Guideline in Focus
Respiratory Management of Patients With Neuromuscular Weakness

Respiratory muscle weakness is a serious concern in patients with neuromuscular diseases (NMD). It can lead to inadequate ventilation, nighttime hypoventilation, and the inability to mobilize secretions, which is frequently the cause of death in this population.

Our panel of experts has provided guidelines for mouthpiece ventilation, transition to home mechanical ventilation, salivary secretion management, and airway clearance therapies.

PUBLISHED March 14, 2023


Key Recommendations

For patients with NMD and chronic respiratory failure, we recommend using noninvasive ventilation (NIV) for treatment.

For patients with NMD requiring NIV, we suggest individualizing NIV treatment to achieve ventilation goals.

For patients with NMD and sialorrhea, we suggest a therapeutic trial of an anticholinergic medication as first-line therapy with continued use only if there are perceived benefits compared to side effects.


Interval for
Pulmonary Function Testing
FVC threshold for
starting NIV with symptoms
FVC threshold for
starting NIV without symptoms
mm Hg
PaCO2 threshold for
starting NIV

Implement this guideline into practice

Our guidelines are aimed at helping you respond to today’s clinical challenges and improve patient health outcomes. Our panel of experts has developed multimedia content to facilitate adoption of this guideline into your day-to-day practice.

Algorithm for Initiation of Noninvasive Ventilation

Algorithm for Initiation of Noninvasive Ventilation

This downloadable flowchart outlines an algorithm for initiation of NIV for adult patients with neuromuscular disease experiencing fatigue, headache, concentration/memory difficulty, and/or respiratory symptoms.


CHEST releases clinical practice guideline on respiratory management of patients with neuromuscular weakness
In a release issued to the media, lead author Akram Khan, MD, speaks to the importance of this guideline in practice. The guideline has been endorsed by the American Association for Respiratory Care, the American Thoracic Society, the American Academy of Sleep Medicine, and the Canadian Thoracic Society.


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