A 53-year-old woman with spinal muscular atrophy and a 7-year history of nocturnal non-invasive ventilation (NIV) use via nasal mask and chinstrap was admitted electively. Outpatient review suggested symptomatic hypercapnia and hypoxaemia. Use of her usual NIV resulted in early morning respiratory acidosis due to excess mouth leak, and continuous face mask NIV was instigated while in hospital. Once stabilised, she elected to return to nasal ventilation. At outpatient review, respiratory acidosis reoccurred despite diurnal use of NIV. Using the patient's routine ventilator and a novel mouthpiece and trigger algorithm, intermittent daytime mouthpiece ventilation (MPV) was introduced alongside overnight NIV. Control of respiratory failure was achieved and, vitally, independent living maintained. Intermittent MPV was practicable and effective where the limits of ventilator tolerance had otherwise been reached. MPV may reduce the need for tracheostomy ventilation and this case serves as a reminder of the increasing options routinely available to NIV clinicians.

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IntroductionNon-invasive ventilation (NIV) in motor neurone disease (MND) is an evidence-based therapy, recommended by NICE. A single centre randomised trial of 41 patients underpins much of current practice,1 it was suggested our patient cohort may differ from those in the original trial work.MethodsRetrospective review of all patients offered NIV from 01.01.2013 to 30.06.2015. Data was taken from the initial neurology referral, and NIV set-up. Demographics were compared with the Newcastle study1 (Table 1). Twelve month survival, and/or death post NIV initiation were assessed.Abstract P195 Table 1NIV trials for MND (January 2013–June 2015)ResultsSixty-three patients were offered trial of NIV; 5 declined admission, and 7 declined NIV. Fifty-one patients were discharged with NIV, of whom 4 rapidly discontinued ventilation. Forty-seven patients were followed as NIV users, 35 for at least a year or to death.Fifty-seven percent were documented as having bulbar symptoms, the severity of which were not formally assessed. Twenty-nine percent received formal carer support at NIV initiation. Of the 35, 24 (68.6%) died within one year of NIV commencement, and median survival for all deaths was 177 days (range 4–630 days). Patients who died were significantly more likely to have bulbar dysfunction (18/24, p = 0.003) with a trend to reduced survival, median 149 vs. 239.5 days non-bulbar (p = 0.09). Twenty patients are alive at data collection, current median survival 292 days (range 7–793 days) and this data will affect results. Those with carers in place had a significantly lower ALSFRS-R (26.3 vs 32.4, p = 0.008) and shorter median survival (135 days). Of those dying or surviving at least a year, 22/35 (63%) were issued with cough-assist support (18/22 mechanical in/exsufflation).ConclusionsOur cohort and outcomes are similar to those in the Bourke trial. Patients with bulbar disease, and/or pre-existing care input may have worse survival. Current users will be followed up to complete the dataset for survival. The impact of bulbar disease, cough augmentation2 and carer need remain uncertain. Ways to better assess and support these groups should be sought, and adequately powered randomised trials in these areas developed.References1 Bourke SC, Tomlinson M, Williams TL. et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006;5:140–72 Rafiq MK, Bradburn M, Proctor AR. et al. A preliminary randomized trial of the mechanical insufflator-exsufflator versus breath-stacking technique in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. Published Online First: 3 Jul 2015. doi:10.3109/21678421.2015.1051992