CHEST Guidelines-Respiratory-Management-of-Patients-With-Neuromuscu

Respiratory-Management-of-Patients-With-Neuromuscu

Pdf Summary

The American College of Chest Physicians developed a clinical practice guideline for respiratory management in patients with neuromuscular diseases (NMDs), emphasizing the prevention and treatment of respiratory failure. This guideline, endorsed by several respiratory and sleep medicine societies, offers 15 recommendations, a good practice statement, and a consensus-based statement.<br /><br />Key recommendations include regular pulmonary function testing (PFT) every six months for patients at risk, especially those with conditions like amyotrophic lateral sclerosis (ALS), to guide the initiation of noninvasive ventilation (NIV) when necessary. PFT is deemed a low-cost tool to predict disease progression.<br /><br />For sleep-disordered breathing in symptomatic NMD patients, polysomnography is suggested when overnight oximetry findings are normal, to determine NIV necessity. The guideline supports NIV for treating chronic respiratory failure and sleep-related disorders in NMD, although evidence quality is low.<br /><br />NIV settings should be individualized to meet patient-specific ventilation goals. Mouthpiece ventilation can be an adjunct to NIV for daytime support in patients with intact bulbar function.<br /><br />In cases where NIV is insufficient due to bulbar dysfunction, frequent aspiration, or other complications, invasive mechanical ventilation through tracheostomy may be considered. Early discussion of mechanical ventilation options, goals, and potential impacts on quality of life is advised.<br /><br />For managing sialorrhea common in NMD, a trial of anticholinergic medications is recommended, with botulinum toxin or radiation therapy as alternatives if necessary.<br /><br />Airway clearance therapies are recommended for better management of secretion mobilization, utilizing techniques like lung volume recruitment, mechanical insufflation-exsufflation (MI-E), and high-frequency chest wall oscillation (HFCWO).<br /><br />The guideline underscores a shared decision-making approach with respect to patient preferences and emphasizes individualized care based on available resources. Future research is encouraged to refine practices and improve patient outcomes.

Keywords

respiratory management

neuromuscular diseases

clinical practice guideline

noninvasive ventilation

pulmonary function testing

sleep-disordered breathing

mechanical ventilation

airway clearance therapies

shared decision-making

patient outcomes