Gaining insight into the views of outpatients with Huntington’s disease regarding their future and the way they deal with their poor prognosis: a qualitative study

Background: Huntington’s disease (HD) has a poor prognosis. Decision-making capacity and communication ability may become lost as the disease progresses. Therefore, HD patients are encouraged to engage in advance care planning (ACP). To improve ACP for HD patients, there is a need to better understand how these patients face their poor prognosis. Aim: To gain insight into the views of HD patients who receive outpatient care regarding their future and the way they deal with the poor prognosis of their disease. Methods: A qualitative study using semi-structured interviews with 12 patients with HD (7 outpatient clinic, 3 day care, 2 assisted living facility). Audio-recorded interviews were transcribed verbatim. Through reading and re-reading interviews, writing memos and discussions in the research team, strategies were identified. Results: Three strategies emerged for facing a future with HD. Participants saw the future: 1) as a period that you have to prepare for; 2) as a period that you would rather not think about; 3) as a period that you do not have to worry about yet. Participants could adopt more than one strategy at a time. Even though participants realized that they would deteriorate and would need more care in the future, they tried to keep this knowledge ‘at a distance’, with the motivation of keeping daily life as manageable as possible. Conclusions: Official ACP guidelines recommend discussing goals and preferences for future treatment and care, but patients tend to want to live in the present. Further research is needed to elucidate the best approach to deal with this discrepancy

Nursing home residents with Huntington's disease: Heterogeneity in characteristics and functioning

Background: In Huntington's disease (HD), admission to a nursing home (NH) is required in advanced disease stages. To gain insight in care needs, more knowledge is needed on the functioning of this group. Objective: Describing patient and disease characteristics, their functioning, and gender differences. Methods: A cross-sectional descriptive design was used to collect data of 173 patients living in eight Dutch HD-specialized NHs. Data were collected on characteristics and functioning. We tested for gender differences. Results: Mean age was 58.3 years and 49.7% were men. Activities of daily living and cognition varied from 46 to 49% mildly impaired to 22–23% severely impaired. Communication was severely impaired in 24%. Social functioning was low in 31% and high in 34%. A majority of patients used psychotropic medications (80.3%) and showed neuropsychiatric signs (74%). Women were on average more dependent in ADL (severely impaired 33.3% vs 12.8%), more often depressed (26.4% vs 11.6%), and prescribed antidepressant medications more often (64.4% vs 48.8%) than men. Conclusions: The population of HD patients in NHs is heterogeneous in terms of patient and disease characteristics, and functioning. As a consequence, care needs are complex leading to implications for the required expertise of staff to provide adequate care and treatment