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Viewpoint
August 2, 2021

Respiratory Care for Patients With Amyotrophic Lateral Sclerosis in the US: In Need of Support

Author Affiliations
  • 1Pulmonary, Allergy, and Critical Care Division, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia
JAMA Neurol. 2021;78(9):1047-1048. doi:10.1001/jamaneurol.2021.2400

Fifteen years have elapsed since noninvasive ventilation was shown to improve survival and quality of life for patients with amyotrophic lateral sclerosis (ALS).1 Since that time, extraordinary technological advances in home-assisted ventilation and airway clearance devices have dramatically expanded opportunities to optimize respiratory care for people with ALS and other neuromuscular diseases.2 Pulmonologists at many medical centers in Western Europe and Canada have embraced the latest technology in patient care and clinical research. By and large, with some notable exceptions, pulmonologists in the US have not. This Viewpoint addresses the implications of that practice gap and asks the greater ALS community to help close it.

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    1 Comment for this article
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    Couple of points on the editorial
    Gregory Holt, PhD RRT DABSM | DME - Respiratory Quality Services
    Dr Flaschen was spot on discussing the relative lack of experienced pulmonary physicians working with the ALS population. The author's comments on new technologies deserves some feedback. The VOCSN, on the surface, appeals to many clinicians and ALS patients alike given it's combination capabilities. Ventec has been addressing problems but it can still be considered not quite ready for ALS. They have corrected the lack of a minimum IPAP setting. The algorithm would have reduced IPAP pressures to minimum settings (EPAP) if the patient was in distress and overventilating. As fatigue & failure set in, the pressures would increase. There is still a problem with the MIE (cough assist). You can't stop ventilation when activating MIE. The device will continue to deliver breaths while the patient attempts to synchronize cough. The majority of ALS patients are either on MIE or should be at some point in the disease process. In general, their lungs are free from pathology and O2 (as loud as one expects from an O2 concentrator behind a patient's head) is not indicated and the same for nebulizer function. The suction functions properly and the only issue is a proprietary disposables cost. Considering the online data capabilities, it is not a streaming function. The data is continuously recorded, but uploaded once daily. There are some nice features besides reviewing utilization and leak rates. The Evo's have compliance & resistance data that can be monitored, but again with ALS, not entirely necessary. Over time I can see the utility as abdominal and respiratory musculature loss influence pressures and lung mechanics. Currently, utilization assists with a need for a secondary ventilator or prompts discussions of tracheostomy (which many ALS patients do not want, but would rather go as far as NIV could take them and decide on end of life measures). I do like the author's call for increased training for both physicians and respiratory therapists alike. The RT's are in a good position to closely monitor respiration in the ALS population, and provide that data to the physician staff. With established ventilatory protocol orders, the RT can also make adjustments to the ventilator settings as the disease progresses (trigger sensitivity, pressure ranges, volume targeted modes, etc). In this respect, I can't agree with the author that respiratory mismanagement is the most common cause of NIV failure. There is that insidious disease process at work and it suggests close ventilatory management by trained clinicians and the support of 3rd party insurance companies to maintain that service for the ALS population.
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