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Correspondence |

Noninvasive Ventilation for Patients With Neuromuscular Disease and Acute Respiratory FailureResponse FREE TO VIEW

Jesús Sancho, MD; Emilio Servera, MD, FCCP
Author and Funding Information

Affiliations: Hospital Clínico Universitario, Universitat de Valencia, Valencia, Spain,  Boston, MA

Correspondence to: Jesús Sancho, MD, Ave Blasco Ibañez 17, 46010 Valencia, Spain; e-mail: cchinesta@eresmas.com



Chest. 2008;133(1):314-315. doi:10.1378/chest.07-2180
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In our opinion, the very interesting article in CHEST (August 2007) by Garpestad et al1successfully 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 patients24 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. The lack of studies with a control group may have allowed Garpestad et al1 to exclude patients with NMD as candidates for NIV in acute settings; but, keeping in mind that most of these patients rejected TV, in our opinion a protocol in which randomization might suppose the death of some of the patients included is both ethically and technically unfeasible. In a previous study4 of patients who were unable to breath, we found that three of the four patients who previously had rejected TV, but not continuous NIV, survived an episode of ARF.

On obtaining the informed patient’s agreement to receive this treatment and in the absence of severe bulbar involvement,4 continuous NIV during ARF in NMD must be performed in a specific designated area with an available cohort of staff who have the appropriate experience. Matters to be considered are the appropriateness of the ventilation devices, the possibility of combining nasal or oronasal with mouthpiece interfaces, and the effectiveness of noninvasive aids to clear the patient’s airway secretions.

Patients should be carefully monitored and, if NIV fails, those who previously have accepted TV should be intubated without delay. In the patients who reject TV, all of the futile procedures (including NIV) should be interrupted, and adequate palliative care should be instituted immediately.

The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

The authors have no conflicts of interest to disclose.

Garpestad, E, Bernnan, J, Hill, NS (2007) Noninvasive ventilation for critical Care.Chest132,711-720. [PubMed] [CrossRef]
 
Vianello, A, Bevilacqua, M, Arcaro, G Noninvasive ventilatory approach to treatment of acute respiratory failure in neuromuscular disorders: a comparision with endotracheal intubation.Intensive Care Med2000;26,384-390. [PubMed]
 
Rabinstein, A, Widjicks, EF BiPAP in acute respiratory failure due to myasthenic crisis may prevent intubation.Neurology2002;59,1647-1649. [PubMed]
 
Servera, E, Sancho, J, Zafra, MJ, et al Alternatives to endotracheal intubation for patients with neuromuscular disease.Am J Phys Med Rehabil2005;84,851-857. [PubMed]
 
To the Editor:

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 review1but 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,2 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.3When 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.4 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.

References
Garpestad, E, Brennan, J, Hill, NS Noninvasive ventilation for critical care.Chest2007;132,711-720. [PubMed] [CrossRef]
 
Meduri, GU, Turner, RE, Abou-Shala, N, et al Noninvasive positive pressure ventilation via face mask.Chest1996;109,179-193. [PubMed]
 
Mehta, S, Hill, NS Noninvasive ventilation.Am J Respir Crit Care Med2001;163,540-577. [PubMed]
 
Bach, JR, Ishikawa, Y, Kim, H Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.Chest1997;112,1024-1028. [PubMed]
 

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Tables

References

Garpestad, E, Bernnan, J, Hill, NS (2007) Noninvasive ventilation for critical Care.Chest132,711-720. [PubMed] [CrossRef]
 
Vianello, A, Bevilacqua, M, Arcaro, G Noninvasive ventilatory approach to treatment of acute respiratory failure in neuromuscular disorders: a comparision with endotracheal intubation.Intensive Care Med2000;26,384-390. [PubMed]
 
Rabinstein, A, Widjicks, EF BiPAP in acute respiratory failure due to myasthenic crisis may prevent intubation.Neurology2002;59,1647-1649. [PubMed]
 
Servera, E, Sancho, J, Zafra, MJ, et al Alternatives to endotracheal intubation for patients with neuromuscular disease.Am J Phys Med Rehabil2005;84,851-857. [PubMed]
 
Garpestad, E, Brennan, J, Hill, NS Noninvasive ventilation for critical care.Chest2007;132,711-720. [PubMed] [CrossRef]
 
Meduri, GU, Turner, RE, Abou-Shala, N, et al Noninvasive positive pressure ventilation via face mask.Chest1996;109,179-193. [PubMed]
 
Mehta, S, Hill, NS Noninvasive ventilation.Am J Respir Crit Care Med2001;163,540-577. [PubMed]
 
Bach, JR, Ishikawa, Y, Kim, H Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.Chest1997;112,1024-1028. [PubMed]
 
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