This is a unique state of the art review written by a group of 21 international recognized experts in the field that gathered during a meeting organized by the European Neuromuscular Centre (ENMC) in Naarden, March 2017. It systematically reports the entire evidence base for airway clearance techniques (ACTs) in both adults and children with neuromuscular disorders (NMD). We not only report randomised controlled trials, which in other systematic reviews conclude that there is a lack of evidence base to give an opinion, but also include case series and retrospective reviews of practice. For this review, we have classified ACTs as either proximal (cough augmentation) or peripheral (secretion mobilization). The review presents descriptions; standard definitions; the supporting evidence for and limitations of proximal and peripheral ACTs that are used in patients with NMD; as well as providing recommendations for objective measurements of efficacy, specifically for proximal ACTs. This state of the art review also highlights how ACTs may be adapted or modified for specific contexts (e.g. in people with bulbar insufficiency; children and infants) and recommends when and how each technique should be applied.

Mechanical insufflation-exsufflation (MI-E) is a strategy to treat pulmonary exacerbations in neuromuscular disorders (NMDs). Pediatric guidelines for optimal setting titration of MI-E are lacking and the settings used in studies vary. Our objective was to assess the actual MI-E settings being used in current clinical treatment of children with NMDs and a survey was sent in July 2016 to European expertise centers. Ten centers from seven countries gave information on MI-E settings for 240 children aged 4 months to 17.8 years (mean 10.5). Settings varied greatly between the centers. Auto mode was used in 71%, triggering of insufflation in 21% and manual mode in 8% of the cases. Mean (SD) time for insufflation (Ti) and exsufflation (Te) were 1.9 (0.5) and 1.8 (0.6) s respectively, both ranging from 1 to 4s. Asymmetric time settings were common (65%). Mean (SD) insufflation (Pi) and exsufflation (Pe) pressures were 32.4 (7.8) and -36.9 (7.4), ranging 10 to 50 and -10 to -60cmHO, respectively. Asymmetric pressures were as common as symmetric. Both Ti, Te, Pi and Pe increased with age (p < 0.001). In conclusion, pediatric MI-E settings in clinical use varied greatly and altered with age, highlighting the need of more studies to improve our knowledge of optimal settings in MI-E in children with NMDs.

When ability to cough is impaired, secretion clearance may be assisted and augmented by Mechanical Insufflation-Exsufflation (MI-E). In some individuals, the efficacy of MI-E may be hampered by counterproductive upper airway reactions, where the airways close in response to positive pressures. In order to fully utilize the therapeutic potential inherent in the MI-E technology, we need a better understanding of the pathophysiology behind these untoward reactions. There is an increasing interest in monitoring and measuring upper airway responses to MI-E, and how such information can be used to optimize the MI-E settings. The purpose of this narrative review is to increase the theoretical understanding of larynx as a respiratory organ, summarize the current literature in the area, and provide insight into how this knowledge can affect current clinical practice.

Long-term mechanical ventilation MI-E Mechanical insufflationexsufflation NMD Neuromuscular disorder SMA Spinal muscular atrophy AIM To determine the prevalence of long-term mechanical insufflation-exsufflation (MI-E) and concomitant mechanical ventilation in children with neurological conditions, with reported reasons behind the initiation of treatment. METHOD This was a population-based, cross-sectional study using Norwegian national registries and a questionnaire. RESULTS In total, 114 of 19 264 children with a neurological condition had an MI-E device. Seventy-three of 103 eligible children (31 females, 42 males), median (min-max) age of 10 years 1 month (1y 5mo-17y 10mo), reported their MI-E treatment initiation. Overall, 76% reported airway clearance as the main reason to start long-term MI-E. A prophylactic use was mainly reported by children with neuromuscular disorders (NMDs). Prevalence and age at initiation differed by diagnosis. In spinal muscular atrophy and muscular dystrophies, MI-E use was reported in 34% and 7% of children, of whom 83% and 57% respectively received ventilator support. One-third of the MI-E users were children with central nervous system (CNS) conditions, such as cerebral palsy and degenerative disorders, and ventilator support was provided in 31%. The overall use of concomitant ventilatory support among the longterm MI-E users was 56%. INTERPRETATION The prevalence of MI-E in a neuropaediatric population was 6 per 1000, with two-thirds having NMDs and one-third having conditions of the CNS. The decision to initiate MI-E in children with neurological conditions relies on clinical judgment.

reported general guidelines for management of daytime MPV in NMDs. This report could not present all the detail regarding the technical issues important for clinical success of MPV. Based on the expert workshop discussions and the evidence from existing studies, the current narrative review aims to identify the technical issues of MPV and offers guidance via a decisional algorithm and educational figures providing relevant information that is important for successful implementation of MPV.

Mechanical insufflation‐exsufflation (MI‐E) is recommended for subjects of all ages with neuromuscular disorders (NMDs) and weak cough. There is a lack of knowledge on the optimal treatment settings for young children. This study aims to determine the MI‐E settings providing high expiratory airflow while using safe inspiratory volumes, and to identify possible limits where the benefit of incrementing the MI‐E settings to achieve a higher expiratory airflow, decreased. Using an MI‐E device and a lung model imitating a 1‐year‐old child with NMD, we explored the impact of 120 combinations of MI‐E pressure and time settings on maximal expiratory airflow and inspiratory volume. High expiratory airflows were achieved with several pressure and time combinations where the exsufflation pressure, followed by insufflation pressure and time, had the greatest impact. The benefit of incrementing the settings to increase the expiratory airflow leveled off for the insufflation pressure and time, but not for the exsufflation pressure. Given exsufflation pressure of −40 or −50 cmH2O and insufflation time longer than 1 second, a plateau in the expiratory airflow curve was present at insufflation pressures from 25 cmH2O, whereas a plateau in the inspired volume curve occurred at insufflation pressures from 35 cmH2O. The present neuromuscular pediatric lung model study showed that expiratory pressure impacts expiratory airflow more than inspiratory pressure and time. An inspiratory and expiratory pressure set between 20 to 30 and −40 cmH2O, respectively, and an inspiratory time longer than 1 second may be considered as a basis when titrating MI‐E settings in young children with NMD. The findings must be confirmed in clinical trials.

Aim To (1) compare the perceived benefit of long‐term mechanical insufflation‐exsufflation (MI‐E) of children with neuromuscular disorders (NMDs) and central nervous system (CNS) disorders, including health care needs and treatment routines and (2) describe the children's health‐related quality of life (HRQoL). Method This cross‐sectional study used a questionnaire and memory card data to assess the perceived benefit of MI‐E via the Visual Analogue Scale (VAS; 10 maximum), willingness to pause treatment, level of health care needs before and after MI‐E initiation, and the children's treatment routines. A DISABKIDS questionnaire assessed HRQoL (100 maximum). Results Seventy‐three children using MI‐E participated (42 males, median [interquartile range {IQR}] age 10 years 2 months [6 years 3 months–14 years 1 month]), 47 with NMDs (such as spinal muscular atrophy and Duchenne muscular dystrophy) and 26 with CNS disorders (such as cerebral palsy, encephalitis, neurometabolic and other diseases). The median (IQR) VAS score for the perceived benefit of MI‐E therapy at stable state and respiratory tract infection were 9 (6–10) and 10 (8.5–10) respectively. Sixty‐two per cent were reluctant or unwilling to pause MI‐E therapy, with no NMD versus CNS disorder group difference. After MI‐E initiation, fewer physician consultations and hospitalizations were reported by the group with NMDs. The MI‐E routine was similar in both groups. The mean (SD) HRQoL score for 26 of 51 eligible children was 71 (16.7). Interpretation MI‐E treatment was generally perceived as beneficial and performed equally in both diagnostic groups. HRQoL was in line with children with a moderate‐to‐severe chronic condition. What this paper adds Mechanical insufflation‐exsufflation (MI‐E) was generally perceived as beneficial by the children and parents. The reported benefit of MI‐E was higher among daily than sporadic MI‐E users. The MI‐E treatment routine did not differ between diagnostic groups. The health‐related quality of life in this neuropaediatric population was in line with that of children with other moderate‐to‐severe chronic conditions.

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