Airway Clearance in Neuromuscular Disease.

High-quality respiratory care and airway clearance is essential for people with neuromuscular disease (pwNMD) as respiratory tract infections are a major cause of morbidity and mortality. This review expands on published guidelines by highlighting the role of cough peak flow along with other options for cough evaluation, and discusses recent key research findings which have influenced the practice of respiratory therapy for pwNMD.

Waves of Precision: A Practical Guide for Reviewing New Tools to Evaluate Mechanical In-Exsufflation Efficacy in Neuromuscular Disorders.

Mechanical insufflation-exsufflation (MI-E) is essential for secretion clearance, especially in neuromuscular disorders. For the best outcomes, initiation of MI-E should be started at the correct time with regular evaluation to the response to treatment. Typically, cough peak flow has been used to evaluate cough effectiveness with and without MI-E.

International Survey of the Tools Used for Assessment, Monitoring and Management of Home Mechanical Ventilation Patients.

Background: There are limited data reporting diagnostic practices, compared to clinical guidelines, for patients with chronic respiratory failure requiring home mechanical ventilation (HMV). There are no data detailing the current use of downloaded physiological monitoring data in day-to-day clinical practice during initiation and follow up of patients on HMV. This survey reports clinicians' practices, with a specific focus on the clinical approaches employed to assess, monitor and manage HMV patients.

Mechanical Insufflation-Exsufflation: Considerations for Improving Clinical Practice.

The provision of mechanical insufflation-exsufflation (MI-E) devices to enhance cough efficacy is increasing. Typically, MI-E devices are used to augment cough in patients with neuromuscular disorders but also in patients who are weak in an acute care setting. Despite a growing evidence base for the use of these devices, there are barriers to the provision of MI-E, including clinician lack of knowledge and confidence.

Psychosocial impact of the COVID-19 pandemic and shielding in adults and children with early-onset neuromuscular and neurological disorders and their families: a mixed-methods study.

Aim: To describe and evaluate the psychosocial impact of the COVID-19 pandemic and measures to reduce the risk of transmission on patients with early-onset neuromuscular and neurological disorders (NMDs) and their families.

Design: A mixed-methods study in which data were collected between 17 September 2020 and 31 December 2020 using a semi-structured telephone questionnaire developed specifically to meet research aims, and were analysed using quantitative methods and qualitative inductive thematic analysis.

Participants: Forty questionnaires were completed by patients with NMDs (eg, muscular dystrophies, spinal muscular atrophy) or their parent.

Patient-Ventilator Synchronization During Non-invasive Ventilation: A Pilot Study of an Automated Analysis System.

Patient-ventilator synchronization during non-invasive ventilation (NIV) can be assessed by visual inspection of flow and pressure waveforms but it remains time consuming and there is a large inter-rater variability, even among expert physicians. SyncSmart™ software developed by Breas Medical (Mölnycke, Sweden) provides an automatic detection and scoring of patient-ventilator asynchrony to help physicians in their daily clinical practice. This study was designed to assess performance of the automatic scoring by the SyncSmart software using expert clinicians as a reference in patient with chronic respiratory failure receiving NIV.

ERS clinical practice guidelines: high-flow nasal cannula in acute respiratory failure.

Background: High-flow nasal cannula (HFNC) has become a frequently used noninvasive form of respiratory support in acute settings; however, evidence supporting its use has only recently emerged. These guidelines provide evidence-based recommendations for the use of HFNC alongside other noninvasive forms of respiratory support in adults with acute respiratory failure (ARF).

Materials And Methodology: The European Respiratory Society task force panel included expert clinicians and methodologists in pulmonology and intensive care medicine.

Mouthpiece ventilation in neuromuscular disorders: Narrative review of technical issues important for clinical success.

In neuromuscular disorders (NMDs), nocturnal non-invasive ventilation (NIV) via a nasal mask is offered when hypercapnic respiratory failure occurs. With disease progression, nocturnal NIV needs to be extended into the daytime. Mouthpiece ventilation (MPV) is an option for daytime NIV.

Preference of neuromuscular patients regarding equipment for daytime mouthpiece ventilation: A randomized crossover study.

Background: Patients with neuromuscular disorders (NMDs) are likely to develop respiratory failure which requires noninvasive ventilation (NIV). Ventilation via a mouthpiece (MPV) is an option to offer daytime NIV.

Objectives: To determine the preferred equipment for MPV by patients with NMDs.

Long-Term Mechanical Insufflation-Exsufflation Cough Assistance in Neuromuscular Disease: Patterns of Use and Lessons for Application.

Background: Mechanical insufflation-exsufflation (MI-E) devices increase expiratory air flow and thereby promote increased cough peak flow (CPF) in conjunction with a cough. There is little research looking at long-term use of MI-E in subjects with neuromuscular disease (NMD), and no long-term study has reported CPF, MI-E device settings, and adherence.

Methods: We evaluated 181 patient records (130 adults, 51 children) of individuals who received a MI-E device from our center between February 2014 and February 2018.

European Respiratory Society guidelines on long-term home non-invasive ventilation for management of COPD.

Background: While the role of acute non-invasive ventilation (NIV) has been shown to improve outcome in acute life-threatening hypercapnic respiratory failure in COPD, the evidence of clinical efficacy of long-term home NIV (LTH-NIV) for management of COPD is less. This document provides evidence-based recommendations for the clinical application of LTH-NIV in chronic hypercapnic COPD patients.

Materials And Methods: The European Respiratory Society task force committee was composed of clinicians, methodologists and experts in the field of LTH-NIV.

Introduction of the harmonised respiratory physiotherapy curriculum.

Building on the core syllabus for postgraduate training in respiratory physiotherapy, published in 2014, the European Respiratory Society (ERS) respiratory physiotherapy task force has developed a harmonised and structured postgraduate curriculum for respiratory physiotherapy training. The curriculum outlines the knowledge, skills and attitudes which must be mastered by a respiratory physiotherapist working with adult or paediatric patients, together with guidance for minimal clinical exposures, and forms of learning and assessment. This article presents the rationale, methodology and content of the ERS respiratory physiotherapy curriculum.

Sleep disordered breathing and ventilatory support in children with Down syndrome.

Study Objectives: Obstructive sleep apnoea (OSAS) in children with Down syndrome (DS) is now well recognized, but other forms of sleep disordered breathing (SDB) in this population are less well described. Anecdotally, respiratory support for SDB treatment in this population is not easily tolerated. We aimed to characterize the types of SDB in children with DS referred to a tertiary respiratory center and to assess the effectiveness and adherence to respiratory support.

Airway clearance techniques in neuromuscular disorders: A state of the art review.

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.

Mouthpiece ventilation and complementary techniques in patients with neuromuscular disease: A brief clinical review and update.

Noninvasive ventilatory support (NVS) is sometimes reported as suboptimal in patients with neuromuscular disease (NMD). The reasons for this include inadequate ventilator settings and/or lack of interface tolerance. NVS has been used for many years in patients with NMD disorders as a viable alternative to continuous ventilatory support via a tracheostomy tube.