Practical Guide to Management of Long-Term Noninvasive Ventilation for Adults With Chronic Neuromuscular Disease

Abstract

Recent technological advances in respiratory support and monitoring have dramatically enhanced the utility of long-term noninvasive ventilation. Improvements in quality of life and prolonged survival at home have been demonstrated for several common chronic neuromuscular diseases. Many adults with progressive neuromuscular respiratory disease can now comfortably maintain normal ventilation at home to near total respiratory muscle paralysis without needing a tracheostomy. However, current practice in many communities falls short of that potential. Mastery of the new technology calls for detailed awareness of the respiratory cycle, expert knowledge of mechanical devices, facial interfaces, quantitative monitoring tools for home ventilation, and a willingness to stay current in a rapidly expanding body of clinical research. The depth and breadth of the expertise required to manage home assisted ventilation is giving rise to a new focused medical subspecialty in chronic respiratory failure at the interface between pulmonology, critical care, and sleep medicine. For clinicians seeking pragmatic "how to" guidance, this primer presents a comprehensive, physician-directed management approach to long-term noninvasive ventilation of adults with chronic neuromuscular respiratory disease. Bilevel devices, portable ventilators, ventilation modalities, terminology, and monitoring strategies are reviewed in detail. Building on that knowledge base, we present a step-by-step guide to initiation, refinement, and maintenance of home noninvasive ventilation that is tailored to patientcentered goals of therapy. The "quantitative" approach recommended here fully incorporates routine monitoring of home assisted ventilation using technologies that have only recently become widely available including cloud-based device telemonitoring and noninvasive measurements of blood gases. Strategies for troubleshooting and problem solving are included.

Translation, Adaptation, and Validation of the Swedish Serious Illness Conversation Guide

“…[25][26][27][28][29][30] Using positive and negative framing can support the clinician in conveying prognostic information 26,30 as well as support patients' prognostic awareness. 29 Patients are often receptive to the dual approach 31 preferring phrases with hope/worry statements when discussing prognostic information. 32 Importantly, consideration of the patient's need for prognostic information is shown by asking if the patient wants to know what the clinician thinks about their illness (physicians) or health (nurses) now and in the future.…”

International Survey of the Tools Used for Assessment, Monitoring and Management of Home Mechanical Ventilation Patients

“…Furthermore, in line with previous EUROVENT data [1], other chronic lung diseases are treated with HMV. Despite guidelines [3,5] stating that carbon dioxide measurements (arterial blood gas or transcutaneous) are mandated in order to make a diagnosis of nocturnal hypoventilation-with recent data highlighting a targeted reduction in daytime and nocturnal carbon dioxide levels improving the patients' clinical outcome [6,7,[16][17][18][19][20]-the current survey showed that when initiating HMV and prior to discharge post initiation, the SpO 2 monitoring and physiological and ventilator downloaded data were employed more frequently than carbon dioxide measurements. Surprisingly, some respondents reported that they never use carbon dioxide measurements to titrate HMV.…”

“…Once established on HMV, the titration of HMV in patients with chronic respiratory failure should target a reduction in daytime PaCO 2 [6][7][8][9] and, therefore, a post initiation overnight assessment of both SpO 2 and TcCO 2 and daytime PaCO 2 are expected. Despite this recommendation, one HMV survey highlighted that clinicians expressed a low level of agreement with the investigations that are required prior to HMV prescription, with SpO 2 as the main driver for the initiation of HMV in chronic obstructive pulmonary disease (COPD) [10].…”

Diagnosis and management of Becker muscular dystrophy: the French guidelines

“…• Mechanical ventilation: Assisted ventilation (nocturnal noninvasive ventilation or invasive ventilation on tracheostomy) must be proposed as soon as diurnal and/or nocturnal alveolar hypoventilation appears (defined as pCO2 > 45 mmHg or TcpCO2 > 50 mmHg at night) [89].…”