Ventilation In Motor Neuron Disease Research Articles

Bispectral Index monitoring of palliative sedation for home withdrawal of tracheostomy ventilation: A case report.

Tracheostomy ventilation in motor neurone disease is an uncommon life-sustaining treatment. Best practice is having a plan for ventilation withdrawal, but the literature to guide practice is limited. Case reports have documented standard doses of opioids and benzodiazepines used for sedation in such cases. A 49-year-old man was diagnosed with motor neurone disease in 2016. He commenced tracheostomy ventilation in 2018. In 2022 and 2023, planning was undertaken, at the patient's request, for withdrawal of tracheostomy ventilation at home, when he was no longer able to communicate with technology. Planning included Bispectral Index monitoring prior to cessation of ventilation, ensuring this only occurred when deep sedation was achieved. After ventilation withdrawal in 2023, a retrospective review of medications given and his level of sedation on monitoring was undertaken, with family consent. Ventilation withdrawal was initiated after deep sedation was achieved, 6 h after commencing subcutaneous infusions of morphine, midazolam, clonazepam and phenobarbital. Doses required to achieve acceptable sedation exceeded literature reports. Achieving deep sedation was a longer than expected process. More research using an objective measure of sedation is required, as clinical assessment of sedation in this context is compromised.

P112 Non-invasive ventilation in motor neurone disease, an audit of a tertiary centers practice

Abstract Background MND is a progressive neurodegenerative disorder with significant morbidity and mortality. The prevalence in Australasia is the second highest in the world with 12.9 cases per 100 000. Impairment of respiratory function contributes to and correlates with QOL. Respiratory complications are the most common causes of death. NIV improves or maintains QOL in patients with MND and prolongs median survival by up to 205 days in patients with normal to moderately impaired bulbar function. NICE guidelines recommend considering a trial NIV in those with symptoms, signs or evidence of impaired respiratory muscle strength. Methods A single centre, retrospective database and chart review of all incident and prevalent cases with two clinical interactions at the Princess Alexandra Hospital MND service between 2013-2021 was conducted. The primary aim was to determine the proportion of patients who trialed NIV. Secondary outcomes include: concordance with NICE Guidelines regarding NIV commencement, proportion of patients treated with riluzole and received PEG. Survival will be estimated by constructing Kaplan-Meier curves for those established on NIV compared with those that were not. Progress to date A total of 211 patients were included in analysis. 62.1% males, 37.9 % female, mean age at diagnosis 62.8 years. Limb phenotype was most common (52.5%) followed by bulbar phenotype (35.9%). 36.5% of the cohort trialed NIV. Further analysis to follow. Intended outcome and impact The outcomes will be compared with those published in the literature facilitating benchmarking of the service and identification of facets of patient care that can be improved.

P027 The clinical role and outcomes of non-invasive ventilation in motor neuron disease: An Australian tertiary hospital experience

Abstract Introduction In motor neuron disease (MND), non-invasive ventilation (NIV) in patients who develop respiratory muscle weakness improves both quality of life and survival. This study aimed to evaluate the current practice and outcomes of NIV use in MND patients in an Australian tertiary hospital. Methods The medical records of all MND patients who attended a specialist multidisciplinary clinic requiring NIV treatment between January 2015 and January 2020 were retrospectively analysed. Progress to date: Forty-five patients have been analysed with a mean age at time of NIV commencement of 61±10(SD) years, 67% were male, 33% were current or past smokers and 7% had OSA with previous CPAP use. MND onset was limb in 58%, bulbar in 36% and respiratory muscle in 7%. Riluzole was prescribed in 47% and PEG/RIG insertion performed in 47%. At time of NIV commencement, 82% were symptomatic and 47% hypercapnic. No patient was commenced based on functional testing alone. NIV adherence (usage ≥4hours/night) was observed in 80%. NIV non-adherence was associated with bulbar subtype (p=0.02) and empirical NIV initiation (p<0.01) on univariate analysis. Average survival from NIV commencement was 17±22(SD)months. Average survival on NIV in adherent patients was 19±24(SD)months and non-adherent patients was 2±2(SD)months, although this did not reach statistical significance (p=0.1). Intended outcome & impact Overall clinical practice and outcomes of NIV use in this study is comparable to literature. The factors influencing NIV tolerance and adherence require further study to optimise outcomes in MND patients with respiratory muscle weakness.

Tracheostomy ventilation in motor neurone disease: a snapshot of UK practice

Introduction: Motor neurone disease (MND) is characterized by rapidly progressive motor neurone degeneration which leads to muscle wasting. Mortality and morbidity are due to respiratory muscle failure which may be offset by ventilation. The aim of this observational study was to quantify the number and characteristics of patients living with MND choosing tracheostomy ventilation (TV) in the UK. Methods: Long-term ventilation services in the UK were invited to undertake a retrospective 5-year audit of MND patients under their care between April 2013 and March 2018 who had TV. Patient characteristics, the time spent on ventilation, hospital length of stay, discharge destination, and survival data were collected. Results: Sixty-eight MND patients were initiated on TV over the 5-year period. Eighty-one percent of patients received TV in an emergency setting with more than a third of these undiagnosed at presentation. Patients choosing elective TV were more likely to be male (85%) have a bulbar presentation (54%) and an increased survival of 10 months over the observation period. The mean length of hospital stay post TV was 136 days. Two-thirds of patients were discharged to their own home. Conclusion: Very few patients living with MND in the UK are currently receiving TV. In those who choose TV, there may be a survival advantage to planning an elective procedure. Despite the long inpatient stay and high care costs involved a majority of patients survived and were discharged to their own home.

Use of non-invasive ventilation in motor neuron disease – a retrospective cohort analysis

Motor neuron disease (MND) is a neurodegenerative disorder which leads to progressive muscle weakness including respiratory muscle decline. The introduction of non-invasive ventilation (NIV) has been shown to improve quality of life, survival and slow the rate of pulmonary function decline. A retrospective chart analysis of patients who attended the MND clinic from 2014 to 2019 at a tertiary-referral, academic, teaching hospital was carried out to evaluate if NIV and greater compliance with NIV was associated with improved survival. 111 patients were included. The mean age at diagnosis was 63.8 years and 61.3% were males. 66.7% of our cohort used NIV and of this 66.7%, 44.1% were compliant. There was a significantly longer survival in those who used NIV (p = 0.002) and in those who used NIV optimally (p = 0.02) when both groups were compared to those who did not use NIV. In the bulbar MND group those who were compliant with NIV survived longer than who those who did not use NIV (p = 0.001). We found a significantly longer survival with the use of NIV, the use of NIV optimally and with use of NIV in those with bulbar onset MND compared to those who did not use NIV.

The Benefit of Non-invasive Ventilation in Motor Neuron Disease.

Background:Motor Neuron Disease (MND) is a progressive neurodegenerative disorder leading to respiratory muscle weakness with dyspnoea, morning headaches, orthopnoea, poor concentration, unrefreshing sleep, fatigue and daytime somnolence. Respiratory failure is the primary cause of death in those with MND.Methods:Although guidelines suggest the use of non-invasive ventilation (NIV) in MND, there lacks clear guidance as to when is the optimal time to initiate NIV and which markers of respiratory muscle decline are the best predictors of prognosis. There have been a number of studies that have found a significant survival advantage to the use of NIV in MND. Similarly, in quality-of-life questionnaires, those treated with NIV tend to perform better and maintain a better quality of life for longer. Furthermore, studies also suggest that improved compliance and greater tolerance of NIV confer a survival advantage.Results and Discussion:Forced Vital Capacity (FVC) has traditionally been the main pulmonary function test to determine the respiratory function in those with MND; however, FVC may not be entirely reflective of early respiratory muscle dysfunction. Evidence suggests that sniff nasal inspiratory pressure and maximum mouth inspiratory pressure may be better indicators of early respiratory muscle decline. These measures have been shown to be easier to perform later in the disease, in patients with bulbar onset disease, and may indeed be better prognostic indicators.Conclusion:Despite ongoing research, there remains a paucity of randomised controlled data in this area. This review aims to summarise the evidence to date on these topics.

Monitoring and promoting effectiveness and adherence to non-invasive ventilation in motor neurone disease using EncoreAnywhere telemonitoring: a pilot and feasibility, randomised controlled trial. (P4.4-009)

Monitoring and promoting effectiveness and adherence to non-invasive ventilation in motor neurone disease using EncoreAnywhere telemonitoring: a pilot and feasibility, randomised controlled trial. (P4.4-009)

Assisted ventilation in motor neurone disease during inpatient palliative care: barriers and utilisation

ObjectivesAn increasing number of patients with motor neuron disease (MND) in the UK and Ireland use assisted ventilation, and a small proportion of these use long-term tracheostomy ventilation (TV).1 2...

Factors influencing the need for nocturnal ventilation in motor neurone disease

Factors influencing the need for nocturnal ventilation in motor neurone disease

Experience of long-term use of non-invasive ventilation in motor neuron disease: an interpretative phenomenological analysis

ObjectiveAlthough non-invasive ventilation (NIV) can promote quality of life in motor neuron disease (MND), previous studies have disregarded the impact of progression of illness. This study explored how patients’ perceptions...

Non-invasive ventilation in motor neuron disease: an update of current UK practice

BackgroundIn motor neurone disease (MND), respiratory muscle weakness causes substantial morbidity, and death is usually due to respiratory failure. Non-invasive ventilation (NIV) improves symptoms, quality of life and survival, but...

Non-invasive ventilation in motor neuron disease: current UK practice.

To evaluate the clinical application of non-invasive ventilation (NIV) in motor neuron disease (MND) in the UK. We conducted a postal survey of the current clinical practice of all consultant neurologists in the UK, with a second mailing to non-responders after 6 weeks. The principal outcome measures assessed were: 1. the percentage of patients with MND receiving NIV, 2. access to a NIV service, 3. routine monitoring of respiratory function, and 4. influence of symptoms, respiratory function, rate of disease progression, level of disability and bulbar involvement on referral for NIV. The response rate was 76%, 1719 new patients had been diagnosed in the preceding 12 months and a total of 2280 patients were under review. Of these, 126 were currently receiving NIV (5.5% of patients under review, estimated 2.6-3.5% of all MND patients). Most neurologists (172/265) had referred no patients for NIV in the preceding year, while three neurologists made 30% of all referrals nationally. Referral was based primarily on symptoms, and was influenced by the number of MND patients under review, level of disability, rate of disease progression and availability of a NIV service. Bulbar involvement was considered a relative contra-indication to NIV by 51% of responders. In the UK, few patients with MND are treated with NIV. There is marked variation in clinical practice. This may reflect uncertainty about the role of non-invasive ventilation in MND, and emphasizes the need for a randomized controlled trial to assess the impact of NIV on quality of life and survival.