Patients suffering from neuromuscular diseases (NMD) often present with breathing difficulties: respiratory failure and the inability to mobilize secretions are frequently the cause of death. To help physicians manage the respiratory of these patients, the American College of Chest Physicians (ACCP) has released guidelines based on the best available evidence. The recommendations cover various aspects, such as lung function monitoring, non-invasive/invasive ventilation and respiratory clearance.
The premises
The ACCP guidelines were published in the journal Chest.The authors of the article are careful to emphasize the heterogeneity of NMDs, which occur at different ages and with more or less rapid progression, including decline in respiratory function. Randomized clinical trials are few and evidence is typically derived from observational studies. «What’s more, a significant portion of the evidence is based on amyotrophic lateral sclerosis (ALS) because data on other slowly progressing diseases are limited,» they write, «elements of the guidelines may need to be tailored to the rate of disease progression of the individual patient.»
Another necessary premise is that the guidelines of the ACCP find the United States of America as a natural area of application. Other scientific societies before the ACCP have already taken an interest in the problem. «The Swiss company and the French have recently proposed guidelines – say the authors of the article – however, [those guidelines] have limited application in the United States due to the absence of respiratory therapists and the national coverage directive to use ventilation mechanics with a different standard of care».Access to certain services or therapies included in the guidelines may depend on local resources and health insurance.
The panel of experts then recommends that each decision be discussed with the patient and family, respecting the patient’s preferences, evaluating treatment goals and taking into account quality of life.
The recommendations of the ACCP
We report here the summary of the guidelines.
Respiratory function monitoring
- In patients with NMD at risk of respiratory complications, pulmonary function tests (PFTs) are recommended to aid patient management decisions (good practice statement).
- In patients at risk of respiratory failure it is suggested to test lung function at least every 6 months, as appropriate depending on the course of each NMD (conditional, unclassified recommendation).
- In symptomatic patients with normal PFT and overnight oximetry, we suggest that polysomnography be considered to ascertain whether non-invasive ventilation (NIV) is clinically indicated (conditional recommendation, very low level of evidence; the strength of the recommendation and the level of evidence are also the same for all the following recommendations).
Non-invasive ventilation (NIV)
- In patients with chronic respiratory failure, NIV is recommended; Indications may depend on the disease, the rate of progression and the age of the patient.
- In patients with sleep-related breathing disorders it is suggested to use NIV.
- In patients with NMD it is suggested to use diagnostic parameters such as forced vital capacity (FVC), Maximum Inspiratory Pressure (MIP)/Maximum Expiratory Pressure (MEP), overnight oximetry (ONO) or evidence of disturbed breathing or hypoventilation in sleep to decide when NIV should be initiated.
- In patients requiring NIV it is suggested to tailor treatment to achieve ventilation goals.
- For patients with preserved bulbar function [patients with bulbar impairment may not tolerate NIV or achieve adequate ventilation] mouthpiece ventilation during the day is suggested in addition to NIV with mask at night.
Mechanical ventilation
- In patients who cannot stand or respond to NIV and in case of worsening bulbar function, frequent aspiration, insufficient cough, lung infections or decline in lung function, home mechanical ventilation (MV) by tracheostomy is suggested as an alternative to NIV.
Management of sialorrhea
- In patients with sialorrhea it is suggested to try an anticholinergic as the first line of therapy and to continue with use only if the benefits outweigh the side effects; Alternatively, anticholinergic patches can be used, more expensive but with longer action.
- In patients who do not respond or tolerate anticholinergic therapy, infiltration of the salivary glands with botulinum toxin is suggested.
- In patients who do not respond or tolerate anticholinergic therapy, radiotherapy of the salivary glands is suggested.
Airway clearance
- It is suggested that consideration be given to glossopharyngeal respiration for lung volume recruitment (LVR) and airway clearance for patients with NMD and hypoventilation.
- For patients with ineffective cough, assisted coughing techniques are suggested independent or in addition to other modalities (eg.LVR).
- For patients with reduced lung function or cough efficacy, regular use of LVR (breath stacking) via flask-ambu or mouthpiece is suggested.
- For patients with reduced cough efficacy who do not take adequate advantage of alternative techniques, the addition of mechanical insufflation-exsuflation (cough machine) is suggested.
- For patients with difficulties in secretion clearance, high-frequency chest wall oscillation (HFCWO) is suggested, which can be combined with clearance therapies such as assisted cough or LVR.
Access to the site is restricted and reserved for healthcare professionals
You have reached the maximum number of visits
Source — https://www.univadis.it/viewarticle/15-raccomandazioni-la-gestione-respiratoria-nelle-malattie-2023a10005xc