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.

Patient perspectives on advance euthanasia directives in Huntington's disease. A qualitative interview study.

BACKGROUND: Huntington's disease (HD) has a poor prognosis. For HD patients in the Netherlands, one way of dealing with their poor prognosis is by drawing up an advance euthanasia directive (AED). Little is known about the perspectives of HD patients on their AED. AIM: To gain insight into patients' views on and attitudes towards their AED, and changes over time. METHODS: A longitudinal qualitative interview study using 1 to 6 semi-structured interviews over a period of maximum three years. Nine HD patients (5 outpatient clinic, 3 day care, 1 assisted living facility) who either had an AED or were thinking about drawing it up participated in this study. RESULTS: We identified two themes that characterize patients' perspectives on their AEDs: (1) general character of the AED; (2) uncertainty around their AED. Ad (1) The conditions that the participants described in their AED were generally not very specific for the person. Mostly they were general notions of unbearable suffering. Familiarity with HD in the family could play a role in drawing up an AED. Ad (2) Participants generally were aware of the tentative character of their AED and could have doubts concerning their own willingness or the willingness of others in the future. Sometimes these doubts were so great, that it prevented them from drawing up an AED. However, patients did not alter their AED during the follow-up period or changed in their view or attitude on their AED. CONCLUSION: HD patients that draw up an AED usually describe general conditions for euthanasia and recognize that these conditions may change as the disease progresses. An AED or the wish to draw one up may be a good conversation starter for conversations about goals and preferences for future care.

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.

Advance Care Planning in Huntington Disease: The Elderly Care Physician's Perspective.

OBJECTIVE: Huntington disease (HD) has a poor prognosis. Decision-making capacity and communication ability may become impaired as the disease progresses. Therefore, HD patients are encouraged to engage in advance care planning (ACP). Elderly care physicians (ECPs) can play an important role in ACP in HD patients. However, little is known about their experiences in this role. The aim of this study is to gain insight into how ECPs practice ACP with HD patients. DESIGN: A qualitative interview study. SETTING AND PARTICIPANTS: Nine ECPs working in HD-specialized nursing homes in the Netherlands. METHODS: We conducted semistructured interviews with ECPs between June 2018 and July 2020. RESULTS: Two phases could be identified in the process of ACP. In the first phase, when the feared future seems to be far away, the ECP asks about the patient's wishes for the future in an accommodating manner. In the second phase, when the feared future is closer, future medical treatment and care becomes less hypothetical. Agreement has to be reached on upcoming treatment decisions. In this phase, the ECP takes a more guiding role, and consequently encounters more difficulties, such as maintaining a positive patient/family-physician relationship while dealing with disagreements with patient or family. Most participants shared their experiences with euthanasia when asked about ACP. When making a comparison of ACP between HD patients and patients with other neurodegenerative disorders in nursing homes, the ECPs emphasized the similarities. CONCLUSIONS AND IMPLICATIONS: ACP in HD can be classified into 2 phases, which differ in ECPs' approach and the complexity experienced by the ECP. Awareness of this finding may help to further develop training and education in ACP, including dealing with euthanasia, to make ECPs feel better equipped in practicing ACP in HD.