Charity financial support to motor neuron disease (MND) patients in Greater London: the impact of patients’ socioeconomic status—a cross-sectional study

<h3>Objective</h3> There is an immense socioeconomic burden for both the patients with motor neuron disease (MND) and their families. The aim of this study is to evaluate the extent to which the provision offered by the Motor Neurone Disease Association is distributed among patients with MND living in the ethnically and socially diverse area of Greater London, according to the patients’ socioeconomic situation and needs. <h3>Setting</h3> Greater London, where age and sex-adjusted prevalence rates of MND in 2016 were calculated. <h3>Participants</h3> Prevalent MND cases in Greater London, using anonymised data extracted from the Association’s database. <h3>Exposure</h3> Demographic and socioeconomic characteristics <h3>Primary and secondary outcome measures</h3> Receiving a Motor Neurone Disease Association grant, and the amount of money received. <h3>Results</h3> 396 individuals with amyotrophic lateral sclerosis were detected, the age-specific and sex-specific prevalence of MND was 4.02 per 100 000 inhabitants, higher among men (5.13 per 100 000) than women (3.01 per 100 000). Men were statistically significantly 40% less likely to receive a grant compared with women; among grant recipients, the younger the age of the participant, the higher the size of the grant received. The Index of Multiple Deprivation was not associated with either receiving a grant nor the amount of money received, among recipients. <h3>Conclusion</h3> Financial support by the Motor Neurone Disease Association is provided across individuals and across boroughs regardless of their socioeconomic circumstances. Differences that benefits women and younger patients were detected.

BackgroundMotor 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. ObjectiveTo 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. MethodsAn 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. ResultsSurvey 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. ConclusionCurrent 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.

Background:Studies on the experiences of consumers with Motor Neurone Disease Associations at end of life and bereavement are lacking, and their role and capability within the broader sectors of health and disability are unknown.Objectives:To ascertain the experiences and views of bereaved motor neurone disease caregivers with Motor Neurone Disease Associations about service gaps and needed improvements before and during bereavement and to propose a model of care that fits with consumer preferences and where Motor Neurone Disease Associations are effective enablers of care.Methods:A national bereavement survey was facilitated in 2019 by all Motor Neurone Disease Associations in Australia. A total of 363 respondents completed the section on support provided by Motor Neurone Disease Associations. A mixed-method design was used.Results:Respondents were generally positive about support received before bereavement (73-76%), except for emotional support (55%). Positive experiences related to the following: information, equipment advice/provision, advocacy/linking to services, showing empathy/understanding, personal contact and peer social support. Negative experiences included lack of continuity in case management and contact, perceived lack of competence or training, lack of emotional support and a lack of access to motor neurone disease services in rural areas. Suggested improvements were as follows: more contact and compassion at end of life and postdeath; better preparation for end of life; option of discussing euthanasia; providing referrals and links for counseling; access to caregiver support groups and peer interaction; provision of a genuine continuum of care rather than postdeath abandonment; guidance regarding postdeath practicalities; and more access to bereavement support in rural areas.Conclusion:This study provides consumer perspectives on driving new or improved initiatives by Motor Neurone Disease Associations and the need for a national standardised approach to training and service delivery, based on research evidence. A public health approach to motor neurone disease end-of-life care, of international applicability, is proposed to address the needs and preferences of motor neurone disease consumers, while supporting the capability of Motor Neurone Disease Associations within a multidisciplinary workforce to deliver that care.

In 2016, twenty people with Motor Neurone Disease (MND) in the Lancashire and South Cumbria region were admitted and died in hospital. Following a meeting in early 2016 with the MND Association, the local hospices were approached to consider how the MND ice bucket challenge money could be utilised for future MND patient support. From this, local MND Association staff met with St John’s Hospice staff to look at how the hospice could support MND patients through our already existing services. The group looked at the gaps in services and staff knowledge, what causes a crisis leading to hospital admission, and what skills are required to keep people with MND at home, if that is their preferred place of care and death. The main issues often leading to admission were documenting and managing advance care planning; supporting cognitive impairment difficulties; and supporting family carers using practical skills such as taking blood gases, managing non-invasive ventilation (NIV) and using cough assist devices. For family support and education, the day services team set up a ‘STAR’ group (Support, Time-out, Advice, Recovery: named by the attendees) for people with MND and their main carer. This addressed advance care planning; hospice services and what they can provide; support to carers; nutrition, and speech and language advice. This would run weekly, following on from the quarterly MND clinics, for four weeks. The team also set up a successful MND study day for professionals, which was well evaluated and covered diagnosis, prognosis, and symptom management. The Hospice at Home and ward teams are presently being developed in the practical skills listed above, in order to support people to stay in their own homes when near end of life. An update on this work will be included in the poster.

BackgroundMotor neurone disease (MND) is a devastating illness which leads to muscle weakness and death, usually within 2-3 years of symptom onset. Respiratory insufficiency is a common cause of morbidity, particularly in later stages of MND and respiratory complications are the leading cause of mortality in MND patients. Non Invasive Ventilation (NIV) is the current standard therapy to manage respiratory insufficiency. Some MND patients however do not tolerate NIV due to a number of issues including mask interface problems and claustrophobia. In those that do tolerate NIV, eventually respiratory muscle weakness will progress to a point at which intermittent/overnight NIV is ineffective. The NeuRx RA/4 Diaphragm Pacing System was originally developed for patients with respiratory insufficiency and diaphragm paralysis secondary to stable high spinal cord injuries. The DiPALS study will assess the effect of diaphragm pacing (DP) when used to treat patients with MND and respiratory insufficiency.Method/Design108 patients will be recruited to the study at 5 sites in the UK. Patients will be randomised to either receive NIV (current standard care) or receive DP in addition to NIV. Study participants will be required to complete outcome measures at 5 follow up time points (2, 3, 6, 9 and 12 months) plus an additional surgery and 1 week post operative visit for those in the DP group. 12 patients (and their carers) from the DP group will also be asked to complete 2 qualitative interviews.DiscussionThe primary objective of this trial will be to evaluate the effect of Diaphragm Pacing (DP) on survival over the study duration in patients with MND with respiratory muscle weakness. The project is funded by the National Institute for Health Research, Health Technology Assessment (HTA) Programme (project number 09/55/33) and the Motor Neurone Disease Association and the Henry Smith Charity. Trial Registration: Current controlled trials ISRCTN53817913. The views and opinions expressed therein are those of the authors and do not necessarily reflect those of the HTA programme, NIHR, NHS or the Department of Health.

Objective To investigate the clinical features and causes of micturition and defecation dysfunction in motor neuron disease (MND) patients. Methods The micturition and defecation function was evaluated by a questionnaire covering storage and voiding of urine and feces respectively in 50 MND patients. The clinical features and external anal sphincter electromyography (EAS-EMG) were analyzed to explore the causes of micturition and defecation dysfunction in MND patients. Results Micturition and defecation dysfunction was detected in 9 of 50 (18.0%) MND patients. The main types of micturition and defecation dysfunction were constipation (4/9), urinary frequency, urgency with or without incontinence, fecal urgency (4/9), powerlessness for micturition and defecation (2/9), hesitancy for micturition (1/9). EAS-EMG was normal in 9 MND patients accompanied with micturition and defecation dysfunction. Conclusions MND patients accompanied with micturition and defecation dysfunction were not very rare. Constipation, urgency and powerlessness were the main types of micturition and defecation dysfunction and they were not related to the function of external anal sphincter. Gastrointestinal dysfunction from abnormal autonomic nerve involvement, muscle weakness and the resulted reduced activity, severe upper motor neuron damage and respiratory muscle weakness may be the main causes of micturition and defecation dysfunction in MND patients. Key words: Motor neuron disease; Amyotrophic lateral sclerosis; Constipation; Urination disorders; Anal canal; Electromyography

We report a rare association of spinocerebellar ataxia and motor neuron disease (MND) in a woman with genetically confirmed SCA2 who subsequently developed a rapidly progressive and fatal form of MND. Considering the rarity of these two neurological conditions, it is interesting to note that the concomitant occurrence of SCA mutations and MND have been previously observed in three cases: in one patient affected by SCA6 and two other cases with SCA2.

Background Although Motor Neurone Disease (MND) caregivers are most challenged physically and psychologically, there is a paucity of population-based research to investigate the impact of bereavement, unmet needs, range of supports, and their helpfulness as perceived by bereaved MND caregivers. Methods: An anonymous national population-based cross-sectional postal and online survey of bereavement experiences of family caregivers who lost a relative/friend to MND in 2016, 2017, and 2018. Recruitment was through all MND Associations in Australia. Results: 393 valid responses were received (31% response rate). Bereaved caregiver deterioration in physical (31%) and mental health (42%) were common. Approximately 40% did not feel their support needs were met. Perceived insufficiency of support was higher for caregivers at high bereavement risk (63%) and was associated with a significant worsening of their mental and physical health. The majority accessed support from family and friends followed by MND Associations, GPs, and funeral providers. Informal supports were reported to be the most helpful. Sources of professional help were the least used and they were perceived to be the least helpful. Conclusions: This study highlights the need for a new and enhanced approach to MND bereavement care involving a caregiver risk and needs assessment as a basis for a tailored “goodness of fit” support plan. This approach requires continuity of care, more resources, formal plans, and enhanced training for professionals, as well as optimizing community capacity. MND Associations are well-positioned to support affected families before and after bereavement but may require additional training and resources to fulfill this role.

BackgroundA legacy was received by the Wirral Branch of the Motor Neurone Disease Association to improve the experience of people with Motor Neurone Disease (pwMND) on Wirral. Challenges faced by...

We describe three unrelated patients with sporadic motor neuron disease (MND) and hereditary amyloid transthyretin (ATTRv) amyloidosis family history, who were asymptomatic carriers of the pVal50Met mutation of transthyretin (TTR) gene. Patients 1 and 2 were a 43-year-old man with a spinal-onset of ALS and a 37-year-old woman with a bulbar-onset of ALS, who died due to respiratory complications five and two years after disease onset, respectively. Patient 3 is a 52-year-old woman, with a two-year history of a probable primary lateral sclerosis, and a frontotemporal dysfunction. Imaging, cerebrospinal fluid (CSF) and nerve conduction and small fiber tests were normal in all. Genetic testing for ALS was negative in the two patients tested. Previous studies in MND patients have identified reduced TTR levels in CSF and neuronal gene overexpression, suggesting a neuroprotective role of TTR. The association of MND in patients with TTR gene mutations has not yet been described.

Background: Motor neurone disease (MND) is an incurable neurodegenerative condition. Recent guidelines from the MND Association UK (MNDA) emphasise prompt diagnosis and monitoring of quality of life (QOL). However, primary literature indicates that diagnosis is often delayed through several factors, including unawareness of how MND presents and delays through multiple secondary referrals. The [hospital] operates an internationally-recognised MND clinic whose service has not been audited against the MNDA UK guidelines for the last 3 years.Aim: To assess how well the MND consultant’s service ensures prompt diagnosis and the provision of end-of-life care or appropriate respiratory or nutritional support.Methods: Using the [hospital’s] database, records from 77 patients consulted consecutively in the MND clinic were obtained. Service parameters were compared against reference standards for diagnostic delay and treatment provision.Results: 84.4% of referrals came from neurologists outside of the MND service, with only 13% from general practitioners. On average, it took 14.7 months for patients to be seen in the MND clinic following symptom onset. Riluzole and percutaneous endoscopic gastrostomy (PEG) were prescribed faster in bulbar-onset versus limb-onset patients, by 4 times and 9 times respectively. End-of-life care discussion was recorded for 26% of patients.Conclusion: Diagnosing MND remains challenging for primary care physicians, which may be alleviated by disseminating recent Red Flag Committee guidelines. Investigation and treatment provision differed between MND subtypes, given the poorer prognosis associated with bulbar-onset. End-of-life care documentation remains low, which is an issue for the multi-disciplinary team (MDT) to resolve through incorporating palliative services.

The state of the immune system, both humoral and cell-mediated, was evaluated in motor neurone disease (MND) patients. The data obtained for the MND patients were confronted with normal controls and a group of patients affected by nervous diseases not involving the immune system. Some differences were observed between MND patients and normal subjects, namely: increase of WBC and gamma-globulin in MND patients. However, such differences were not observed between MND patients and pathological controls, and therefore are probably due to a higher frequency of infectious complications in MND patients in respect to normal controls. The capacity of the immune system to respond to an adequate stimulus was normal, and no precipitating anti-CNS antibodies were detected in MND sera. Furthermore, no sex-linked differences were observed and the CSF abnormalities observed in 2 out of 16 MND patients were probably reflecting only destruction of CNS cells. The data are discussed in view of the possible pathogenetic mechanisms of MND.

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BackgroundCare provision for Motor Neurone Disease (MND) patients require a Multidisciplinary Team (MDT) approach. Healthcare Professionals (HCPs) and MND patients often find it challenging to communicate effectively. Communication is particularly...

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