The Huntington's Disease health-related Quality of Life questionnaire (HDQoL): a disease-specific measure of health-related quality of life

The Huntington's disease health-related quality of life questionnaire: a disease-specific measure of health-related quality of life. Huntington's disease (HD) is a genetic neurodegenerative disorder characterized by motor, cognitive and psychiatric disturbances, and yet there is no disease-specific patient-reported health-related quality of life outcome measure for patients. Our aim was to develop and validate such an instrument, i.e. the Huntington's Disease health-related Quality of Life questionnaire (HDQoL), to capture the true impact of living with this disease. Semi-structured interviews were conducted with the full spectrum of people living with HD, to form a pool of items, which were then examined in a larger sample prior to data-driven item reduction. We provide the statistical basis for the extraction of three different sets of scales from the HDQoL, and present validation and psychometric data on these scales using a sample of 152 participants living with HD. These new patient-derived scales provide promising patient-reported outcome measures for HD

Meaningful and measurable health domains in Huntington’s: large-scale validation of the Huntington’s disease health-related quality of life questionnaire (HDQoL) across severity stages

Objectives: While health-related quality of life is key for patients with long-term neurodegenerative conditions, measuring this is less straightforward and complex in Huntington’s disease. This study aimed to refine and validate a fully patient-derived instrument, the Huntington’s Disease health-related Quality of Life questionnaire (HDQoL), and to elucidate health domains that are meaningful to patients’ lived experience. Methods: Five-hundred and forty-one participants, from pre-manifest to end-stage disease completed the HDQoL, together with generic quality of life measures, and in-person motor, cognitive and behavioural assessments. The psychometric properties of the HDQoL were examined using factor analysis and Rasch analysis. Results: Four HDQoL domains emerged reflecting the classical triad of HD features - they were Physical-Functional, Cognitive, and two different behavioural aspects i.e. Mood-Self domain, as well as a distinct Worries domain. These domains clarify the behavioural sequelae as experienced by patients, and all showed good to excellent internal consistency. Known groups analyses illustrated significant and graded changes in clinical assesments and corresponding HDQoL domains across severity levels. Convergent and discriminant validity was demonstrated by the expected pattern of correlations between specific HDQoL domains and corresponding domain-relevant clinical assesments as well as patient-reported measures. The data demonstrate robust support for the refined HDQoL across disease stages. Conclusions: The HDQoL with its two distinct behavioural domains of Mood-Self and of Worries, as well as a Physical-Functional and a Cognitive domain, is a relevant, reliable and valid patient-derived instrument to measure the impact of Huntington’s disease across all severity stages

Sexual function and sexual satisfaction following spinal cord injury: an interpretative phenomenological analysis of partner experiences

Purpose This study explored how partners/spouses of people with Spinal Cord Injury (SCI) experienced intimacy, sexual function, and sexual satisfaction post-SCI. Materials and methods Qualitative, semi-structured interviews were conducted with the partners/spouses of people with SCI living in the community in the United Kingdom. Twelve participants (7 females; 5 males) were recruited using purposive sampling. A nine-item semi-structured interview design was used. Interviews were transcribed verbatim and analysed via Interpretative Phenomenological Analysis (IPA). Results Three superordinate themes were demonstrated: (1) Stolen sex through unpreparedness; (2) Redefining sex; and (3) Compromised commitment. Partners struggled to come to terms with the shock of radically altered post-SCI sexual relationships, questioning how their changed sexual relationship and sexual identity conflicted with caring requirements. To minimise post-SCI relationship changes, some partners engaged in strategies to protect against, distract from and avoid sexual intimacy, whereas others were able to retain adapted pre-injury patterns of intimate behaviour. Conclusion Compromised sexual function and satisfaction significantly disrupt relationship dynamics post-SCI, initiate voluntary celibacy, and limit the perceived viability of continued sexual intimacy. Specialist partner-support provisions are urgently needed, recognising that partner support needs are not restricted to the inpatient rehabilitation phase but importantly extend long past discharge into the community. Implications for Rehabilitation Sexual function and satisfaction are highly challenging areas for partners post-spinal cord injury (SCI). Support and education for the partner must be incorporated into SCI rehabilitation to help with both adjustment and acceptance to changes in sexual functioning post-SCI. Partner contributions to and experiences of sexual function and satisfaction should be given more value, as these aid in the sustainability of healthy sexual relationships and psychological wellbeing following SCI

Rumination in dementia and its relationship with depression, anxiety, and attentional biases

Rumination (self-referential and repetitive thinking), attentional biases (AB), and impaired cognitive control are theorized as being integral factors in depression and anxiety. Yet, research examining the relationship between rumination, mood, and AB for populations with reduced cognitive control, e.g., people living with dementia (PwD), is lacking. To explore whether literature-based relationships are demonstrated in dementia, PwD (n = 64) and healthy controls (HC) (n = 75) completed an online self-report survey measuring rumination and mood (twice), and a telephone cognitive status interview (once). Rumination was measured as an emotion-regulation style, thinking style, and response to depression. We examined the test-retest reliability of PwD’s (n = 50) ruminative-scale responses, ruminative-scale internal consistency, and correlations between rumination, age, cognitive ability, and mood scores. Also, nine participants (PwD = 6, HC = 3) completed an AB measure via eye-tracking. Participants fixated on a cross, naturally viewed pairs of facial images conveying sad, angry, happy, and neutral emotions, and then fixated on a dot. Exploratory analyses of emotional-face dwell-times versus rumination and mood scores were conducted. Except for the HC group’s reflective response to depression measure, rumination measures were reliable, and correlation strengths between rumination and mood scores (.29 to .79) were in line with literature for both groups. For the AB measure subgroup, ruminative thinking style scores and angry-face metrics were negatively correlated. The results of this study show that literature-based relationships between rumination, depression, and anxiety are demonstrated in dementia, but the relationship between rumination and AB requires further investigation

Health-related quality of life in Huntington’s Disease patients: a comparison of proxy assessment and patient self-rating using the disease-specific Huntington’s Disease health-related quality of life questionnaire (HDQoL)

Huntington’s disease (HD) is a fatal, neurodegenerative disease for which there is no known cure. Proxy evaluation is relevant for HD as its manifestation might limit the ability of persons to report their health-related quality of life (HrQoL). This study explored patient–proxy ratings of HrQoL of persons at different stages of HD, and examined factors that may affect proxy ratings. A total of 105 patient–proxy pairs completed the Huntington’s disease health-related quality of life questionnaire (HDQoL) and other established HrQoL measures (EQ-5D and SF-12v2). Proxy–patient agreement was assessed in terms of absolute level (mean ratings) and intraclass correlation. Proxies’ ratings were at a similar level to patients’ self-ratings on an overall Summary Score and on most of the six Specific Scales of the HDQoL. On the Specific Hopes and Worries Scale, proxies on average rated HrQoL as better than patients’ self-ratings, while on both the Specific Cognitive Scale and Specific Physical and Functional Scale proxies tended to rate HrQoL more poorly than patients themselves. The patient’s disease stage and mental wellbeing (SF-12 Mental Component scale) were the two factors that primarily affected proxy assessment. Proxy scores were strongly correlated with patients’ self-ratings of HrQoL, on the Summary Scale and all Specific Scales. The patient–proxy correlation was lower for patients at moderate stages of HD compared to patients at early and advanced stages. The proxy report version of the HDQoL is a useful complementary tool to self-assessment, and a promising alternative when individual patients with advanced HD are unable to self-report

“Data is the new oil”: citizen science and informed consent in an era of researchers handling of an economically valuable resource

As with other areas of the social world, academic research in the contemporary healthcare setting has undergone adaptation and change. For example, research methods are increasingly incorporating citizen participation in the research process, and there has been an increase in collaborative research that brings academic and industry partners together. There have been numerous positive outcomes associated with both of these growing methodological and collaborative processes; nonetheless, both bring with them ethical considerations that require careful thought and attention. This paper addresses the ethical considerations that research teams must consider when using participatory methods and/or when working with industry and outlines a novel informed consent matrix designed to maintain the high ethical standard to which academic research in the healthcare arena has traditionally adhered

Cognitive rehabilitation, self-management, psychotherapeutic and caregiver support interventions in progressive neurodegenerative conditions: a scoping review

BACKGROUND: Despite their potentially significant impact, cognitive disability may be overlooked in a number of progressive neurodegenerative conditions, as other difficulties dominate the clinical picture. OBJECTIVE: We examined the extent, nature and range of the research evidence relating to cognitive rehabilitation, self-management, psychotherapeutic and caregiver support interventions in Parkinsonian disorders, multiple sclerosis (MS), frontotemporal dementias (FTD), motor neuron disease and Huntington’s disease. METHODS: Scoping review based on searches of MEDLINE and CINAHL up to 15 March 2016. RESULTS: We included 140 eligible papers. Over half of the studies, and almost all the randomised controlled trials, related to MS, while a number of single case studies described interventions for people with FTD. CR interventions addressed functional ability, communication and interaction, behaviour or memory. The majority of psychotherapy interventions involved cognitive behavioural therapy for depression or anxiety. Self-management interventions were mainly available for people with MS. There were few reports of interventions specific to caregivers. Numerous methodological challenges were identified. CONCLUSIONS: The limited range of studies for all conditions except MS suggests a need firstly to synthesise systematically the available evidence across conditions and secondly to develop well-designed studies to provide evidence about the effectiveness of CR and other psychological interventions

Exploring the potential of virtual reality technology to investigate the health and well being benefits of group singing

There is a growing body of academic research aiming to quantify and understand the associated health and well being benefits of group singing. The social interaction is known to strongly contribute to perceived improvements to mental and physical health but there are also indications that singing together elicits better well being outcomes that other community activities. This paper introduces the Vocal Interaction in an Immersive Virtual Acoustic (VIIVA) system, which allows the user to take part in a group singing activity in 360 degree virtual reality, hearing themselves in the recorded venue alongside the other singers. The VIIVA is intended to make group singing accessible to those unable to attend real community choirs but also as a tool for experimental research into the health and well being benefits of group singing. This paper describes the system and presents a number of methodologies and applications which are discussed in relation to three ongoing research projects. Preliminary work indicates that the VIIVA system and the devised setups provide a promising tool with which to study the health and well being benefits of group singing, and in particular to control for the social interactions inherent in real group singing activities