A New Clinical Guideline on Respiratory Management of Patients with Neuromuscular Weakness

20 Mar 2023 • The American College of Chest Physicians (CHEST) recently released a new clinical guideline on respiratory management of patients with neuromuscularweakness. Published in the journal CHEST, the guideline contains 15 evidence-based recommendations, a good practice statement and an ungraded consensus-based statement. The guideline recommendations include mouthpiece ventilation, transition to home mechanical ventilation, salivary secretion management and airway clearance therapies. It includes the following highlighted recommendations:

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

Each recommendation is classified as strong, referred to as “recommended,” or conditional, referred to as “suggested.” The panel offers graded recommendations when there is sufficient evidence and ungraded consensus-based statements in areas that were thought to warrant guidance, despite an insufficient grade of evidence.

“Respiratory muscle weakness is a serious concern in patients with neuromuscular diseases. It can lead to inadequate ventilation, nighttime hypoventilation and the inability to mobilize secretions, which is frequently 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 on the guideline. “We anticipate this guideline will standardize and improve the care provided to patients with neuromuscular diseases and subsequent weakness.”

To read the complete guidelines from CHEST Journal, click here to download PDF

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