Respiratory Management of Patients with Neuromuscular Weakness
Summary of Recommendations
Use and Timing of Pulmonary Function Testing
For patients with neuromuscular disease (NMD) at risk for respiratory complications, we recommend pulmonary function testing (PFT) to assist with management decisions. (GPS, )
Remarks: PFT is a low-cost intervention. The panel recommends that spirometry with forced 79 or slow vital capacity (FVC or SVC) and maximum inspiratory and expiratory pressure (MIP/MEP) or SNIP and PCF be considered in patients with NMD when available according to regional practice patterns.
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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. (C, U)
Remarks When performing spirometry, the panel suggests one or more of the following: vital capacity FVC or SVC, MIP/MEP, SNIP, and PCF at least every six months, according to regional practice patterns and availability. Clinicians should adjust the testing frequency based on the progression rate of individual NMD.
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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. (C, VL)
Remarks: A PSG can be used to assess whether NIV is indicated in symptomatic patients with normal PFT and ONO. This may require an appropriate testing facility in the community, preferably with ADA access, NMD protocols, equipment for NMD, and space for bedside caregivers. Clinical indications may vary based on the patient's age and disease progression. PSG may be the preferred option for pediatric patients.
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Use of Non-invasive Ventilation
For patients with NMD and chronic respiratory failure, we recommend using NIV for treatment. (S, VL)
Remarks: The clinical indications for NIV can vary depending on NMD, patient age, and rate of disease progression. Any fall in FVC to <80% of predicted with symptoms or FVC to <50% of predicted without symptoms or SNIP /MIP to < -40 cm H2O or hypercapnia would support the initiation of NIV or further testing as clinically indicated for individual NMD.
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For patients with NMD and sleep-related breathing disorders, we suggest using NIV for treatment. (C, VL)
Remarks: The panel suggests using the AASM criteria for sleep-disordered breathing and hypoventilation for adult patients and the ERS criteria for pediatric patients.
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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. (C, VL)
Remarks: PSG is not necessary for adult patients to initiate NIV, and PFT criteria alone may be adequate. See the comments for recommendations 4 & 5 above.
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For patients with NMD requiring NIV, we suggest individualizing NIV treatment to achieve ventilation goals. (C, VL)
Remarks: NIV can be optimized by adjusting parameters such as mode of ventilation, inspiratory time, and inspiratory and expiratory pressures. There is no strong evidence to support one mode of ventilation over another, although a backup respiratory rate may lead to better patient-ventilator synchrony and improved gas exchange. Patients with bulbar impairment may not be able to tolerate NIV or achieve adequate ventilation. The panel suggests an ongoing assessment of sleep quality, digital downloads, leaks, oximetry (capnography where available), and determining optimal settings along with optimizing secretion management.
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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. (C, VL)
Remarks: Although MPV has been used in various NMDs to help delay the transition to mechanical ventilation (MV), disease-specific considerations such as the development of bulbar symptoms in certain NMDs (e.g., ALS) may limit the use of this option.
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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. (C, VL)
Remark: Discussions regarding the use of MV should be started early in the course of the illness and include goals of care discussion, the potential need for institutionalization, and the burden on caregivers. The panel suggests optimizing secretion management and airway clearance, using patient preference, treatment goals, quality of life considerations, and available resources (cost and care providers) to help make decisions.
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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. (C, VL)
Remarks: The panel suggests an initial trial of an inexpensive oral anticholinergic medication. Clinicians can also consider more expensive but potentially longer-acting anticholinergic patch medication as the first or second-line therapy for sialorrhea.
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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. (C, VL)
Remarks: See individual studies for doses of BT. It is unclear whether clinicians should consider BT or radiation therapy (RT) first and can base their decision on local expertise.
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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). (C, VL)
Remarks: Data on RT are limited. See individual studies for doses. It is unclear whether clinicians should consider BT or RT first and can base their decision on local expertise.
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Airway Clearance Therapies
We suggest clinicians consider glossopharyngeal breathing for lung volume recruitment (LVR) and airway clearance for patients with NMD and hypoventilation. (C, VL)
Remarks: LVR is low-cost and can be performed by the patient independently with minimal assistance and training.
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For patients with NMD and reduced cough effectiveness, we suggest manually assisted cough techniques independently or added to other modalities such as LVR. (C, VL)
Remarks: Manually assisted cough techniques are low-cost but require caregiver assistance and training.
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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. (C, VL)
Remarks: LVR with a handheld device or mouthpiece is low-cost but requires caregiver assistance and training. Manually assisted cough is more effective when added to volume recruitment or the expiratory phase of mechanical cough assist.
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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). (C, VL)
Remarks: Implementing the recommendation requires caregiver assistance and training and a Mechanical In-Exsufflation device (cough assist device), which can increase costs and should be considered based on local resources.
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For patients with NMD and difficulties with secretion clearance, we suggest using high226 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. (C, VL)
Remarks: Implementing the recommendation requires caregiver assistance and training and an HFCWO device, which can increase costs and should be considered based on local resources and shared decision-making.
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Table - Recommended Therapies for Sialorrhea
| Therapy | Suggestions | Remarks |
| Anticholinergic medications | An initial trial of an inexpensive oral anticholinergic is suggested. Continue to use if the benefits are greater than the side effects. More expensive and potentially longeracting anticholinergic patch medication can also be considered. | Relatively inexpensive and readily available. Individual patient benefits and adverse events can be easily assessed. |
| Botulinum toxin (Botox) therapy to salivary glands | Limited data, doses are not defined. See individual studies for doses in the supplement. | Inexpensive, lasting beneficial effects on salivary function. May need to be repeated. Associated with viscous saliva & mild to moderate pain |
| Salivary gland radiation therapy (RT) | Limited data, doses not defined. See individual studies for doses in the supplement. | Long-lasting relief, however, is associated with irreversible dryness. Suggest reserving RT to experienced centers. |
Table - Recommended Airway Clearance Therapies
| Technique | Indications | Description | Remarks |
| Glossopharyngeal breathing (GBP/"frog breathing") | Hypoventilation | Positive pressure breathing method using muscles of the mouth, tongue pharynx, & larynx | Low cost Performed by the patient independently |
| Manually assisted cough (MAC) | Reduced cough effectiveness | Abdominal thrust or lateral costal compression to generate expiratory flow | Low cost Requires caregiver assistance |
| Lung volume recruitment (LVR/"breath stacking") | Reduced lung function or cough effectiveness | Handheld resuscitation bag or mouthpiece to inflate lungs to maximum inspiratory capacity without intervening expiration | Low cost Requires caregiver assistance |
| Mechanical insufflationexsufflation (cough assist device) | Reduced cough effectiveness not improved with alternative techniques | Alternating positive and negative pressure using a facemask or artificial airway. Effective for both upper and lower airway secretions | Expensive MI-E device Requires caregiver assistance. Reduces morbidity & hospitalization. Can have procedure intolerance |
| High-frequency chest wall oscillation (HFCWO) combined with cough assistance or LVR | Difficulties with secretion clearance | Fit-tested vest that produces vibrations to mobilize peripheral airway secretions which are then cleared with cough / LVR to improve expiratory airflow | Expensive HFCWO device. Requires caregiver assistance. Can have procedure intolerance |
Recommendation Grading
Disclaimer
The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.
Overview
Title
Respiratory Management of Patients with Neuromuscular Weakness
Authoring Organization
American College of Chest Physicians
Publication Month/Year
March 13, 2023
Document Type
Guideline
Country of Publication
US
Document Objectives
Respiratory failure is a significant concern in neuromuscular diseases (NMD). This CHEST guideline examines the literature on the respiratory management of patients with NMD to 11 provide evidence-based recommendations.
Inclusion Criteria
Male, Female, Adult, Older adult
Health Care Settings
Hospital
Intended Users
Nurse, nurse practitioner, physician, physician assistant, respiratory therapist
Scope
Management
Diseases/Conditions (MeSH)
D012124 - Respiratory Care Units, D012138 - Respiratory Therapy, D056152 - Respiratory Rate, D018908 - Muscle Weakness
Keywords
Neuromuscular Weakness, respiratory management
Source Citation
Khan A, Frazer-Green L, Amin R, Wolfe L, Faulkner G, Casey K, Sharma G, Selim B, Zielinski D, Aboussouan LS, McKim D, Gay P, Respiratory Management of Patients with Neuromuscular Weakness: An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report, CHEST (2023), doi: https://doi.org/10.1016/j.chest.2023.03.011