Can resting lung function predict the response of a person living with motor neuron disease to a hypoxic challenge test?

Objective: People living with MND (PlwMND) are at risk of altitude-related hypoxia during flight. The Hypoxic Challenge Test (HCT) determines whether in-flight oxygen is required but can be expensive and inaccessible. To assist with travel recommendations, we investigated the relationship between altitude simulation-induced hypoxemia and baseline lung function.

NIV@Home: a pilot randomized controlled trial of in-home noninvasive ventilation initiation compared to a single-day admission model.

: Noninvasive ventilation (NIV) is the primary treatment for respiratory insufficiency in neuromuscular disease. NIV implementation is usually conducted within hospitals; however, in-home implementation with intensive follow-up is an effective alternative. This pilot study aimed to assess model feasibility, acceptability, and NIV usage at 12-weeks after a single visit in-home implementation of NIV with remote monitoring follow-up (NIV@Home) compared to an in-hospital day admission NIV initiation plus planned polysomnography (Usual care).

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.

The complexity of multidisciplinary respiratory care in amyotrophic lateral sclerosis.

Unlabelled: Motor neurone disease/amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with no known cure, where death is usually secondary to progressive respiratory failure. Assisting people with ALS through their disease journey is complex and supported by clinics that provide comprehensive multidisciplinary care (MDC). This review aims to apply both a respiratory and a complexity lens to the key roles and areas of practice within the MDC model in ALS.

Prevalence of central sleep apnea in people with tetraplegic spinal cord injury: a retrospective analysis of research and clinical data.

Study Objectives: Over 80% of people with tetraplegia have sleep-disordered breathing (SDB), but whether this is predominantly obstructive or central is unclear. This study aimed to estimate the prevalence of central sleep apnea (CSA) in tetraplegia and the contributions of central, obstructive, and hypopnea respiratory events to SDB summary indices in tetraplegia.

Methods: Research and clinical data from 606 individuals with tetraplegia and full overnight polysomnography were collated.

The role of lung volume recruitment therapy in neuromuscular disease: a narrative review.

Respiratory muscle weakness results in substantial discomfort, disability, and ultimately death in many neuromuscular diseases. Respiratory system impairment manifests as shallow breathing, poor cough and associated difficulty clearing mucus, respiratory tract infections, hypoventilation, sleep-disordered breathing, and chronic ventilatory failure. Ventilatory support (i.

A Randomized Controlled Clinical Trial of Lung Volume Recruitment in Adults with Neuromuscular Disease.

Clinical care guidelines advise that lung volume recruitment (LVR) be performed routinely by people with neuromuscular disease (NMD) to maintain lung and chest wall flexibility and slow lung function decline. However, the evidence base is limited, and no randomized controlled trials of regular LVR in adults have been published. To evaluate the effect of regular LVR on respiratory function and quality of life in adults with NMD.

Rapidly and slowly progressive neuromuscular disease: differences in pulmonary function, respiratory tract infections and response to lung volume recruitment therapy (LVR).

Introduction: Reduced lung volumes are a hallmark of respiratory muscle weakness in neuromuscular disease (NMD). Low respiratory system compliance (C) may contribute to restriction and be amenable to lung volume recruitment (LVR) therapy. This study evaluated respiratory function and the immediate impact of LVR in rapidly progressive compared to slowly progressive NMD.

Respiratory muscle training in neuromuscular disease: a systematic review and meta-analysis.

Background: Neuromuscular disease causes a progressive decline in ventilatory function which respiratory muscle training may address. Previous systematic reviews have focussed on single diseases, whereas this study systematically reviewed the collective evidence for respiratory muscle training in children and adults with any neuromuscular disease.

Methods: Seven databases were searched for randomised controlled trials.

Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial.

Background: Impaired cough results in airway secretion retention, atelectasis and pneumonia in individuals with Duchenne muscular dystrophy (DMD). Lung volume recruitment (LVR) stacks breaths to inflate the lungs to greater volumes than spontaneous effort. LVR is recommended in DMD clinical care guidelines but is not well studied.

Objective measurement of lung volume recruitment therapy: laboratory and clinical validation.

Lung volume recruitment manoeuvres are often prescribed to maintain respiratory health in neuromuscular disease. Unfortunately, no current system accurately records delivered dose. This study determined the performance characteristics of a novel, objective, manual lung volume recruitment bag counter ('the counter') with bench and healthy volunteer testing, as well as in individuals with neuromuscular disease.

Pneumothorax in neuromuscular disease associated with lung volume recruitment and mechanical insufflation-exsufflation.

A 25-year-old male with Duchenne muscular dystrophy and a 73-year-old male with motor neurone disease both presented with chest pain and increasing dyspnoea following routine mechanical insufflation-exsufflation or lung volume recruitment, on a background of long-term non-invasive ventilation. In each case, chest radiograph revealed a pneumothorax. In both cases the pneumothorax fully resolved following insertion of an intercostal catheter.

Randomised controlled trial of polysomnographic titration of noninvasive ventilation.

Noninvasive ventilation (NIV) settings determined during wakefulness may produce patient-ventilator asynchrony (PVA) during sleep, causing sleep disruption and limiting tolerance. This study investigated whether NIV titrated with polysomnography (PSG) is associated with less PVA and sleep disruption than therapy titrated during daytime alone.Treatment-naive individuals referred for NIV were randomised to control (daytime titration followed by sham polysomnographic titration) or PSG (daytime titration followed by polysomnographic titration) groups.

Respiratory adjuncts to NIV in neuromuscular disease.

Muscle weakness is an intrinsic feature of neuromuscular diseases (NMD). When the respiratory muscles are involved, the ability to take a deep breath is compromised, leading to reduced lung volumes and a restrictive ventilatory impairment. Inspiratory, expiratory and bulbar muscle weakness can also impair cough, which may impede secretion clearance.

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.

A randomised controlled trial of CPAP versus non-invasive ventilation for initial treatment of obesity hypoventilation syndrome.

Background: Obesity hypoventilation syndrome (OHS) is the most common indication for home ventilation, although the optimal therapy remains unclear, particularly for severe disease. We compared Bi-level and continuous positive airways pressure (Bi-level positive airway pressure (PAP); CPAP) for treatment of severe OHS.

Methods: We conducted a multicentre, parallel, double-blind trial for initial treatment of OHS, with participants randomised to nocturnal Bi-level PAP or CPAP for 3 months.

Cough Augmentation in Subjects With Duchenne Muscular Dystrophy: Comparison of Air Stacking via a Resuscitator Bag Versus Mechanical Ventilation.

Background: Air stacking improves cough effectiveness in people with Duchenne muscular dystrophy (DMD) and respiratory muscle weakness. However, it is not known whether air stacking is more effective via a resuscitator bag or a home ventilator.

Methods: This prospective randomized study investigated the effect of air stacking via a volume-cycled home ventilator versus via a resuscitator bag in participants with DMD.

Improved survival with an ambulatory model of non-invasive ventilation implementation in motor neuron disease.

Non-invasive ventilation (NIV) increases survival and quality of life in motor neuron disease (MND). NIV implementation historically occurred during a multi-day inpatient admission at this institution; however, increased demand led to prolonged waiting times. The aim of this study was to evaluate the introduction of an ambulatory model of NIV implementation.