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Objective: Understand the practice and beliefs of healthcare professionals (HCPs) supporting the decision-making of people with MND (pwMND) about gastrostomy placement, including identifying differences between professions., Methods: An online cross-sectional survey disseminated to HCPs who support the decision-making of pwMND about gastrostomy placement., Results: A total of 139 participants completed the survey including representation from a range of healthcare professions. A third (36/101, 36%) initiated discussions about gastrostomy later in practice than they believed was ideal. In relation to the outcome of declining compared to accepting gastrostomy, participants were more likely to discuss aspiration (80% vs. 68%), choking (76% vs. 58%) and prognosis (36% vs. 22%). Participants believed gastrostomies should be placed after a mean 8.1% weight loss since symptom-onset. More participants favored gastrostomy placement before pwMND presented with respiratory symptoms (45%) compared to onset of dysphagia (11%). Half believed pwMND placed gastrostomies too late. Participants were more likely to 'often'/'always' recommend pwMND to have a gastrostomy (23%) than continue without (7%) or decline (4%) gastrostomy, when believing these were the best option for pwMND. Nurses and dietitians discussed the broadest range of information, while doctors were more likely to discuss mortality risk and prognosis., Conclusion: There is variation in HCPs practice and beliefs about initiating discussions, the sharing of information and recommendations, and timing, about gastrostomy placement. The information shared varies by profession and there is evidence of sub-optimal communication between HCPs. Further research is required to understand how these findings may impact on the decision-making of pwMND about gastrostomy.

Genetic testing is a key decision-making point for people with motor neuron disease (MND); to establish eligibility for clinical trials, better understand the cause of their condition, and confirm the potential risk to relatives, who may be able to access predictive testing. Given the wide-reaching implications of MND genetic and predictive testing, it is essential that families are given adequate information, and that staff are provided with appropriate training. In this report we overview the information resources available to people with MND and family members around genetic testing, and the educational and training resources available to staff, based on information obtained through a freedom of information request to UK-based NHS Trusts. MND Association resources were most commonly used in information sharing, though we highlight distinctions between neurology and genetics centers. No respondents identified comprehensive training around MND genetic testing. We conclude with practice implications and priorities for the development of resources and training.

Objective: This study uses three linked datasets to provide an estimate of incidence of motor neuron disease (MND) in England from 1998 to 2019. Comparison is made to previous British studies. It examines age at diagnosis and ethnicity of those affected. Methods: The literature was searched for studies of MND incidence in Great Britain from 1995 to date. The QResearch and linked Hospital Episode Statistics and Death register databases were searched from 1998 to 2019 for cases of MND, and incidence calculated from 16.8 million adults and 112 million adult years of data. Results: We found 6437 adults with a diagnosis of MND giving an incidence of MND of 5.69/100,000 person years (95% CI 5.51-5.88); 6.57 (6.41-6.99) in men and 4.72 (4.49-4.97) in women when age-standardized to the 2011 UK population. The median age of diagnosis was 72 years. Peak incidence occurred in the 80-84 year age group in men and 75-79 in women. Age-standardized incidence was as high in Bangladeshi, Black Caribbean, Indian, other Asian and Pakistani people as in White people. Black African and Chinese people had a lower incidence. Conclusion: The use of three linked national datasets captured 33% more people than a primary care dataset alone. Patients were older than in previous studies and rates were high in all ethnic groups studied except Black African and Chinese people. We present the highest incidence of MND reported globally in the past 50 years. Methodological differences may in part explain differences with previous reports. The use of national datasets may have captured additional MND patients with serious comorbidities who have not seen a neurologist before death. A limitation of this approach is that unlike population registers, which minimize false positive diagnosis by neurologist review of each patient, we cannot review diagnosis for individuals as data are anonymized.

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). [ABSTRACT FROM AUTHOR]

The Covid-19 pandemic has impacted healthcare. Our aim was to identify how amyotrophic lateral sclerosis (ALS) care in the UK has been affected by the pandemic by exploring the experiences of people living with ALS (plwALS), healthcare professionals (HCPs) working with plwALS, and ALS care centers. Three surveys were carried out to explore the experiences of plwALS, HCPs and ALS care centers during the pandemic. Quantitative data were analyzed using descriptive and inferential statistics and triangulated with the qualitative data which were analyzed thematically. Responses from 53 plwALS, 73 HCPs and 23 ALS care centers were analyzed. Five main themes were identified: keeping safe, losses, negative emotions, delivering care and alternative care delivery in a pandemic. PlwALS and HCPs felt that care was sub-optimal as a result of the pandemic. Changes to care included longer waiting times and face-to-face appointments being canceled or replaced by virtual consultations. While benefits of virtual consultations were reported, concerns were raised about incomplete clinical assessments and the disruption of provision of testing and interventions. ALS care has changed as a result of the pandemic. Patients have had a lack of face-to-face contact with HCPs and have experienced delays to investigations and treatments. PlwALS and HCPs were concerned about the impact of this change, but the long-term implications remain unclear. We propose recommendations for HCPs caring for plwALS, that will promote continuity of evidenced based care in the context of a pandemic. [ABSTRACT FROM AUTHOR]

Background: Motor neuron disease (MND) is a rapidly progressive, fatal neurodegenerative disease that predominantly affects motor neurons from the motor cortex to the spinal cord and causes progressive wasting and weakening of bulbar, limb, abdominal and thoracic muscles. Prognosis is poor and median survival is 2-3 years following symptom onset. Psychological distress is relatively common in people living with MND. However, formal psychotherapy is not routinely part of standard care within MND Care Centres/clinics in the UK, and clear evidence-based guidance on improving the psychological health of people living with MND is lacking. Previous research suggests that Acceptance and Commitment Therapy (ACT) may be particularly suitable for people living with MND and may help improve their psychological health.Aims: To assess the clinical and cost-effectiveness of ACT modified for MND plus usual multidisciplinary care (UC) in comparison to UC alone for improving psychological health in people living with MND.Methods: The COMMEND trial is a multi-centre, assessor-blind, parallel, two-arm RCT with a 10-month internal pilot phase. 188 individuals aged ≥ 18 years with a diagnosis of definite, laboratory-supported probable, clinically probable, or possible familial or sporadic amyotrophic lateral sclerosis, and additionally the progressive muscular atrophy and primary lateral sclerosis variants, will be recruited from approximately 14 UK-based MND Care Centres/clinics and via self-referral. Participants will be randomly allocated to receive up to eight 1:1 sessions of ACT plus UC or UC alone by an online randomisation system. Participants will complete outcome measures at baseline and at 6- and 9-months post-randomisation. The primary outcome will be quality of life at six months. Secondary outcomes will include depression, anxiety, psychological flexibility, health-related quality of life, adverse events, ALS functioning, survival at nine months, satisfaction with therapy, resource use and quality-adjusted life years. Primary analyses will be by intention to treat and data will be analysed using multi-level modelling.Discussion: This trial will provide definitive evidence on the clinical and cost-effectiveness of ACT plus UC in comparison to UC alone for improving psychological health in people living with MND.Trial Registration: ISRCTN Registry, ISRCTN12655391. Registered 17 July 2017, https://www.isrctn.com/ISRCTN12655391 .Protocol Version: 3.1 (10/06/2020). [ABSTRACT FROM AUTHOR]

The HeadUp collar (previously known as the Sheffield Support Snood) provides support for neck weakness caused by amyotrophic lateral sclerosis (ALS) and has shown to be superior to alternative options in a small cohort of patients from one single center. Here we report the assessment of the HeadUp collar in a larger cohort of patients, exploring the use in other neurological conditions and expanding to other centers across the UK and Ireland. An interventional cross-sectional study design was implemented to investigate the usability and acceptability of the HeadUp collar. A total of 139 patients were recruited for the study, 117 patients had a diagnosis of ALS and 22 patients presented with neck weakness due to other neurological conditions. Participants were assessed at baseline, fitted a HeadUp collar and followed-up one month later. The performance of the HeadUp collar was rated favorably compared to previously worn collars in terms of the ability to eat, drink and swallow. Findings suggest that the collar also permitted a more acceptable range of head movements whilst maintaining a good level of support. We conclude that the HeadUp collar is a suitable option for patients with neck weakness due to ALS and other neurological conditions. [ABSTRACT FROM AUTHOR]

Objectives: Patient and public involvement (PPI) is a relatively new practice whereby researchers involve patients and the public in the conduct of their research. The Sheffield Motor Neurone Disorders Research Advisory Group (SMNDRAG) is one of the first groups to specialise in motor neuron disease (MND). Its members include people living with MND, carers, relatives, volunteers, clinicians, and scientists. Our aim was to explore the experiences of those who participate in, organise and work with the SMNDRAG. Methods: We conducted 13 semi-structured interviews: 10 with members of the SMNDRAG and three with researchers who have worked with the group. We used thematic analysis to identify ways in which the group influenced research and the barriers and enablers to PPI. Results: A number of motivations for participating in the SMNDRAG were reported but the majority were altruistic. The SMNDRAG offered individuals psychosocial and intellectual benefits. The SMNDRAG has overcome a number of practical and psychological barriers to developing a successful and effective collaborative partnership resulting in a positive impact on research and researchers at each stage of the research process. For example, the group identified research priorities which ensured that research was patient-focused. However, several barriers remain, including the lack of diversity within the group and the perception that PPI participation requires a "certain type of person." Conclusions: PPI can make a valuable contribution to all aspects of research and can have a positive impact on those involved. We recommend ways in which PPI can and should be incorporated into research. [ABSTRACT FROM AUTHOR]

Objective: Understanding the use of coping strategies and which factors are associated with strategy utilization might help clinical staff anticipate which coping strategies individuals are more likely to utilize. In this study, we assess coping strategy use in the motor neuron disease (MND, also known as amyotrophic lateral sclerosis [ALS]) population and examine associations of demographic and disease variables with individual coping strategies. Participants and Methods: A total of 233 participants with MND/ALS were recruited into the ongoing Trajectories of Outcomes in Neurological Conditions study from MND clinics across the United Kingdom. Participants completed a questionnaire pack collecting data on demographics and a range of patient‐reported measures including the Coping Orientations to Problems Experienced scale. Associations between demographic and clinical characteristics and coping strategies were examined by simple and multiple ordinal logistic regression. Results: The most commonly used strategy was Acceptance, followed by Active Coping, Planning and Positive Re‐interpretation and Growth. The least used strategies were Substance Use, Turning to Religion and Denial. Ten out of the fifteen strategies showed statistically significant associations with demographic and clinical characteristics. Most markedly, females were found to utilize many strategies more than males, namely Restraint, Seeking Instrumental Social Support, Seeking Emotional Social Support, Focus on and Venting of Emotions, Behavioural Disengagement and Mental Disengagement. Conclusion: Clinical staff should be aware that coping strategy use is associated with several demographic and disease characteristics. Targeted advice on coping may improve coping capacity and facilitate psychosocial adjustment. [ABSTRACT FROM AUTHOR]

Background: The diagnosis of motor neurone disease (MND) is known to be challenging and there may be delay in patients receiving a correct diagnosis. This study investigated the referral process for patients who had been diagnosed with MND, and whether a newly-developed tool (The Red Flags checklist) might help General Practitioners (GPs) in making referral decisions.Methods: We carried out interviews with GPs who had recently referred a patient diagnosed with MND, and interviews/surveys with GPs who had not recently referred a patient with suspected MND. We collected data before the Red Flags checklist was introduced; and again one year later. We analysed the data to identify key recurring themes.Results: Forty two GPs took part in the study. The presence of fasciculation was the clinical feature that most commonly led to consideration of a potential MND diagnosis. GPs perceived that their role was to make onward referrals rather than attempting to make a diagnosis, and delays in correct diagnosis tended to occur at the specialist level. A quarter of participants had some awareness of the newly-developed tool; most considered it useful, if incorporated into existing systems.Conclusions: While fasciculation is the most common symptom associated with MND, other bulbar, limb or respiratory features, together with progression should be considered. There is a need for further research into how decision-support tools should be designed and provided, in order to best assist GPs with referral decisions. There is also a need for further work at the level of secondary care, in order that referrals made are re-directed appropriately. [ABSTRACT FROM AUTHOR]

Introduction: The contribution of cranial and thoracic region electromyography (EMG) to diagnostic criteria for amyotrophic lateral sclerosis (ALS) has not been evaluated.Methods: Clinical and EMG data from each craniospinal region were retrospectively assessed in 470 patients; 214 had ALS. Changes to diagnostic classification in Awaji-Shima and revised El Escorial criteria after withdrawal of cranial/thoracic EMG data were ascertained.Results: Sensitivity for lower motor neuron involvement in ALS was highest in the cervical/lumbar regions; specificity was highest in cranial/thoracic regions. Cranial EMG contributed to definite/probable Awaji-Shima categorization in 1.4% of patients. Thoracic EMG made no contribution. For revised El Escorial criteria, cranial and thoracic data reclassified 1% and 5% of patients, respectively.Conclusion: Cranial EMG data make small contributions to both criteria, whereas thoracic data contribute only to the revised El Escorial criteria. However, cranial and thoracic region abnormalities are specific in ALS. Consideration should be given to allowing greater diagnostic contribution from thoracic EMG. Muscle Nerve 54: 378-385, 2016. [ABSTRACT FROM AUTHOR]

Current practice and guidelines recommend the use of neck orthoses for people with amyotrophic lateral sclerosis (ALS) to compensate for neck weakness and to provide surrogate neck control. However, available options are frequently described by patients as restrictive and unsuitable and there was a need for a new device that addressed the needs of people with ALS. This project utilized a co-design process to develop a new neck orthosis that was more flexible yet supportive. Following development of a prototype device, a mixed methods cohort study was undertaken with patients and carers, in order to evaluate the new orthosis. Twenty-six patients were recruited to the study, with 20 of these completing all phases of data collection. Participants described the impact of neck weakness on their life and limitations of existing supports. Evaluation of the new orthosis identified key beneficial features: notably, increased support while providing a greater range of movement, flexibility of use, and improved appearance and comfort. In conclusion, the results of this evaluation highlight the value of this alternative option for people with ALS, and potentially other patient groups who require a neck orthosis. [ABSTRACT FROM PUBLISHER]

Gastrostomy feeding is commonly used to support MND patients with dysphagia. In this paper we review three main methods of gastrostomy insertion (PEG, RIG, PIG); conduct a meta-analysis of mortality data following gastrostomy; and present a survey of current practice. A review of the literature revealed a lack of high quality evidence to indicate the optimal method and timing for gastrostomy insertion in patients with MND. A survey of 20 MND clinics demonstrated a clinic-based variability of gastrostomy practices due to factors such as clinician preference, availability of method, and patient respiratory function. The meta-analysis demonstrated that the estimate of the absolute difference in mortality rates was 2.1% higher for PEG (− 6.3%, + 11.2%), suggesting that RIG and PIG methods may be safer than PEG. These results and observations highlight the need for more research to evaluate and compare the safety of the differerent gastrostomy insertion methods in MND care. [ABSTRACT FROM AUTHOR]

This study aimed to examine the experiences of patients with motor neuron disease and their carers following the recommendation to use non-invasive ventilation (NIV). Qualitative interviews were carried out with 20 patients and 17 carers within one month of NIV being initiated. The study identified a range of potential barriers to usage including: adverse impressions of the technology; sleep disturbance; the sensation of pressure and pulsing; dry mouth; and mask design issues. Patients/carers perceived benefits related to: increased energy; improved sleeping; enhanced carer well-being; improved breathing and increased speech clarity. A key factor described by patients and carers was the need to persevere to overcome the challenges associated with early NIV use. The study highlights the importance of patient perceptions of gains as a factor in their NIV usage decisions. While recognizing that older individuals with limb-onset disease were over-represented in the sample, key recommendations from the study are: availability of easily accessible in-person support for patients; for clinicians to pre-empt potential obstacles by discussing options such as humidification or alternative mask interfaces; the importance of discussing potential benefits in detail with patients; and optimization of secretion management prior to NIV trial. [ABSTRACT FROM AUTHOR]

Many centres in the UK care for patients with motor neuron disease (MND) in a multidisciplinary clinic (MDC). It has been demonstrated that such care results in better prognosis for survival than care from a general neurology clinic (GNC). Whether this is due to higher use of disease-modifying interventions or an independent factor of attendance at a specialist clinic has not been established. Hence, we performed a retrospective review of hospital notes of patients with MND who were diagnosed and followed up in a GNC between 1998 and 2002 and in an MDC between 2006 and 2010. Overall, 162 patients attended a GNC, and 255 attended the MDC. The median survival from diagnosis was 19 months for patients who attended the MDC, compared to 11 months for those attending the GNC (hazard ratio 0.51, 95% CI 0.41-0.64). The Cox hazards model identified attendance at an MDC as an independently positive prognostic factor (HR 1.93, 95% CI 1.37-2.72, p < 0.001). We concluded that care at an MDC improves survival. While this effect is augmented by the increased use of riluzole, NIV and PEG, the data suggest that coordinated care independently improves the prognosis of MND patients. [ABSTRACT FROM AUTHOR]