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Guideline Addresses Respiratory Management of Patients With Neuromuscular Weakness
A clinical guideline for the respiratory management of patients with neuromuscular weakness was recently released by the American College of Chest Physicians. Published in the journal CHEST, the guideline is based on a systematic review of 128 studies and includes 15 graded recommendations, one good practice statement, and one consensus-based statement.
“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,” said guideline lead author Akram Khan, MD, FCCP, of Oregon Health & Science University, Portland, Oregon. “We anticipate this guideline will standardize and improve the care provided to patients with neuromuscular diseases and subsequent weakness.”
The guidance provides advice for mouthpiece ventilation, transition to home mechanical ventilation, salivary secretion management, and airway clearance therapies. Recommendations are classified as strong and referred to as “recommended,” or conditional and referred to as “suggested.”
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In the guideline’s only strong recommendation, the authors advise noninvasive ventilation for treatment for patients with neuromuscular diseases and chronic respiratory failure.
Conditional suggestions in the guideline include individualizing noninvasive ventilation in patients requiring it to achieve ventilation goals, pulmonary function testing at least every 6 months as appropriate to the course of the specific neuromuscular disease for patients at risk of respiratory failure, and a therapeutic trial of an anticholinergic medication as first-line therapy for patients with neuromuscular diseases and sialorrhea.
“The guideline panel emphasizes that neuromuscular disease pathologic characteristics represent a diverse group of disorders with differing rates of decline in lung function,” the authors wrote. “The clinician’s role is to add evaluation at the bedside to shared decision-making with patients and families, including respect for patient preferences and treatment goals, considerations of quality of life, and appropriate use of available resources in decision-making.”
The guideline was 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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