Respiratory adjuncts to NIV in neuromuscular disease

Abstract

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. Non-invasive ventilation (NIV) is an established and indispensable therapy to manage hypoventilation and respiratory failure. The role of other therapies that support respiratory health is less clearly defined, and the evidence of efficacy is also harder to summarize as the underlying data are of a lesser quality. This narrative review appraises the evidence for respiratory therapies in adults with NMD and respiratory system involvement. Techniques that assist lung inflation and augment cough, such as lung volume recruitment (LVR) and mechanical insufflationexsufflation (MI-E), are a particular focus of this review. The evidence suggests that LVR, MI-E and various combinations thereof have clinical utility generally, but important methodological limitations limit the strength of clinical recommendations and hamper the integration of evidence into practice. Future trials should prospectively assess the long-term impact of LVR and cough augmentation on clinical outcomes and burden of care in addition to lung mechanics, as well as determine clear predictors of benefit from these techniques.

Contemporary Concise Review 2019: Sleep and ventilation

“…Research on such technologies is, however, always challenged by both the wide range of devices and settings available. An assessment for need of respiratory adjuncts to NIV to assist with cough, impaired secretion clearance, speech and aspiration is also required in the comprehensive and individualized management of neuromuscular patients Obesity continues to rise but despite the clear association between obesity and OHS, OHS frequently goes undiagnosed .…”

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

“…Neuromuscular disease (NMD) often involves muscles of the respiratory system, and may result in ventilatory failure and increased risk of respiratory tract infection [1,2]. Mechanical ventilatory support, such as non-invasive ventilation (NIV) is the primary respiratory therapy for patients with NMD and respiratory failure; however, clinical adjuncts focusing on cough augmentation, lung inflation, and chest wall mobility are frequently used both in hospital and the community [1]. Common examples of these adjunctive therapies are lung volume recruitment (LVR) and mechanical insufflation-exsufflation (MI-E).…”

“…MI-E uses a dedicated device for delivering assisted inspiration at a set pressure followed by a rapid cycle to exsufflation at negative pressure. LVR can be performed using a manual resuscitation bag with or without a oneway valve, glossopharyngeal breathing or the inspiratory phase of a (typically) volume cycled NIV device [1,3]. Because both MI-E and LVR increase inspiratory capacity above a maximal spontaneous inspiration, it is important to consider the risk of pneumothorax or other overexpansion injury when prescribing these techniques.…”

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

“…Neuromuscular disease (NMD) often involves muscles of the respiratory system, and may result in ventilatory failure and increased risk of respiratory tract infection . Mechanical ventilatory support, such as non‐invasive ventilation (NIV) is the primary respiratory therapy for patients with NMD and respiratory failure; however, clinical adjuncts focusing on cough augmentation, lung inflation, and chest wall mobility are frequently used both in hospital and the community . Common examples of these adjunctive therapies are lung volume recruitment (LVR) and mechanical insufflation‐exsufflation (MI‐E).…”