Background Huntington's disease (HD) is a progressive, inherited neurodegenerative condition, with onset usually in mid-life, affecting motor, cognitive and neuropsychiatric functions. Not surprisingly, studies have suggested that HD has a detrimental effect on the quality of life (QoL) of individuals with the HD gene expansion and also by association their families. The present literature exploring the impact for spouses predominantly investigates the later stages of HD. This study, however, uses clearly defined groups of both premanifest and early-stage symptomatic gene-carriers and their spouse/partners.
Methods/techniques Self-report measures of quality of life and neuropsychiatric domains (SF-36; Quality of Life Index; Frontal Systems Behaviour Inventory (FrSBe)), clinical rating of motor function (UHDRS motor), Cognitive measures (Emotion Recognition, Trails-B) and the Short version of Problem Behaviours Assessment for HD (PBA-s) were completed by 86 participants from the TRACK-HD study. This included 23 pre-manifest gene carriers, 20 early, clinical stage 1 and 2, HD patients and 43 gene-negative spouse controls. Between-group differences and relationships between neuropsychiatric and cognitive data from the premanifest and symptomatic participants and QoL for their spouses were examined.
Results/outcomes Largest between-group differences were evident between spouses and their partners with early stage disease; and different QoL domains were impacted in the different participant groups. The data presented shows the QoL domains affected in each group; and the impact of specific neuropsychiatric, cognitive and motor symptoms on spouse partners. Identification of the specific domains impacted in spouse/partners at an early stage of disease is crucial for establishing appropriate and long-term support.
- Quality of life