Patients with neuromuscular diseases (NMD) face an increased risk of respiratory muscle weakness, which can contribute to various health problems. These include chronic respiratory failure, sleep-related breathing disorders, sialorrhea, and reduced cough effectiveness. In collaboration with AASM, AARC, and ATS, CHEST has developed guidelines to help clinicians manage patients with NMD. using the population, intervention, comparator, and outcome (PICO) format using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluations) methodology.
A few of the key recommendations are as follows:
1. Addressing the use and timing of pulmonary function tests (PFT), the panel suggests measuring vital capacity (FVC or SVC), MIP/MEP, SNIP, or PCF in patients with NMD every 6 months.
2. For the detection of respiratory failure and sleep-related breathing disorders in symptomatic patients with NMD who have normal PFT and overnight oximetry (ONO), the panel suggested that clinicians consider polysomnography (PSG) to assess whether noninvasive ventilation (NIV) would be beneficial. Adult patients do not have to have PSG to manage NMD if the PFT or ONO criteria support using NIV.
3. The panel recommends the use of NIV for the treatment of respiratory failure. To guide the initiation of NIV, clinicians can use 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. The panel recommended individualizing treatment.
4. The panel suggested mouth piece ventilation (MPV) for daytime ventilatory support in patients with preserved bulbar function. Its desirable effects include delaying or avoiding tracheostomy and improving speech, cough effectiveness, and coordination of breathing and swallowing.
5. Invasive home mechanical ventilation (MV) by tracheostomy was identified as an acceptable option for patients with progressive respiratory failure, particularly those who were unable to clear secretions. Because of the high costs and caregiver burden, the guideline highlights the need to consider patient preferences, tolerability, the ability to maintain mouthpiece ventilation, and the availability of resources when choosing an appropriate treatment option.
6. The panel suggested practicing clinicians address the management of sialorrhea and airway clearance techniques in patients with NMD, as they face the risk of aspiration and pneumonia. For sialorrhea, the panel suggests starting with a trial of anticholinergic agents, as they are inexpensive and readily available. The panel also provided advice on botulinum toxin therapy and radiation therapy, which have limited data and should be reserved for experienced centers.
7. The panel reviewed data on airway clearance techniques, including glossopharyngeal breathing (GPB), mechanical insufflation-exsufflation (MI-E), also commonly known as cough-assist device, manually assisted cough, lung volume recruitment (LVR) by air stacking, and high-frequency chest wall oscillation (HFCWO). The panel suggested using airway clearance techniques based on local resources, expertise, and shared decision-making with patients.
The panel stressed the importance of respect for patient preferences, treatment goals, and quality of life considerations. The panel emphasized the need to modernize and improve access to ventilatory support for patients with NMD and the role of shared decision-making in improving quality of life and long-term outcomes. The panel also suggests that randomized controlled trials in patients with NMD would help establish a higher grade of evidence.
Dr. Hubel and Dr. Khan are from the Division of Pulmonary Allergy and Critical Care Medicine, Oregon Health and Science University, Portland.
Reference
Khan A et al. Respiratory management of patients with neuromuscular weakness: An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report [published online ahead of print, 2023 Mar 13]. Chest. 2023;S0012-3692(23)00353-7. doi: 10.1016/j.chest.2023.03.011.