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Highlights • Pre workshop international survey on MPV • Best practice guidline on the provision of MPV in NMD's by reviewing and recommend the most appropriate monitoring, mode, settings and equipment required for MPV. • Best practice algorithm on MPV for daytime ventilatory support (see Figure 1 ).Figure 1Figure 1 shows the best practice algorithm for the initiation of mouthpiece ventilation (MPV) in the long-term setting. For each stage of the algorithm there is a description to assist in the provision of this service.Figure 1 • Future studies in MPV.

Introduction Spirometry plays an important role in the assessment of possible respiratory failure in children with neuromuscular disorders (NMDs). However, obtaining reliable spirometry results is a major challenge. We studied the relation between Forced Oscillation Technique (FOT) and spirometry results. FOT is an easy, non-invasive method to measure respiratory mechanics, i.e. respiratory resistance R and respiratory reactance X. We hypothesized an increased resistance R and reduced reactance X in patients with more reduced lung function. Methods In this prospective single center study we included all children with NMDs able to perform spirometry. We consecutively measured respiratory resistance R and reactance X at 5, 11 and 19 Hz and (Forced) Vital Capacity, Peak Expiratory Flow. Spearman correlation coefficients were calculated and regression curves were estimated. Results We included 148 patients, with a median age of 13 years (IQR 8-16). All correlation coefficients were statistically significant with p = 0.000. A negative correlation was found between resistance R and spirometry outcomes (spearman correlation coefficient (ρ) between -0.5 and -0.6). A positive correlation was found between reactance X (i.e. less negative outcomes) and spirometry outcomes (ρ between 0.4 and 0.6). Highest correlation was found at lower frequencies. Regression analysis showed a non-linear relation between FOT and spirometry results. Conclusion We found a non-linear relation between FOT and spirometry results with increased resistance R and reduced reactance X in patients with more restrictive lung function decline. Given the difficulties with performing spirometry, FOT may be a promising surrogate measure of lung function.

Background: Conflicting results are available of effect of surgical correction of scoliosis on pulmonary function. In children with a severely decreased respiratory function even limited improvement of respiratory function after scoliosis surgery could be of major clinical relevance. Aim: This study aimed to evaluate the short-term effect of scoliosis surgery on pulmonary function in children diagnosed with non-idiopathic scoliosis by comparing pre-operative spirometry results to results three months after surgery. Methods: This prospective, single-centre, observational study included children, diagnosed with non-idiopathic scoliosis, able to perform spirometry. Wilcoxon signed-rank test was used to compare spirometry results. Results: 28 children were included with median age of 13 years (interquartile rang (IQR) 10-15). Half were children with neuromuscular disease. There was a statistically significant higher relative expiratory forced vital capacity prior (median 58%, IQR 43-76%) compared to 3 months after surgery (55%, IQR 32-78%), p=0.001). No statistically significant difference of both absolute and relative values of peak expiratory flow and maximal vital capacity were seen prior and after surgery. A trend towards higher relative peak expiratory flow 3 months after surgery was observed (median 60%, IQR 42-77%) versus 50%, IQR 40-88%, p=0.053). Results were similar in the most severely affected children. Conclusion: Spirometry results 3 months after surgery for non-idiopathic scoliosis showed no statistically significant difference compared to results prior to surgery. Even a decline of relative expiratory forced vital capacity was observed.

Introduction: The ability to cough requires coordinated use of inspiratory, bulbar and expiratory muscles. This is often compromised in children with neuromuscular diseases, thereby affecting airway clearance. This may lead to recurrent chest infections and diminished gas exchange. In 1990 air-stacking was introduced in The Netherlands aiming to increase cough peak flow. Air-stacking is a challenge for many children, because of required technique and cooperation. The cough-assist was introduced in 2007 and has the advantage that it easier to use, even in young children. Few studies describe a beneficial effect of cough-assist in neuromuscular patients, but evidence is limited. Methods: We initiate air-stacking in children with CPF of 270l/min. We introduce cough-assist in young children with neuromuscular diseases who are unable to perform air-stacking manoeuvers or still suffer from frequent chest infections despite air-stacking. In this observational study we retrospectively review patient characteristics of children using the cough-assist at home. Results: Since 2007 cough-assist is used by 23 children in our hospital (table 1) Hospital admissions because of chest infections were less the year after introduction of cough-assist when compared to the year before. Two children returned their cough-assist to the hospital as they did not use it at home. They had sufficient result with air-stacking. Conclusion: Use of cough-assist in selected children with neuromuscular diseases appears to have beneficial effect on hospital admissions due to chest infection