'A lightbulb moment': carers' experiences of behavioural symptoms in motor neurone disease before and after MiNDToolkit.

BACKGROUND: To explore carers' experiences of behavioural symptoms in Motor Neurone Disease (MND), before and after using the MiNDToolkit, a novel internet-based psychoeducational intervention to support management of behavioural symptoms (BehSymp) in MND. The study also investigated carers' views and acceptability of MiNDToolkit. METHODS: A qualitative process evaluation of carers engagement with, and acceptability of, the MiNDToolkit conducted using semi-structured interviews with carers (n = 11). All interviews were audio-recorded, professionally transcribed verbatim and analysed thematically. RESULTS: Five themes were identified: (1) In the dark: carers' experiences and reactions to BehSymp; (2) Others can see: the role of HCPs in identifying symptoms - and perceived opportunities for carers to receive support; (3) Shedding light: carers implementation and perceived impact of the MiNDToolkit content; (4) Acceptability and carers' engagement with MiNDToolkit; (5) Future implementation. Carers' experience of BehSymp was particularly distressing when symptoms were apparently out of context. MiNDToolkit appeared to support learning that BehSymp were part of MND. Content resonated with carers, who reported learning about the full picture of MND, which led to acceptance and use of newly learned strategies. Engagement with the platform was good, with varied input from HCPs. Greater and nuanced involvement from HCPs seem important to support management of BehSymp. Recommendations for a full-scale trial emerged, including adding a paper booklet to accompany the intervention and creation of new modules on emotional lability, changes in relationships, and transitioning to a care home. CONCLUSIONS: MiNDToolkit was acceptable to carers overall. Recommended improvements should be actioned in a full-scale trial.

Implementation of the MiNDToolkit intervention for the management of behavioral symptoms in MND by healthcare professionals: a mixed-methods process evaluation.

OBJECTIVE: MiNDToolkit is a novel psychoeducational intervention for carers to support management of behavioral symptoms in people living with motor neuron disease (PlwMND). Implementation of MiNDToolkit involves delivery of an online intervention to carers, which is reinforced by trained healthcare professionals (HCPs). METHODS: A mixed-methods process evaluation of the MiNDToolkit feasibility trial was conducted, focusing on reinforcement of the intervention by HCPs. Quantitative data, descriptively analyzed, were included from platform analytics, questionnaire, and 10 semi-structured interviews with HCPs. Interviews were transcribed verbatim; data were inductively analyzed using Reflective Thematic Analysis. RESULTS: The MiNDToolkit training and platform is a beneficial and acceptable resource for HCPs with potential to increase knowledge and confidence in identifying and managing behavioral symptoms in MND. Implementation barriers included HCPs' perceptions that highlighting behavior changes would be burdensome to carers and assumptions that carers would take the initiative to ask for support from clinicians. Degree of intervention reinforcement varied, with most HCPs delegating intervention delivery solely to the online platform. CONCLUSIONS: Implementation of the MiNDToolkit was viewed to be feasible and the platform thought to increase accessibility of support to carers. The flexible approach to delivery (online platform and optional HCP reinforcement) is acceptable as an intervention for supporting carers of PlwMND with behavioral symptoms. However, MiNDToolkit should not negate HCP involvement in providing medical and practical information to PlwMND and families. Future research should explore ways to incorporate support for carers in the management of PlwMND alongside standard care, alongside tools such as the MiNDToolkit.

Acceptance and Commitment Therapy plus usual care for improving quality of life in people with motor neuron disease (COMMEND): a multicentre, parallel, randomised controlled trial in the UK.

BACKGROUND: Motor neuron disease is a progressive, fatal neurodegenerative disease for which there is no cure. Acceptance and Commitment Therapy (ACT) is a psychological therapy incorporating acceptance, mindfulness, and behaviour change techniques. We aimed to evaluate the effectiveness of ACT plus usual care, compared with usual care alone, for improving quality of life in people with motor neuron disease. METHODS: We conducted a parallel, multicentre, two-arm randomised controlled trial in 16 UK motor neuron disease care centres or clinics. Eligible participants were aged 18 years or older with a diagnosis of definite or laboratory-supported probable, clinically probable, or possible familial or sporadic amyotrophic lateral sclerosis; progressive muscular atrophy; or primary lateral sclerosis; which met the World Federation of Neurology's El Escorial diagnostic criteria. Participants were randomly assigned (1:1) to receive up to eight sessions of ACT adapted for people with motor neuron disease plus usual care or usual care alone by a web-based system, stratified by site. Participants were followed up at 6 months and 9 months post-randomisation. Outcome assessors and trial statisticians were masked to treatment allocation. The primary outcome was quality of life using the McGill Quality of Life Questionnaire-Revised (MQOL-R) at 6 months post-randomisation. Primary analyses were multi-level modelling and modified intention to treat among participants with available data. This trial was pre-registered with the ISRCTN Registry (ISRCTN12655391). FINDINGS: Between Sept 18, 2019, and Aug 31, 2022, 435 people with motor neuron disease were approached for the study, of whom 206 (47%) were assessed for eligibility, and 191 were recruited. 97 (51%) participants were randomly assigned to ACT plus usual care and 94 (49%) were assigned to usual care alone. 80 (42%) of 191 participants were female and 111 (58%) were male, and the mean age was 63·1 years (SD 11·0). 155 (81%) participants had primary outcome data at 6 months post-randomisation. After controlling for baseline scores, age, sex, and therapist clustering, ACT plus usual care was superior to usual care alone for quality of life at 6 months (adjusted mean difference on the MQOL-R of 0·66 [95% CI 0·22-1·10]; d=0·46 [0·16-0·77]; p=0·0031). Moderate effect sizes were clinically meaningful. 75 adverse events were reported, 38 of which were serious, but no adverse events were deemed to be associated with the intervention. INTERPRETATION: ACT plus usual care is clinically effective for maintaining or improving quality of life in people with motor neuron disease. As further evidence emerges confirming these findings, health-care providers should consider how access to ACT, adapted for the specific needs of people with motor neuron disease, could be provided within motor neuron disease clinical services. FUNDING: National Institute for Health and Care Research Health Technology Assessment and Motor Neurone Disease Association.

An online intervention for carers to manage behavioral symptoms in motor neuron disease (MiNDToolkit): a randomized parallel multi-center feasibility trial.

BACKGROUND: Evidence on management of behavioral symptoms in motor neuron disease (MND) is lacking. The MiNDToolkit, an online psychoeducational platform, supports carers dealing with behavioral symptoms (BehSymp). The study objectives were to ascertain recruitment and retention rates, carer and healthcare professional (HCP) use of the platform, and completion of online assessments, to inform a full-scale trial. Design: Randomized, parallel, multi-center, feasibility trial. SETTING: England and Wales, across diverse MND services; recruitment from July/21 to November/22; last participant follow-up in March/23. PARTICIPANTS: Carers of people with motor neuron disease (PwMND) with BehSymp, recruited through MND services. After confirming eligibility, participants completed screening and baseline assessments online via the MiNDToolkit platform and were randomized centrally in a 1:1 ratio to MiNDToolkit or control. INTERVENTION: MiNDToolkit offered tailored modules to carers for the 3-month study period. Carers in the intervention group could receive additional support from MiNDToolkit trained HCPs. The control group was offered access to the intervention at the end of the study. Data were collected on platform usage and psychosocial variables. MAIN OUTCOMES: One hundred and fifty-one carers from 11 sites were invited to join the study (letter, face-to-face); 30 were screened; 29 were randomized. Fifteen people were allocated to the control arm; 14 to intervention. Carers were mostly female; median age for was 62.5 (IQR: 58, 68; intervention) and 57 (IQR: 56, 70; controls). Study retention was high (24/29 = 82.76%); carers engaged with the platform on average 14 times (median (IQR):14.0 (10.0, 18.5)) during the study period. CONCLUSION: The MiNDToolkit study was feasible and well accepted by carers and trained HCPs. A definitive trial is warranted.

End of life decision making when home mechanical ventilation is used to sustain breathing in Motor Neurone Disease: patient and family perspectives.

BACKGROUND: Motor Neurone Disease (MND) leads to muscle weakening, affecting movement, speech, and breathing. Home mechanical ventilation, particularly non-invasive ventilation (NIV), is used to alleviate symptoms and support breathing in people living with MND. While home mechanical ventilation can alleviate symptoms and improve survival, it does not slow the progression of MND. This study addresses gaps in understanding end-of-life decision-making in those dependent on home mechanical ventilation, considering the perspectives of patients, family members, and bereaved families. METHODS: A UK-wide qualitative study using flexible interviews to explore the experiences of people living with MND (n = 16), their family members (n = 10), and bereaved family members (n = 36) about the use of home mechanical ventilation at the end of life. RESULTS: Some participants expressed a reluctance to discuss end-of-life decisions, often framed as a desire to "live for the day" due to the considerable uncertainty faced by those with MND. Participants who avoided end-of-life discussions often engaged in 'selective decision-making' related to personal planning, involving practical and emotional preparations. Many faced challenges in hypothesising about future decisions given the unpredictability of the disease, opting to make 'timely decisions' as and when needed. For those who became dependent on ventilation and did not want to discuss end of life, decisions were often 'defaulted' to others, especially once capacity was lost. 'Proactive decisions', including advance care planning and withdrawal of treatment, were found to empower some patients, providing a sense of control over the timing of their death. A significant proportion lacked a clear understanding of the dying process and available options. CONCLUSIONS: The study highlights the complexity and evolution of decision-making, often influenced by the dynamic and uncertain nature of MND. The study emphasises the need for a nuanced understanding of decision-making in the context of MND.

Cost-effectiveness of acceptance and commitment therapy for people living with motor neuron disease, and their health-related quality of life.

BACKGROUND: Given the degenerative nature of the condition, people living with motor neuron disease (MND) experience high levels of psychological distress. The purpose of this research was to investigate the cost-effectiveness of acceptance and commitment therapy (ACT), adapted for the specific needs of this population, for improving quality of life. METHODS: A trial-based cost-utility analysis over a 9-month period was conducted comparing ACT plus usual care (n = 97) versus usual care alone (n = 94) from the perspective of the National Health Service. In the primary analysis, quality-adjusted life years (QALYs) were computed using health utilities generated from the EQ-5D-5L questionnaire. Sensitivity analyses and subgroup analyses were also carried out. RESULTS: Difference in costs was statistically significant between the two arms, driven mainly by the intervention costs. Effects measured by EQ-5D-5L were not statistically significantly different between the two arms. The incremental cost-effectiveness was above the £20,000 to £30,000 per QALY gained threshold used in the UK. However, the difference in effects was statistically significant when measured by the McGill Quality of Life-Revised (MQOL-R) questionnaire. The intervention was cost-effective in a subgroup experiencing medium deterioration in motor neuron symptoms. CONCLUSIONS: Despite the intervention being cost-ineffective in the primary analysis, the significant difference in the effects measured by MQOL-R, the low costs of the intervention, the results in the subgroup analysis, and the fact that ACT was shown to improve the quality of life for people living with MND, suggest that ACT could be incorporated into MND clinical services.

The Palliative Care Needs of Patients with Multiple Sclerosis, Parkinson's Related Diseases, and Motor Neurone Disease: A Secondary Analysis of the OPTCARE Neuro Trial Data.

Background: Long-term neurological conditions include multiple sclerosis, Parkinson's-related diseases, and motor neurone disease. National and international guidelines recommend a palliative approach for advancing neurological disease, but there is little research describing and comparing the palliative care needs of these patients side by side. Objective: The aim of this study was to describe and compare the symptom burden and psychological distress of patients with multiple sclerosis, Parkinson's-related diseases, and motor neurone disease. Design: A cross-sectional secondary analysis of the OPTCARE Neuro trial data was performed. Setting/Subjects: Recruitment was from seven sites across the United Kingdom. Patients aged 18 years or older, severely affected by advanced stages of multiple sclerosis or Parkinson's-related diseases or any stage of motor neurone disease, with an unresolved symptom, and one other issue despite usual care were eligible. Measurements: Baseline demographics, Integrated Palliative care Outcome Scale (IPOS) Neuro, and Hospital Anxiety and Depression Scale (HADS) results were analyzed. Results: Data from 348 participants were analyzed. The mean IPOS Neuro-S24 score was 27, with no statistical difference found between groups (p = 0.341). The most common symptoms were poor mobility (68.5%), problems using legs (63%), and fatigue (34.8%). The HADS revealed that a quarter of participants met the criteria for a diagnosis of anxiety and depression. Conclusions: Multiple sclerosis, Parkinson's-related disease, and motor neurone disease patients who were eligible for the OPTCARE Neuro trial have unmet needs in the form of distressing physical and psychological symptoms. It is unclear how to address these needs. The answer likely lies in a collaborative approach between neurology, palliative care, psychology, and specialized allied health professionals. Future work should focus on investigating this.

An introduction to neuropalliative care: A growing need.

Palliative care (PC) defined as 'an approach improving the quality of life of patients and their families facing problems associated with life-limiting illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial and spiritual' aims to enhance the improve the remaining time that patients have, emphasising choice for patients and families.1 Patients with neurological disease such as Parkinson's (PD) and motor neurone disease (MND) benefit from PC earlier in disease with increasing emphasis over time. Understanding and communicating uncertain trajectories, honest prognostic communication when patients are ready and careful symptom control has been shown to enhance quality of life in patients and caregivers, giving greater autonomy to these patients when supported in decision-making by a palliative approach. Although obstacles to palliative care are frequent, there are strategies which can help overcome them.

Fluctuating salience in those living with genetic risk of motor neuron disease: A qualitative interview study.

BACKGROUND: Motor neuron disease (MND) (also known as amyotrophic lateral sclerosis) is a life-limiting neurodegenerative condition. In up to 20% of people with MND, a pathogenic variant associated with autosomal dominant inheritance can be identified. Children of people carrying a pathogenic variant have a 50% chance of inheriting this and a higher, although harder to predict, chance of developing the disease compared to the general adult population. This paper explores the experience of living with the genetic risk of MND. METHODS: We undertook a UK-based interview study with 35 individuals, including: 7 people living with genetically-mediated forms of MND; 24 asymptomatic relatives, the majority of whom had an increased risk of developing the disease; and 4 unrelated partners. RESULTS: We explore how individuals make sense of genetic risk, unpacking the interplay between genetic knowledge, personal perception, experiences of the disease in the family, age and life stage and the implications that living with risk has for different aspects of their lives. We balance an emphasis on the emotional and psychological impact described by participants, with a recognition that the salience of risk fluctuates over time. Furthermore, we highlight the diverse strategies and approaches people employ to live well in the face of uncertainty and the complex ways they engage with the possibility of developing symptoms in the future. Finally, we outline the need for open-ended, tailored support and information provision. CONCLUSIONS: Drawing on wider literature on genetic risk, we foreground how knowledge of MND risk can disrupt individuals' taken-for-granted assumptions on life and perceptions of the future, but also its contextuality, whereby its relevance becomes more prominent at critical junctures. This research has been used in the development of a public-facing resource on the healthtalk.org website. PATIENT OR PUBLIC CONTRIBUTION: People with experience of living with genetic risk were involved throughout the design and conduct of the study and advised on aspects including the topic guide, sampling and recruitment and the developing analysis. Two patient and public involvement contributors joined a formal advisory panel.

Quality of life, cognitive and behavioural impairment in people with motor neuron disease: a systematic review.

PURPOSE: Motor neuron disease (MND) is a neurodegenerative disease, progressively impacting function and self-perceived quality of life (QoL). Up to 50% of people with MND can present with cognitive and behavioural impairment, with an associated increase in caregiver burden or strain. However, there has been no systematic exploration of the relationship between QoL and cognitive or behavioural impairment in MND. The aim was to determine if there is a relationship between QoL and cognitive/behavioural impairment in MND, while also supplementarily looking to determine the types of cognitive/behavioural and QoL measures utilised in these studies. METHODS: A systematic search was performed across multiple databases (PsychINFO, Embase, Medline, AMED) for research published up to the date of February 22, 2023. Studies utilising quantitative methods of measuring QoL, cognitive/behavioural functioning/impairment were included. Findings examining relationships between QoL-cognitive/behavioural impairment were extracted and synthesised. RESULTS: A total of 488 studies were identified, with 14 studies included in the systematic review. All 14 studies were observational (11 cross-sectional, 3 longitudinal). 13 studies utilised MND non-specific measures, particularly in relation to QoL and cognitive impairment. Of 8 studies measuring behavioural impairment 62.5% (N = 5) found either a lower QoL difference or association. Only 33.3% (N = 4) of 12 studies measuring cognitive impairment found a lower QoL difference or association. CONCLUSIONS: This systematic review shows that behavioural impairment may have an impact on QoL in MND. There is variability in types of assessments used to measure QoL and also cognitive/behavioural impairment, most of which are disease-non-specific. Recommendations for future research are to use comprehensive disease-specific, multidomain measures to further elucidate the QoL-cognitive/behavioural impairment relationship.

Understanding neck collar preferences and user experiences in motor neuron disease: A survey-based study.

BACKGROUND: Motor Neurone Disease (MND), is a debilitating neurodegenerative condition, which significantly impacts the quality of life of those affected. Neck weakness is one challenge faced by those living with MND and as such may require a neck collar to assist. However, the user experience and requirements related to these neck collars have not been comprehensively explored. Understanding these priorities is crucial for enhancing the well-being of MND patients. OBJECTIVE: To understand the priorities of people living with Motor Neurone Disease (MND) including user experience, requirements and the importance of neck collars used to aid neck weakness. METHODS: An online survey was used to investigate the perspectives and experiences of off the shelf neck collars used by people living with MND. The MND Association was selected as a strategic partner by their affiliations and access to large data base of MND patients. RESULTS: Survey highlighted a disparity between the actual duration MND patients wear their current neck collars and their desired duration, emphasising the need to integrate collars into daily activities. Key areas for improvement with existing neck collars centred on comfort and reduced restriction, with respondents expressing a preference for collars that offer support without impeding movement. Additionally, addressing pressure on the anterior neck region during collar use emerged as a critical requirement. CONCLUSION: Current collars do not cause any clinical complications; however, they do fall short of meeting the expected needs of people living with MND, including discomfort, restricted movement, and pressure to the anterior region of the neck. This study highlights need to improve current collar designs to provide better quality of life for MND patients.

Exploring clinical management of cognitive and behavioural deficits in MND. A scoping review.

OBJECTIVES: Little is known about how cognitive and behavioural decline in MND is managed clinically. This review aimed to summarise clinical management approaches of cognitive and behavioural decline in MND reported in peer-reviewed and grey literature. METHODS: A scoping review was conducted across Embase, Medline, Psychinfo and Emcare in October 2022. Grey literature was also searched across Google Scholar and Google in October 2022. RESULTS: A total of N = 26 studies and 8 documents were included. Thematic analysis revealed six key areas of clinical management: i. Assessment, ii. Education, iii. Advance Care Planning, iv. Adaptation of Care Plan, v. Communication and vi. Carer Support. CONCLUSIONS: The literature on management of cognitive and behavioural decline in MND is sparse. Most peer-reviewed literature consists of expert commentary and there is a lack of primary data to guide practitioners and families on how to manage cognitive and behavioural change in MND. PRACTICE IMPLICATIONS: Determining as early as practicable the presence of cognitive and behavioural changes in pwMND will enable practitioners to make adaptations to communication, provide education and supported decision-making for forward planning. This will enable individualised care, planned in partnership with families with MND, which incorporates personal needs and wishes.

Factors influencing decisions people with motor neuron disease make about gastrostomy placement and ventilation: A qualitative evidence synthesis.

BACKGROUND: People with motor neuron disease (pwMND) are routinely offered gastrostomy feeding tube placement and (non-invasive and invasive) ventilation to manage the functional decline associated with the disease. This study aimed to synthesise the findings from the qualitative literature to understand how individual, clinical team and organisational factors influence pwMND decisions about these interventions. METHODS: The study design was guided by the enhancing transparency in reporting the synthesis of qualitative research (ENTREC) statement. The search of five bibliography databases and an extensive supplementary search strategy identified 27 papers that included qualitative accounts of pwMND, caregivers and healthcare professionals' (HCPs) experiences of making decisions about gastrostomy and ventilation. The findings from each study were included in a thematic synthesis. FINDINGS: Making decisions about interventions is an emotional rather than simply a functional issue for pwMND. The interventions can signal an end to normality, and increasing dependence, where pwMND consider the balance between quality of life and extending survival. Interactions with multiple HCPs and caregivers can influence the process of decision-making and the decisions made. These interactions contribute to the autonomy pwMND are able to exert during decision-making. HCPs can both promote and threaten pwMND perceived agency over decisions through how they approach discussions about these interventions. Though there is uncertainty over the timing of interventions, pwMND who agree to interventions report reaching a tipping point where they accept the need for change. CONCLUSION: Discussion of gastrostomy and ventilation options generate an emotional response in pwMND. Decisions are the consequence of interactions with multiple external agents, including HCPs treading a complex ethical path when trying to improve health outcomes while respecting pwMND right to autonomy. Future decision support interventions that address the emotional response and seek to support autonomy have the potential to enable pwMND to make informed and timely decisions about gastrostomy placement and ventilation. PATIENT OR PUBLIC CONTRIBUTION: The lead author collaborated with several patient and participant involvement (PPI) groups with regards to the conceptualisation and design of this project. Decisions that have been influenced by discussions with multiple PPI panels include widening the scope of decisions about ventilation in addition to gastrostomy placement and the perceptions of all stakeholders involved (i.e., pwMND, caregivers and HCPs).

Methods for informing people with amyotrophic lateral sclerosis/motor neuron disease of their diagnosis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), causes increasing physical impairment and disability. People with ALS/MND face huge physical challenges, and the diagnosis can be a source of great psychological distress for both people with ALS/MND and their carers. In such a context, how news of the diagnosis is broken is important. At present, there are no systematic reviews of methods for informing people with ALS/MND of their diagnosis. OBJECTIVES: To examine the effects and effectiveness of different methods for informing people of a diagnosis of amyotrophic lateral sclerosis/motor neuron disease (ALS/MND), including effects on the person's knowledge and understanding of their disease, its treatment, and care; and on coping and adjustment to the effects of ALS/MND, its treatment, and care. SEARCH METHODS: We searched the Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, PsycINFO, and two trials registers (February 2022). We contacted individuals or organisations to locate studies. We contacted study authors to obtain additional unpublished data. SELECTION CRITERIA: We planned to include randomised controlled trials (RCTs) and quasi-RCTs of techniques for informing people with ALS/MND of their diagnosis. We planned to include adults (aged 17 years or over) with ALS/MND, according to the El Escorial criteria. DATA COLLECTION AND ANALYSIS: Three review authors independently reviewed the results of the search to identify RCTs, and three review authors identified non-randomised studies to include in the discussion section. We planned that two review authors would independently extract data, and three would assess the risk of bias in any included trials. MAIN RESULTS: We did not identify any RCTs that met our inclusion criteria. AUTHORS' CONCLUSIONS: There are no RCTs that evaluate different communication strategies for breaking the bad news for people diagnosed with ALS/MND. Focused research studies are needed to assess the effectiveness and efficacy of different communication methods.

Requested withdrawal of gastrostomy feeding in motor neurone disease.

NICE (National Institute for Health & Care Excellence) guidance recommends that healthcare professionals with expertise in palliative care should be an integral part of the multidisciplinary team in managing patients with motor neuron disease (MND). Those in the poorest prognostic group may benefit from early referral to help manage rapidly progressive symptoms, psychological distress and offer additional support with complex decision-making and early robust advance care planning. Patients frequently develop dysphagia and gastrostomy feeding can be used to prolong survival and improve quality of life. As the disease progresses patients may request withdrawal of life-sustaining treatment such as gastrostomy feeding; however, a literature search found no evidence or guidance on how best to facilitate this. We present the case of a patient with MND admitted to the hospice inpatient unit requesting withdrawal of gastrostomy feeding, outline the challenges and need for further consensus guidelines to inform practice.

The impact of communication on healthcare involvement for people living with motor neurone disease and their carers: A longitudinal qualitative study.

BACKGROUND: Communication and cognitive impairments are known barriers to shared decision-making. Most people diagnosed with motor neurone disease (MND) will develop a motor speech impairment over the disease course. Some will develop cognitive, linguistic or behavioural disturbance. Despite this, the impact of communication and cognitive impairment on personal healthcare decision-making in MND is not well known. AIMS: This exploratory, longitudinal study aimed to capture the perspectives of people living with MND (plwMND) and family members on managing their healthcare with, or in anticipation of, a communication impairment. METHODS & PROCEDURES: Semi-structured interviews and functional assessments were conducted with plwMND and family members over one to three time points between December 2017 and January 2020. Participants were recruited from a specialist MND clinic using a maximum variation sampling approach. Interview transcripts were analysed using trajectory data analysis: a matrix-based approach for thematic analysis of longitudinal data. The study was underpinned by interpretive descriptive methodology. OUTCOMES & RESULTS: A total of 19 plwMND with a range of MND phenotypes and 15 family members were recruited. Disease progression and participant withdrawal resulted in attrition, however 12 plwMND and seven family members participated at all three time points. Consistent cognitive screening was not feasible, which limited the opportunity to explore the impact of cognitive change. An overarching theme 'Communicating takes effort' was identified and illustrates the efforts required to compensate for, or circumnavigate, impairments to maintain involvement in healthcare. Assistance from family and accommodation from healthcare professionals (HCPs) was needed for ongoing engagement. Where plwMND were dependent on alternative communication devices, this assistance was essential and primarily carried out by family members. Despite these efforts, the quality, quantity and accuracy of communication were sometimes compromised. Participants equated good communication with receiving good healthcare, and some expressed anxiety in the anticipation of being unable to express their needs to healthcare workers. CONCLUSION & IMPLICATIONS: Communication impairment has a direct impact on healthcare involvement. This study demonstrates the effort required by plwMND and their carers to maintain or maximize ongoing involvement. This effort may not always be visible to HCPs. This information may prompt clinicians to consider the best ways to conduct clinical consultations to accommodate patients' abilities. Compromised communication experiences can be moderated by accommodations and support from HCPs and appropriate adjustments in the health system. Asking patients about their communication preferences and needs, allowing extra time and conducting multidisciplinary sessions are examples of such support. WHAT THIS PAPER ADDS: What is already known on this subject? Communication and cognitive impairments are known contributors to negative health outcomes and barriers to shared decision-making generally. The existing literature in decision-making in MND does not address the specific impact of these impairments on personal healthcare involvement for plwMND and their carers. What this paper adds to existing knowledge? This paper reports the findings of a research project that interviewed 19 plwMND and 15 carers on one to three occasions over a 26-month period to obtain their perspectives of the impact of communication on healthcare involvement. Whilst a priori the intention was to look at both communicative and cognitive decline, only the former was achieved. The effort and often 'invisible' activity undertaken to manage or maintain involvement in healthcare is identified. Communication impairment requires support and accommodation, otherwise healthcare involvement can be compromised. Results show participants may associate effective communication with good healthcare. What are the potential or actual clinical implications of the work? Clinicians may wish to use these insights from plwMND and their carers to guide adjustments to their professional practice to maximize healthcare involvement for their patients. Tailored education for different healthcare groups is needed to improve understanding of MND-related communication impairments and supportive strategies so that involvement in healthcare is not compromised.

Flexibility to manage and enhance quality of life among people with motor neurone disease.

OBJECTIVES: To identify influential factors for quality of life (QoL) among individuals with motor neurone disease (MND) and explore how regulatory flexibility and psychological flexibility may contribute towards maintaining and improving QoL. METHODS: Semi-structured interviews were conducted with 26 individuals with MND. Thematic analysis, using both inductive and deductive analyses, was employed to examine subjective QoL in view of previous understanding of QoL. RESULTS: Four factors were important for the QoL of participants: perceived illness prognosis, sense of self, concerns for significant others, and life to enjoy. These factors reflected psychological stress caused by MND, the participant's value system, and their beliefs about life. In optimising QoL, both regulatory flexibility and psychological flexibility were essential to maintain and enhance QoL. Often, regulatory flexibility was perceived among those employing a mindful approach, and psychological flexibility was found to involve savouring positive experiences. CONCLUSIONS: People with MND reported that seeking ways to both maintain and enhance their QoL is crucial, and that this may be accomplished by increasing flexibility through mindfulness and savouring. (171/200 words)Implications for rehabilitationPeople with motor neurone disease (MND) seek to maintain quality of life (QoL) following changes caused by the condition, whilst attempting to enhance QoL by maximising their positive experiences.Regulatory flexibility is essential to maintain QoL and it was closely associated with mindful approach so that MND is not perceived as an inevitable threat to QoL.Psychological flexibility was found to enhance QoL and it involves savouring positive experiences, while abandoning fault-finding of the current situation.Positive inter-personal interactions can encourage people with MND to engage with mindfulness and savouring for favourable outcomes in terms of QoL; current support services should orient towards both mindfulness and savouring.

A qualitative meta-synthesis of evidence (meta-ethnography) exploring the personal experiences of gastrostomy tube in neurodegenerative diseases: a case of motor neurone disease.

PURPOSE: The lived experiences of a gastrostomy tube (GT) in adults with neurodegenerative diseases (NDDs) are not well understood. The aim of this qualitative meta-synthesis was to review and synthesise the available evidence to inform clinical practice and identify research gaps. METHODS: Meta-ethnographic synthesis of qualitative studies was conducted with systematic searching of eight databases from inception to March 2021. Qualitative studies reporting personal experiences of GTs in adults with NDDs were identified. New theories were developed during translation of concepts from each study and combined as a "line-of-argument" synthesis. Patient and public involvement was incorporated as two of the authors are living with an NDD and a GT. RESULTS AND CONCLUSIONS: Of 2863 unique records identified, only nine fulfilled the review criteria. All studies recruited participants with motor neurone disease (MND); no other NDDs were represented. Two main themes emerged: decision making and living with GT. Decision making was the predominant theme and data regarding living with GT were sparse. There is limited research on the lived experience of a GT in adults with NDDs. The lived experience of GT in MND is complex and individualised. Future research is indicated to inform clinical practice.Implications for rehabilitationEvidence related to the lived experiences of gastrostomy tube (GT) in neurodegenerative diseases (NDDs) is lacking especially in relation to the time after GT insertion.Decision making in relation to GT is a complex and individualised psychological process for some people, while others perceive no decisional conflict.Support from healthcare professionals is crucial during the decision-making time and should not cease after GT insertion.Support from healthcare professionals can help resolve any clinical complications and also incorporate GT into everyday routines.Healthcare professionals should be aware that their views on the benefits and problems related to GT may differ to those of individuals with a NDD.