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.

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The Benefit of Non-invasive Ventilation in Motor Neuron Disease

The Open Respiratory Medicine Journal ◽

10.2174/1874306402014010053 ◽

2020 ◽

Vol 14 (1) ◽

pp. 53-61

Author(s):

Laura J. Walsh ◽

Desmond M. Murphy

Keyword(s):

Quality Of Life ◽

Motor Neuron ◽

Motor Neuron Disease ◽

Respiratory Muscle ◽

Survival Advantage ◽

Inspiratory Pressure ◽

Invasive Ventilation ◽

Non Invasive ◽

Non Invasive Ventilation

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.

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P192 Clinical effectiveness of non-invasive ventilation in patients with Motor Neuron Disease

Thorax ◽

10.1136/thoraxjnl-2015-207770.329 ◽

2015 ◽

Vol 70 (Suppl 3) ◽

pp. A173.1-A173

Author(s):

D Freeman ◽

A Jothieswaran ◽

M Mascareno ◽

N Chaudhry ◽

S Bokhari ◽

...

Keyword(s):

Motor Neuron ◽

Motor Neuron Disease ◽

Clinical Effectiveness ◽

Invasive Ventilation ◽

Non Invasive ◽

Non Invasive Ventilation

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

Chronic Respiratory Disease ◽

10.1177/14799731211063886 ◽

2021 ◽

Vol 18 ◽

pp. 147997312110638

Author(s):

Laura J Walsh ◽

Kevin F Deasy ◽

Fernando Gomez ◽

Elizabeth O’Sullivan ◽

Joseph Eustace ◽

...

Keyword(s):

Motor Neuron ◽

Motor Neuron Disease ◽

Neurodegenerative Disorder ◽

Cohort Analysis ◽

Invasive Ventilation ◽

Non Invasive ◽

Progressive Muscle Weakness ◽

Non Invasive Ventilation ◽

Retrospective Cohort Analysis ◽

Bulbar Onset

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.

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Experiences of end of life care of patient's with motor neuron disease on non invasive ventilation

BMJ Supportive & Palliative Care ◽

10.1136/bmjspcare-2012-000196.277 ◽

2012 ◽

Vol 2 (Suppl 1) ◽

pp. A94.2-A94 ◽

Cited By ~ 1

Author(s):

Kimberley Steel ◽

Aruna Hodgson ◽

David Waterman ◽

Sophie Harrison ◽

Andrew Bentley

Keyword(s):

Motor Neuron ◽

Motor Neuron Disease ◽

End Of Life ◽

End Of Life Care ◽

Life Care ◽

Invasive Ventilation ◽

Non Invasive ◽

Non Invasive Ventilation

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Exploring the feasibility of a music-assisted relaxation intervention to assist with the initiation of non-invasive ventilation in people with Motor Neuron Disease

Music and Medicine ◽

10.47513/mmd.v9i2.504 ◽

2017 ◽

Vol 9 (2) ◽

pp. 86

Author(s):

Jeanette Tamplin ◽

Felicity A Baker ◽

Rebecca Davies ◽

Eleanor Bajo ◽

Karen Bolger ◽

...

Keyword(s):

Quality Of Life ◽

Motor Neuron ◽

Motor Neuron Disease ◽

Qualitative Data ◽

Small Sample ◽

Invasive Ventilation ◽

Positive Effects ◽

Non Invasive ◽

Non Invasive Ventilation

Background: Transitioning to non-invasive ventilation (NIV) can cause anxiety in people with Motor Neuron Disease (MND), which may reduce adherence rates. Music therapy has demonstrated positive effects with mechanical (invasive) ventilation.Method: This feasibility study examined the effects of music-assisted relaxation (MAR) on NIV adherence, anxiety, and quality of life for people with MND. Data were collected at baseline, 7-days post, and 3-months post NIV implementation.Results: Of 18 consenting participants, 15 chose the MAR condition. Results suggested that supporting NIV transition within the first 7 days may be advantageous for long-term adherence. No effects were found for anxiety or quality of life. Limitations included small sample size, lack of an adequate control, and possible ceiling effects on the instruments used. Qualitative data indicated most participants considered the relaxing and distracting effects of MAR were useful.Conclusions: In this small sample, we found some demand for and acceptability of a music-based intervention in this setting. Participants reported differing experiences of using MAR, and there were technical and logistical issues regarding timely and accessible provision of a MAR intervention within the treatment trajectory of NIV implementation. Conflicting quantitative and qualitative data support the need for mixed method research in this area.

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