Noninvasive Ventilation for Patients With Neuromuscular Disease and Acute Respiratory Failure: Response

Abstract

We strongly agree with Sancho and Servera et al that patients with acute respiratory failure (ARF) associated with neuromuscular disease (NMD) can be treated successfully with noninvasive ventilation (NIV) in the critical care setting. We did not mention this in our recent review1Garpestad E Brennan J Hill NS Noninvasive ventilation for critical care.Chest. 2007; 132: 711-720Abstract Full Text Full Text PDF PubMed Scopus (115) Google Scholar but did not mean to suggest that such patients should be excluded as candidates for NIV. Our review was an update focusing on developments over the previous several years. Applications of NIV for ARF in NMD patients are relatively uncommon in most acute care hospitals,2Meduri GU Turner RE Abou-Shala N et al.Noninvasive positive pressure ventilation via face mask.Chest. 1996; 109: 179-193Abstract Full Text Full Text PDF PubMed Scopus (425) Google Scholar and there have been few recent relevant publications. Also, as Servera et al point out, there are no randomized controlled trials evaluating this application of NIV, although this should not discourage the use of NIV for appropriate NMD patients. However, NIV should be used with extreme caution in NMD patients with rapidly progressive NMD syndromes such as myasthenia gravis or Guillian Barre syndrome, especially when bulbar muscles are involved. In the outpatient setting, NIV for NMD has assumed a more prominent role as the ventilatory mode of first choice for most such patients.3Mehta S Hill NS Noninvasive ventilation.Am J Respir Crit Care Med. 2001; 163: 540-577Crossref PubMed Scopus (913) Google Scholar When these patients have acute exacerbations, we have them go on NIV around the clock and use cough-assist techniques such as the mechanical inexsufflator as often as necessary to facilitate secretion removal.4Bach JR Ishikawa Y Kim H Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.Chest. 1997; 112: 1024-1028Abstract Full Text Full Text PDF PubMed Scopus (426) Google Scholar Acute hospitalization can be very disruptive for NMD patients who are unfamiliar to the hospital and its staff, and can be successfully managed at home by experienced family members and well-trained caregivers. When such patients must be hospitalized, usually because of difficulty in handling secretions, we agree that they should be placed in a specialized unit, usually an ICU, and not on a regular medical floor where the nursing staff is usually inadequately equipped to prevent problems with secretion retention. Techniques to aid secretion removal must be applied aggressively, but even then temporary intubation may be necessary. Once secretions have abated, though, the patient can often be extubated and NIV resumed. Noninvasive Ventilation for Patients With Neuromuscular Disease and Acute Respiratory FailureCHESTVol. 133Issue 1PreviewIn our opinion, the very interesting article in CHEST (August 2007) by Garpestad et al1 successfully contributed to a better understanding of noninvasive ventilation (NIV). However, we missed one potentially important indication for NIV, part time or continuous ventilatory support during an episode of acute respiratory failure (ARF) in patients with neuromuscular disease (NMD). Although the few studies on these patients234 have been designed without a randomized control group that utilized tracheostomy ventilation (TV), all of them have underlined the effectiveness of NIV on the basis of two consistent outcomes: preventing endotracheal intubation; and avoiding mortality during these episodes. Full-Text PDF