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Changing Trends in the Management of End-Stage Neuromuscular Respiratory Muscle Failure

Recommendations of an International Consensus

Bach, John R., MD; Gonçalves, Miguel R., PhD; Hon, Alice, MD; Ishikawa, Yuka, MD; De Vito, Eduardo Luis, MD; Prado, Francisco, MD; Dominguez, Marie Eugenia, MD

American Journal of Physical Medicine & Rehabilitation: March 2013 - Volume 92 - Issue 3 - p 267–277
doi: 10.1097/PHM.0b013e31826edcf1
Review & Analysis

Objective Respiratory management of patients with end-stage respiratory muscle failure of neuromuscular disease has evolved from no treatment and inevitable respiratory failure to the use of up to continuous noninvasive intermittent positive pressure ventilatory support (CNVS) to avert respiratory failure and to permit the extubation of “unweanable” patients without tracheostomy. An international panel experienced in CNVS was charged by the 69th Congress of the Mexican Society of Pulmonologists and Thoracic Surgeons to analyze changing respiratory management trends and to make recommendations.

Design Neuromuscular disease respiratory consensuses and reviews were identified from PubMed. Individual respiratory interventions were identified; their importance was established by assessing the quality of evidence-based literature for each one and their patterns of use over time. The panel then determined the evidence-based strength for the efficacy of each intervention and made recommendations for achieving prolonged survival by CNVS.

Results Fifty publications since 1993 were identified. Continuous positive airway pressure, oxygen therapy, bilevel positive airway pressure used at both low and high spans, “air stacking,” manually assisted coughing, low pressure (<35 cm H2O) and high pressure (≥40 cm H2O) mechanically assisted coughing, noninvasive positive pressure ventilation part time (<23 hrs per day) and full time (>23 hrs per day; CNVS), extubation and decannulation of ventilator-dependent patients to CNVS, and oximetry feedback for noninvasive positive pressure ventilation and mechanically assisted coughing were identified. All noted interventions are being used with increasing frequency and were unanimously recommended to achieve prolonged survival by CNVS, with the exception of supplemental oxygen and continuous positive airway pressure, which are being used less and were not recommended for this population.

Conclusions CNVS and extubation of unweanable patients to CNVS are increasingly being used to prolong life while avoiding invasive interfaces.

From the Department of Physical Medicine and Rehabilitation, New Jersey Medical School, The University Hospital, University of Medicine and Dentistry of New Jersey, Newark (JRB, AH); the Department of Pulmonology, University Hospital of S. João, Faculty of Medicine, University of Porto, Porto, Portugal (MRG); National Organization Yakumo Hospital, Hokkaido, Japan (YI); Instituto de Investigaciones Médicas Alfredo Lanari, Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina (ELD); Domiciliary Noninvasive Ventilation Program, Health Chilean Ministry, Department of Pediatrics, Faculty of Medicine, Universidad de Chile, Santiago de Chile, Chile (FP); and Instituto National de Enfermidades Respiratorias, Universidad Nacional Autonoma de Mexico, Mexico City, Mexico (MED).

All correspondence and requests for reprints should be addressed to: John R. Bach, MD, Department of Physical Medicine and Rehabilitation, University Hospital B-403, 150 Bergen St, Newark, NJ 07103.

Miguel R. Gonçalves and John R. Bach wrote all the drafts of the article and gathered and analyzed all the data regarding the reviews and consensus. Alice Hon, Eduardo Luis De Vito, Yuka Ishikawa, Francisco Prado, and Marie Eugenia Dominguez gathered information from all the reviews and consensuses and added material to the text of the article. All authors read and approved the final article.

Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article.

© 2013 Lippincott Williams & Wilkins, Inc.