Overview and audit against Motor Neurone Disease (MND) Association guidelines of a MND consultant’s practice over the last 3 years at the [Hospital

Reversing the hub and spoke model: patient experiences of the development of a community based multi disciplinary motor neurone disease (MND) clinic to meet the motor neurone disease association's standards of care

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

Percutaneous endoscopic gastrostomy (PEG) is reported as the favoured approach in patients without chronic respiratory failure with radiologically inserted gastrostomy insertion reserved for patients with severe respiratory compromise.1 The aim of this study was to evaluate the outcome for patients with chronic respiratory failure receiving PEG insertion as part of a multidisciplinary team approach in a respiratory intensive care unit setting. A retrospective observational study was conducted with all patients admitted for PEG insertion between September 2014 and April 2018. PEG were inserted with procedural intravenous anaesthesia and analgesia (Propofol infusion and Fentanyl boluses, and local anaesthesia to skin with Lidocaine 1%) using invasive, via a tracheostomy, or non-invasive mechanical ventilation on pressure control mode. An established gastrostomy insertion team, including a) gastroenterologist consultant, senior assistant and two senior nurses/practitioners; b) respiratory consultant and senior nurse; and c) anaesthetic consultant and operating departmental practitioner, performed all the procedures. 129/132 (98%) of the PEG procedures were performed successfully. There were 79 males with age 52±19 years. Six (5%) were quaternary referrals following failed PEG insertion and 25 (19%) were for PEG replacements (table 1). Length of stay for motor neurone disease (MND) (3 days; 2, 4 days) was lower compared to the other diagnoses (6 days; 3, 73 days; p PEG insertion in complex neuromuscular patients in a respiratory intensive care setting is safe and effective. However, MND survival post PEG insertion is lower than other diagnostic groups. PEG insertion in MND patients should be carefully considered. Reference Stavroulakis T, Walsh T, Shaw PJ, McDermott CJ. Gastrostomy use in motor neurone disease (MND): A review, meta-analysis and survey of current practice. Amyotrophic Lateral Scler Frontotemporal Degener2013;14:96–104.

Purpose: To explore the views of people with motor neurone disease (MND) on the barriers, facilitators and potential benefits of using home-based e-Health service delivery (telehealth) to access MND multidisciplinary clinic care.Methods: Twelve patients from three MND multidisciplinary clinics and an MND support association group completed a survey of information technology (IT) use and participated in interviews, to gather participants’ experiences and perceptions of home-based telehealth for MND clinic care. Survey data were analyzed descriptively, with interview data analyzed using a stepwise inductive approach.Results: Surveys revealed that participants used IT to communicate with family and friends, but were less likely to use the phone, email or videoconferencing with health professionals. Two themes of participants’ use of IT in MND care reflected their experiences of MND care; and personal preferences for modes of healthcare delivery. Participants were willing to use telehealth for MND care, with family members acting as patients’ main support for telehealth participation. Nevertheless, participants preferred face-to-face contact with the MND clinic team in the initial and early stages of the disease.Conclusions: People living with MND may wish to participate in individual care planning to facilitate their access to a variety of e-Health service modalities. Additionally, individual care planning may allow healthcare professionals to deliver e-Health-based care, such as telehealth, to increase the scope of care provided. Research to ascertain the views of health professionals and family members as co-participants in service delivery via telehealth is needed to fully assess the potential contribution of e-Health.Implications for RehabilitationPeople living with MND face a range of barriers to attending specialized multidisciplinary care, including fatigue, caregiver availability and logistical challenges to travel.Patients have indicated willingness to use e-Health applications to improve their access to care.Use of telehealth could expand service delivery to people with MND living long distances from multidisciplinary clinics, and increase the patient-centred focus of care by tailoring care planning.By offering telehealth services routinely, MND multidisciplinary clinics could also improve the quality and timelines of services offered.

Motor Neurone Disease (MND) is an incurable, progressive condition with an average prognosis of 2–3 years from onset of symptoms (Worms, 2001; Oliver 2019). NICE guidance (2016) recommends that a health care professional with expertise in palliative care should be an integral part of the MDT in managing often rapidly progressive symptoms, psychological distress and complex future care planning. Nutrition and weight loss are predictors of survival (Leigh et al, 2003) and often relate to progressive dysphagia (Heffernan et al, 2004). Gastrostomy can be used to administer nutrition with studies suggesting this can both prolong survival and improve quality of life (Mazzini et al, 1995; Ganzini, 2006). It is important that the option to have gastrostomy is discussed at an early stage taking the patient’s personal preferences into account (MND association, 2019). Patients with MND often have other interventions to try and optimise QOL such as Non-Invasive Ventilation (NIV). Recent literature describes the withdrawal of NIV at the request of patients with MND (LeBon & Fisher, 2011; Messer et al, 2019) and the APM (2016) has issued guidance which considers the legal and ethical implications of this. However, a systematic literature search has found no such evidence or guidance on the withdrawal of gastrostomy feeding in patients with MND. We present the challenges faced when a patient with MND was transferred to hospice IPU requesting withdrawal of PEG feeding and the need for further research; as hospices may increasingly play a role in withdrawal of treatment in MND in future.

To examine factors influencing percutaneous endoscopic gastrostomy (PEG) uptake and outcomes in motor neuron disease (MND) in a tertiary care centre. Case notes from all patients with a confirmed diagnosis of MND who had attended the clinic at the Repatriation General Hospital between January 2007 and January 2011 and who had since died, were audited. Data were extracted for demographics (age and gender), disease characteristics (date of onset, bulbar or peripheral predominance, complications), date and nature of discussion of gastrostomy insertion, nutritional status [weight measurements, body mass index (BMI)], date of gastrostomy insertion and subsequent progress (duration of survival) and quality of life (QoL) [Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R)]. In addition, the type of clinician initiating the discussion regarding gastrostomy was recorded as Nutritional Support Team (involved in providing nutrition input viz Gastroenterologist, Speech Pathologist, Dietitian) and other (involved in non-nutritional aspects of patient care). Factors affecting placement and outcomes including length of survival, change in weight and QoL were determined. Case records were available for all 86 patients (49 men, mean age at diagnosis 66.4 years). Thirty-eight patients had bulbar symptoms and 48 had peripheral disease as their presenting feature. Sixty-six patients reported dysphagia. Thirty-one patients had undergone gastrostomy insertion. The major indications for PEG placement were dysphagia and weight loss. Nine patients required immediate full feeding, whereas 17 patients initially used the gastrostomy to supplement oral intake, 4 for medication administration and 1 for hydration. Initially the PEG regime met 73% ± 31% of the estimated total energy requirements, increasing to 87% ± 32% prior to death. There was stabilization of weight in patients undergoing gastrostomy [BMI at 3 mo (22.6 ± 2.2 kg/m(2)) and 6 mo (22.5 ± 2.0 kg/m(2)) after PEG placement compared to weight at the time of the procedure (22.5 ± 3.0 kg/m(2))]. However, weight loss recurred in the terminal stages of the illness. There was a strong trend for longer survival from diagnosis among MND in PEG recipients with limb onset presentation compared to similar patients who did not undergo the procedure (P = 0.063). Initial discussions regarding PEG insertion occurred earlier after diagnosis when seen by nutrition support team (NST) clinicians compared to other clinicians. (5.4 ± 7.0 mo vs 11.9 ± 13.4 mo, P = 0.028). There was a significant increase in PEG uptake (56% vs 24%, P = 0.011) if PEG discussions were initiated by the NST staff compared to other clinicians. There was no change in the ALSFRS-R score in patients who underwent PEG (pre 34.1 ± 8.6 vs post 34.8 ± 7.4), although in non-PEG recipients there was a non-significant fall in this score (33.7 ± 7.9 vs 31.6 ± 8.8). Four patients died within one month of the procedure, 4 developed bacterial site infection requiring antibiotics and 1 required endoscopic therapy for gastric bleeding. Less serious complications attributed to the procedure included persistent gastrostomy site discomfort, poor appetite, altered bowel function and bloating. Initial discussion with NST clinicians increases PEG uptake in MND. Gastrostomy stabilizes patient weight but weight loss recurs with advancing disease.

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.

IntroductionMotor neurone disease (MND) is a fatal, progressive, neurodegenerative disease with a median survival from onset of symptoms of 4.32 years. No controlled trials indicate a benefit, although current guidelines...

Background The Sunshine Coast Hospital and Health Service (SCHHS) Motor Neurone Disease (MND) Multidisciplinary Clinic has been running since early 2020 and is the only one of its kind north of Brisbane. Patients with MND have complex care needs and are thought to benefit from the collaborative nature of such clinics. We seek to directly assess the patient experience of our MND Clinic through the use of a patient questionnaire, which addresses both access to appropriate health professionals at Clinic appointments as well as whether specific MND care needs are being addressed through the multidisciplinary team, with reference to the National Institute for Health and Care Excellence (NICE) MND Assessment and Management Guidelines. Methods This is a prospective quality assurance project designed to collect anonymous responses from patients currently attending the SCHHS MND Multidisciplinary Clinic. Questionnaires will be distributed to patients both via e-mail and when attending their clinic appointment, and the results will be collated into a password protected database for analysis. Progress to date Ethics approval is in progress and questionnaires are expected to be distributed in the next few months. Intended outcome and impact This quality assurance project will be used as part of an audit of the SCHHS MND Multidisciplinary Clinic, in order to assess patient satisfaction and identify priority areas for expansion.

BackgroundTwo hospices provide separate support across the same county, for individuals and their loved ones affected by Motor Neurone Disease (MND). To achieve equal access to regular collaborative holistic assessments...

IntroductionMotor neuron disease (MND) is a progressive neurodegenerative disease. For patients with dysphagia or weight loss, the decision to recommend enteral tube feeding is complex, and it remains controversial that...

British Journal of Neuroscience NursingVol. 12, No. 1 BriefingCognition and motor neurone disease: the factsJackie HillJackie HillSearch for more papers by this authorJackie HillPublished Online:26 Feb 2016https://doi.org/10.12968/bjnn.2016.12.1.38AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References Abrahams S, Leigh PN, Goldstein LH (2005). Cognitive change in ALS: a prospective study. Neurology 64(7): 1222–26 Crossref, Google ScholarAbrahams S, Newton J, Niven E et al. (2014) Screening for cognition and behaviour changes in ALS. Amyotroph Lateral Scler Frontotemporal Degener 15(1–2): 9–14 Crossref, Google ScholarBäumer D, Talbot K, Turner MR (2014) Advances in motor neurone disease. J R Soc Med 107(1): 14–21 Crossref, Google ScholarChiò A, Ilardi A, Cammarosano S et al. (2012) Neurobehavioral dysfunction in ALS has a negative effect on outcome and use of PEG and NIV. Neurology 78(14): 108–9 Crossref, Google ScholarElamin M, Phukan J, Bede P et al. (2011) Executive dysfunction is a negative prognostic indicator in patients with ALS without dementia. Neurology 76(14): 1263–9 Crossref, Google ScholarGibbons ZC, Richardson A, Neary D et al. (2008) Behaviour in amyotrophic lateral sclerosis. Amyotrop Lateral Scler 9(2): 67–74 Crossref, Google ScholarGirardi A, MacPherson SE, Abrahams S (2011) Deficits in emotional and social cognition in amyotrophic lateral sclerosis. Neuropsychology 25(1): 53–65 Crossref, Google ScholarGoldstein LH, Abrahams S (2013) Changes in cognition and behavioural in amyotrophic lateral sclerosis: nature of impairment and implications of assessment. Lancet Neurol 12(4): 368–80 Crossref, Google ScholarHogden A, Greenfield D, Nugus P et al. (2013) What are the roles of carers in decision-making for amyotrophic lateral sclerosis multidisciplinary care? Patient Prefer Adherence 7: 171–81 Crossref, Google ScholarLillo P, Hodges JR (2009) Frontotemporal dementia and motor neurone disease: overlapping clinic-pathological disorders. J Clin Neurosci 16(9): 113–5 Crossref, Google ScholarLillo P, Mioshi E, Hodges JR (2012) Caregiver burden in amyotrophic lateral sclerosis is more dependent on patients' behavioural changes than physical disability: a comparative study. BMC Neurol 12: 156 Crossref, Google ScholarLomen-Hoerth MD, Murphy J, Langmore S et al. (2003) Are amyotrophic lateral sclerosis patients cognitively normal? Neurology 60(7): 1094–7 Crossref, Google ScholarMerrilees J, Klapper J, Murphy J et al. (2010) Cognitive and behavioural challenges in caring for patients with frontotemporal dementia and amyotrophic lateral sclerosis. Amyotroph Lateral Scler 11(3): 298–302 Crossref, Google ScholarMioshi E, Bristow M, Cook R et al. (2009) Factors underlying caregiver stress in frontotemporal dementia and Alzheimer's disease. Dement Geriatr Cogn Disord 27(1): 76–81 Crossref, Google ScholarMioshi E, Caga J, Lillo P et al. (2014) Neuropsychiatric changes precede classic motor symptoms in ALS and do not affect survival. Neurology 82(2): 149–155 Crossref, Google ScholarMotor Neurone Disease (MND) Association (2015) For professionals. http://tinyurl.com/zq3ak8x (accessed 29 January 2016) Google ScholarMurphy JM, Henry RG, Langmore S (2007) Continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol 64(4): 530–4 Crossref, Google ScholarNational Clinical Guideline Centre (2015) Motor neurone disease: assessment and management. http://tinyurl.com/j9uqqdq (accessed 29 January 2016) Google ScholarNewsom-Davis IC, Lyall RA, Leigh PN, Moxham J, Goldstein LH (2001). The effect of non-invasive positive pressure ventilation (NIPPV) on cognitive function in amyotrophic lateral sclerosis (ALS): a prospective study. J Neurol Neurosurg Psychiatry 71(4): 482–7 Crossref, Google ScholarPijnenburg Y (2011) New diagnostic criteria for the behavioral variant of frontotemporal dementia. Eur Neurol Rev 6(4): 234–7 Crossref, Google ScholarStrong MJ, Grace GM, Freedman M et al. (2009) Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scer 10(3): 131–46 Crossref, Google ScholarZago S, Poletti B, Morelli C et al. (2011) Amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD). Arch Ital Biol 149(1): 39–56 Google Scholar FiguresReferencesRelatedDetailsCited byCommunication and cognitive impairments and health care decision making in MND: A narrative review8 July 2019 | Journal of Evaluation in Clinical Practice, Vol. 25, No. 6The challenges of dysphagia in treating motor neurone diseaseSiobhan Vesey7 July 2017 | British Journal of Community Nursing, Vol. 22, No. Sup7 2 February 2016Volume 12Issue 1ISSN (print): 1747-0307ISSN (online): 2052-2800 Metrics History Published online 26 February 2016 Published in print 2 February 2016 Information© MA Healthcare LimitedPDF download

The optimal technique for long-term enteral feeding has not yet been established. Both percutaneous endoscopic gastrostomy (PEG) and percutaneous radiologic gastrostomy (PRG) are widely used. Aim was to extensively review outcomes of PEG and PRG. A systematic review using Medline, Embase, and Cochrane was performed, using standardized tools for assessing bias. Main outcomes were infectious and tube-related complications, procedure related and 30-day mortality. Pooled risk differences (RDs) with corresponding 95% confidence intervals (95% CIs) were calculated using random effects. Arcsine transformations were applied. In total, 344 studies were identified, of which 16 were included, reporting on 934 PEGs and 1093 PRGs. No differences were found for infectious complications [RD, 0.03 (-0.05 to 0.11)], procedure-related mortality [RD, 0.01 (-0.04 to 0.06)], or 30-day mortality [RD, 0.06 (-0.01 to 0.13)]. Tube-related complications were higher in PRG [RD, 0.16 (0.06-0.26)]. Subgroup analysis was performed for head and neck cancer (HNC) and motor neuron disease. In HNC, this revealed significantly lower tube-related complications and procedure-related mortality after PEG. In motor neuron disease, no differences were seen. The level of evidence appears sufficient considering the low degree of heterogeneity. No differences were found with regard to mortality or infectious complications. PEG showed lower risk of tube-related complications. Subgroup analysis revealed PEG to be favorable in HNC based on lower rates of procedure-related mortality and tube-related complications. Local experience and availability should be taken into account in the decision process.

Motor neuron disease (MND) is a heterogeneous group of neurodegenerative disorders defined by a progressive upper motor neuron (UMN) and lower motor neuron (LMN) loss in a varying combination, encompassing...

Introduction: Motor neurone disease is a life limiting progressive neurological condition that requires a team-based approach to care. Person-centred care (PCC), integrated care and access to specialised motor neurone disease (MND) multidisciplinary clinics (MDC) are optimal approaches to the delivery of quality service for people living with MND (plwMND) for improved quality of life, health outcomes and care experiences. These MND MDCs models of care vary in design, implementation, and are predominantly specialist hospital based. Therefore, not all MND services are delivered consistently highlighting a need to explore the evidence on current practices that contribute to more effective, efficient person-centred integrated care practices across the continuum. Aim: This rapid review explores best practices of integrated care for people living with motor neurone disease, and their families and carers. Methods: A search was undertaken of six databases, Medline (Ovid), Embase (Ovid), CINAHL, Cochrane, Joanna Briggs Institute and Google Scholar. The 1469 retrieved abstracts were screened for eligibility against the inclusion criteria, and full text review was conducted on 149 studies. Data were extracted from 58 studies and a quality assessment conducted on final 45 included studies. The following information was gathered from the included studies to inform the review. 1.What is ‘best practice’ in the management/care of plwMND 2.What models of (integrated) care have been developed to support plwMND 3.What are the common elements of integrated care approaches that have been implemented in MND? 4.What impacts, outcomes, or effects of integrated care approaches have been reported for plwMND? Results: The initial review analysis indicated specialised MND multidisciplinary clinics (MDC) as ‘best practice’ in the optimal management of MND, but revealed no standardised approaches in design and implementation, the multidisciplinary workforce, and degree of integration and partnerships between hospitals and the community. MND MDC are predominantly hospital based, but the inclusion of either outreach community teams, hub and spoke approaches, and/or a hybrid design, (care delivered in person and /or by telehealth), improved integration, knowledge sharing and coordination across services. The timing of introduction and delivery of palliative care services differs but there is greater focus on integration into MND MDC, or earlier referrals to dedicated palliative care service and/or specific palliative care training of neurologist/multidisciplinary team, to optimise symptom management, decision-making and end of life care. Additional features that enable integration, improve care satisfaction and equity are one or a mix of 1) telehealth, 2) care coordination, 3) case management, 4) partnerships with MND associations, and/or 5) MND helpline. Conclusion: This review highlights a range of practices of integrated care that are not universal and predominantly practiced in high income countries. Adapting delivery of model of care to stage and duration of MND, and differing expectations, needs and priorities of plwMND and caregivers, is a common theme throughout. There has been greater uptake in telehealth and technology to support the equitable delivery of integrated approaches to care, with rapid development in this area during COVID 19 pandemic.