Respiratory Management of Patients with Neuromuscular Weakness

The American College of Chest Physicians® (CHEST) recently published a new clinical guideline on the respiratory management of patients with neuromuscular weakness. Published in the journal CHEST ®, the guideline contains 15 evidence-based recommendations, a good practice statement, and an ungraded consensus-based statement.

Endorsed by the American Respiratory Care Association, the American Thoracic Society, the American Academy of Sleep Medicine, and the Canadian Thoracic Society, the guideline recommendations include mouthpiece ventilation, transition to home mechanical ventilation, management of salivary secretion, and therapies to clear the airways.

“Respiratory muscle weakness is a serious concern in patients with neuromuscular diseases. It can lead to inadequate ventilation, nocturnal hypoventilation, and inability to mobilize secretions, which is often the cause of death in this population,” says Akram Khan, MD, FCCP, Associate Professor, Pulmonary, Allergy and Critical Care Medicine, Oregon Health & Science University, and the lead author of the guide. “We anticipate that this guideline will standardize and improve the care provided to patients with neuromuscular diseases and subsequent weakness.”

The guide includes the following highlighted recommendations:

For patients with neuromuscular diseases (NMD) and chronic respiratory failure, we recommend using non-invasive ventilation (NIV) for treatment. (Strong recommendation).
For patients with neuromuscular diseases (NMD) requiring NIV, we suggest individualizing NIV treatment to achieve ventilation goals. (Conditional recommendation).
For patients with neuromuscular diseases (NDM) at risk for respiratory failure, we suggest pulmonary function testing at least every 6 months, as appropriate to the course of the specific NMD. (Conditional recommendation).
For patients with neuromuscular diseases (NMD) and sialorrhea, we suggest a therapeutic trial of an anticholinergic medication as first-line therapy with continued use only if benefits are perceived compared to side effects. (Conditional recommendation).

Each recommendation is classified as strong, called "recommended," or conditional, called "suggested." The panel provides graded recommendations where there is sufficient evidence and ungraded consensus-based statements in areas thought to warrant guidance, despite an insufficient degree of evidence.

The full list of population, intervention, comparison, and outcomes recommendations and questions included in the guideline can be accessed through the CHEST journal website .

Download the complete Guide in PDF format in English by clicking here