour and functional scores (p < 0.05). Residential home admission was associated with TFC scores (p < 0.001). Major indications for prescription were depression, chorea/HD, dyskinesias, insomnia, anxiety, and psychosis. CARE score was moderately correlated with TFC, behaviour, and functional scores (p < 0.001). 86.4% strongly agreed their relatives were dependent on them, however in only 33.8% a strong wish to escape was reported, and most were neither ashamed of (92.9%) nor angry with (80.4%) their relative.
Conclusions LS-HD have a poor QoL, mostly due to physical, emotional and fatigue domains. Most live at home, dependent on sympathetic carers, who are mainly drained due to the behaviour and functional status of patients.
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