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

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.