Akram Khan, MD, FCCP, Associate Professor of Pulmonary, Allergy and Critical Care Medicine at Oregon Health & Science University, discusses the new clinical guidelines on respiratory management of patients with neuromuscular weakness, which was recently released by the American College of Chest Physicians (CHEST).
As Dr. Khan explains, the guidelines were recently published in the journal, CHEST, and include 15 evidence-based recommendations for respiratory management of neuromuscular weakness, as well as a good practice statement, and an ungraded consensus-based statement. The guidelines have been endorsed by the American Association for Respiratory Care, the American Thoracic Society, the American Academy of Sleep Medicine, and the Canadian Thoracic Society. Evidence of best practices for respiratory management in neuromuscular disorders (NMDs) is based primarily on observational data in amyotrophic lateral sclerosis (ALS) but is applicable to other NMDs such as Pompe disease, myasthenia gravis, spinal muscular atrophy, and muscular dystrophy.
Summary of the guidelines are listed below.
Use and Timing of Pulmonary Function Testing:
- For patients with NMD at risk for respiratory complications, we recommend pulmonary function testing (PFT) to assist with management decisions (Good Practice Statement).
- For patients with NMD at risk for respiratory failure, we suggest pulmonary function testing at a minimum of every six months as appropriate to the course of the specific NMD. (Conditional Recommendation, Ungraded Consensus-based Statement).
Screening for Respiratory Failure and Sleep-Related Breathing Disorders:
- For symptomatic patients with NMD who have normal PFT and overnight oximetry (ONO), we suggest that clinicians consider polysomnography (PSG) to assess whether non-invasive ventilation (NIV) is clinically indicated (conditional recommendation, very low certainty of evidence).
Use of Non-invasive Ventilation:
- For patients with NMD and chronic respiratory failure, we recommend using NIV for treatment (strong recommendation, very low certainty of evidence).
- For patients with NMD and sleep-related breathing disorders, we suggest using NIV for treatment (conditional recommendation, very low certainty of evidence).
Respiratory Parameters for Initiation of NIV:
- For patients with NMD, we suggest the use of diagnostic tests such as forced vital capacity (FVC), MIP/MEP, ONO, or evidence of sleep-disordered breathing or hypoventilation on PSG to predict the timing of NIV initiation (conditional recommendation, very low certainty of evidence).
- For patients with NMD requiring NIV, we suggest individualizing NIV treatment to achieve ventilation goals (conditional recommendation, very low certainty of evidence).
- For patients with NMD and preserved bulbar function using NIV, we suggest mouthpiece ventilation (MPV) for daytime ventilatory support as an adjunct to nocturnal mask NIV (conditional recommendation, very low certainty of evidence).
Use of Mechanical Ventilation:
- For NMD patients failing NIV or intolerant of NIV (including extended daytime NIV use), worsening bulbar function, frequent aspiration, insufficient cough, episodes of chest infection despite adequate secretion management, or declining lung function, we suggest invasive home MV via tracheostomy as an alternative to NIV (conditional recommendation, very low certainty of evidence).
Sialorrhea Management:
- 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 (conditional recommendation, very low certainty of evidence).
- For patients with NMD and sialorrhea who have an inadequate response or are intolerant of the side effects of anticholinergic therapy, we suggest botulinum toxin (BT) therapy to salivary glands (conditional recommendation, very low certainty of evidence).
- For patients with NMD and sialorrhea who have an inadequate response or are intolerant of the side effects of anticholinergic therapy, we suggest salivary gland radiation therapy (RT) (conditional recommendation, very low certainty of evidence).
Airway Clearance Therapies:
- We suggest clinicians consider glossopharyngeal breathing for lung volume recruitment (LVR) and airway clearance for patients with NMD and hypoventilation (conditional recommendation, very low certainty of evidence).
- For patients with NMD and reduced cough effectiveness, we suggest manually assisted cough techniques independently or added to other modalities such as LVR (conditional recommendation, very low certainty of evidence).
- For patients with NMD and reduced lung function or cough effectiveness, we suggest regular use of LVR (breath stacking) using a handheld resuscitation bag or mouthpiece (conditional recommendation, very low certainty of evidence).
- For patients with NMD and reduced cough effectiveness, which cannot be adequately improved with alternative techniques, we suggest the addition of regular Mechanical Insufflation-Exsufflation (cough assist device) (conditional recommendation, very low certainty of evidence).
- For patients with NMD and difficulties with secretion clearance, we suggest using high-frequency chest wall oscillation (HFCWO) for secretion mobilization. In addition, we suggest that HFCWO be combined with airway clearance therapies such as cough assistance or LVR (conditional recommendation, very low certainty of evidence).
To view the guidelines in full, visit here.
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