Clinical significance of self-descriptive apathy assessment in patients with neurological form of Wilson’s disease

Background and aim Apathy is one of the neuropsychiatric symptoms of Wilson’s disease (WD) which typically affects the brain’s fronto-basal circuits. Lack of agreed diagnostic criteria and common use of self-description assessment tools lead to underestimation of this clinical phenomenon. The aim of this study was to investigate whether subjective and informant-based clinical features of apathy in patients with WD enable clinicians to make a valid diagnosis. Methods Multiple aspects of goal-oriented behavior were assessed in 30 patients with the neurological form of WD and 30 age-matched healthy participants using two questionnaires, the Lille Apathy Rating Scale (LARS) and the Dysexecutive Questionnaire (DEX). Both included a self-descriptive and a caregiver/proxy version. Cognitive functioning was estimated with the use of Addenbrooke’s Cognitive Examination-Revised. Results Patients obtained significantly worse scores on all clinical scales when more objective measures were considered. Features of apathy and executive dysfunction were revealed in patients’ caregiver versions of LARS and DEX, which may indicate poor self-awareness of patients with WD. Roughly 30% of participants were likely to present with clinically meaningful symptoms, independent of cognitive dysfunction. Conclusions Methods relying on self-description appear inferior to informant-based scales when diagnosing apathy. More objective criteria and measurement tools are needed to better understand this clinical syndrome.

How are you doing in the eyes of your spouse? Level of agreement between the self-completed EQ-5D-5L and two proxy perspectives in an orthopaedic population: a randomized agreement study

Objectives To determine the level of agreement between both proxy versions and the self-completed EQ-5D-5L. Design A randomized agreement study. Setting and participants We recruited 120 patients (compos mentis) and their proxies at the orthopaedic outpatient clinic. Patients completed the regular EQ-5D-5L and their proxy completed the proxy version of the EQ-5D-5L and rated the patients’ health from their own (proxy-proxy) perspective (i.e. how do you rate the health of the patient), and from the patient’s (proxy-patient) perspective (i.e. how do you think the patient would rate their own health if they were able to). Measures The primary outcome was the agreement between patients and their proxy, quantified as the intra class correlation coefficient for the EQ-5D-5L Utility score. Results Average Utility scores were 0.65 with the self completed EQ-5D-5L, versus 0.60 with the proxy-patient version and 0.58 with the proxy-proxy version. The ICC was 0.66 (95% CI 0.523, 0.753) for the proxy-patient perspective and 0.58 (95% CI 0.411, 0.697) for the proxy-proxy perspective. The mean gold standard score of the VAS-Health was 69.7 whereas the proxy-proxy perspective was 66.5 and the proxy-patient perspective was 66.3. Conclusion and implications The proxy-patient perspective yielded substantial agreement with the self completed EQ-5D-5L, while the agreement with the proxy-proxy perspective was moderate. In this study population of patients without cognitive impairment, proxies tended to underestimate the quality of life of their relative.

Understanding the spiritual and emotional needs of siblings of chronically sick children with a rare disease

Background Family morbidity is a concern in the treatment of paediatric patients but guidance and research lacks reference to siblings. Siblings of chronically sick children are at risk of adverse outcomes if needs are not recognized and met. A literature review demonstrated that positive outcomes are possible with appropriate support. Objective To identify emotional and spiritual needs of children and young people (CYP) with a sibling with a chronic disease locating this within a wider understanding of the needs of this population.Method A mixed methods study including: validated, UK normed 52-item Kidscreen questionnaire for siblings, proxy version for parents; interviews of each sibling and focus groups drawn from these siblings were recorded, transcribed and subjected to iterative thematic analysis. The setting was Birmingham Children’s Hospital NHSFT, UK. Participants were 16 siblings and 16 parents of children with a chronic rare disease aged 8-16, one specialist nurse.Results The mean of parents’ scores on the questionnaire rated the sibling below the 50th percentile on all ten areas covered. A mean of 34.9 (median 31) for home life shows a significant impact of having a sick child. Siblings consistently rated themselves higher than parents. From the thematic analysis, largely negative consequences of having a sick sibling appeared in all domains. Coping mechanisms were in two main categories: 1. Creative and leisure activities, 2. Family and friends.Discussion and Conclusion Having a chronically unwell sibling negatively impacts global wellbeing, and, specifically in this study, their emotional and spiritual development. Further research will be needed to determine if this can be ameliorated

Development of the Experienced Communication in Dementia Questionnaire: A Qualitative Study

Communication problems with their caregivers are common in people with dementia. Although interventions for improvement of communication are being developed, a tool to measure how participants experience their communication is lacking. The objective of this article is to describe the development of a questionnaire that measures the “experienced communication” of persons with dementia (ECD-P) as well as of their caregivers (ECD-C). Interviews were conducted with five person with dementia—caregiver dyads who had recently received a new communication intervention. Reflexive thematic analysis was performed on the transcripts using ATLAS.ti. Codes were created, categories and themes were identified, and items for the questionnaires were generated. Selection of items and response scales was done in collaboration with the same dyads. The final version was established after pilot testing with seven other dyads and discussion with five experts in the field of dementia care. Analysis of the transcripts resulted in 212 codes and 17 categories within four themes: caregiver competence, social communication, communication difficulties in daily life, and experienced emotions during conversations. The final version of the ECD-P consists of part 1 with 22 items and 4-point Likert scales, and part 2 with two items and 1 to 10 scales. In the final ECD-C (proxy version), part 1 and part 2 are similar to the ECD-P, while a part 3 was added to assess caregivers’ own perspective and emotions (five items). Based on the experiences of people with dementia and their caregivers, we constructed a face-valid questionnaire. This justifies future research to test its clinimetric characteristics.

Understanding the spiritual and emotional needs of siblings of chronically sick children with a rare disease

Background Family morbidity is a concern in the treatment of paediatric patients but guidance and research lacks reference to siblings. Siblings of chronically sick children are at risk of adverse outcomes if needs are not recognized and met. Positive outcomes are possible with appropriate support. Objective To identify emotional and spiritual needs of children and young people (CYP) with a sibling with a chronic disease. Method A mixed methods study including: validated, UK normed 52-item Kidscreen questionnaire for siblings, proxy version for parents; interviews of each sibling and focus groups drawn from these siblings were recorded, transcribed and subjected to iterative thematic analysis. The setting was Birmingham Children’s Hospital NHSFT, UK. Participants were 16 siblings and 16 parents of children with a chronic rare disease aged 8–16, one specialist nurse. Results The mean of parents’ scores on the questionnaire rated the sibling below the 50th percentile on all ten areas covered. A mean of 34.9 (median 31) for home life shows a significant impact of having a sick child. Siblings consistently rated themselves higher than parents. From the thematic analysis, largely negative consequences of having a sick sibling appeared in all domains. Coping mechanisms were in two main categories: 1. Creative and leisure activities, 2. Family and friends. Discussion and Conclusion Having a chronically unwell sibling negatively impacts global wellbeing, and, specifically in this study, their emotional and spiritual development. Further research will be needed to determine if this can be ameliorated