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Editorials: Point and Counterpoint |

Rebuttal From Dr Bach FREE TO VIEW

John R. Bach, MD
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FINANCIAL/NONFINANCIAL DISCLOSURES: None declared.

Department of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School, Newark, NJ

CORRESPONDENCE TO: John R. Bach, MD, Department of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School, University Hospital B-403, 150 Bergen Street, Newark, NJ 07103


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2017;151(5):968-969. doi:10.1016/j.chest.2016.11.041
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Dr Panitch’s thoughtful commentary deserves further consideration. The French and Italian users of NIV (ie, bilevel positive airway pressure [PAP]) who underwent tracheotomies included no CNVS users; thus, when needing more than sleep NIV or when intubated, they underwent tracheotomy. We routinely extubate “unweanable” patients with SMA1, including infants, to CNVS, thereby avoiding tracheotomies indefinitely provided that parents learn and administer CNVS and MIE. Infants capable of 50-mL tidal volumes (intermediate type) invariably wean postextubation from CNVS to sleep NVS, for one-half of them until after 10 years of age, and 80% develop functional speech. No patients with intermediate or severe SMA1 who undergo tracheotomy subsequently develop speech and few if any wean from continuous to sleep-only tracheostomy ventilatory support. Indeed, almost invariably in England and France, the infants with severe SMA1 who would require continuous support before 5 months of age die rather than receive CNVS or undergo tracheotomy. However, the medications and other treatments in the pipeline today can only benefit the living.

Dr. Panitch noted that five of the patients in our series underwent tracheotomy after becoming CNVS dependent, but for three patients it was because they were out-of-state and not transferred to us for extubation. He noted that in the Italian study, 4-year survival was greater for the tracheostomy ventilatory support users but, because they automatically placed tracheotomies for unweanable intubated infants (rather than extubate to CNVS and MIE), there were no CNVS users to compare them with; in the French study, only six of 33 patients with SMA1 survived until the time of publication, and even they were only a mean 5 years of age. Currently, we have six CNVS users in our care who had no autonomous ability to breathe from as early as age 4 months who are now aged > 20 years.

Dr Panitch cited skin breakdown as a complication of CNVS but because all of the CNVS users in our series alternate interfaces, we have avoided this issue. He notes that although “nasal prong systems are reportedly useful at supplying bilevel support, the excessive leak that occurs…often leads to poor patient-ventilator synchrony.” However, we stopped using bilevel PAP years ago because pressure preset NVS from ventilators is easier for weak infants to trigger for synchrony and to reverse paradoxing.

When using MIE, airway collapse is transitory, which is why oxygen saturation levels return to baseline or greater several minutes after use. In addition, the reason MIE provides inadequate flows for bulbar amyotrophic lateral sclerosis but very effective flows for SMA1, especially after 3 years of age, is that upper motor neuron disease causes stridor and upper airflow obstruction, whereas with SMA, there is little or no stridor; thus, some of the 20-year-olds in our series with SMA1 generate > 350 L/m of MIE expiratory flows. Thus, intubation/hospitalization rates decrease dramatically after 3 years of age. The 23-year-old patient in Figure 5 was intubated 11 times; however, 10 intubations occurred by age 4½ years and one subsequently. It is time to stop assuming that all “ventilatory support” must be invasive, and “NIV” only bilevel PAP, and use full ventilator setting NVS instead.

References

Panitch H.B. . Counterpoint: Is noninvasive ventilation always the most appropriate manner of long-term ventilation for infants with spinal muscular atrophy type 1? No. Chest. 2017;151:965-968 [PubMed]journal
 
Bach J.R. . Point: Is noninvasive ventilation always the most appropriate manner of long-term ventilation for infants with spinal muscular atrophy type 1? Yes, almost always. Chest. 2017;151:962-965 [PubMed]journal
 
Gonçalves MR, Bach JR, Ishikawa Y, Saporito L, Winck JC. Outcomes of noninvasive management of end-stage neuromuscular respiratory muscle failure: a multicenter retrospective analysis. Eur Respir J Monographs. In press.
 
Villanova M. .Bach J.R. . Allogeneic mesenchymal stem cell therapy outcomes for three patients with spinal muscular atrophy type 1. Am J Phys Med Rehabil. 2015;94:410-415 [PubMed]journal. [CrossRef] [PubMed]
 

Figures

Tables

References

Panitch H.B. . Counterpoint: Is noninvasive ventilation always the most appropriate manner of long-term ventilation for infants with spinal muscular atrophy type 1? No. Chest. 2017;151:965-968 [PubMed]journal
 
Bach J.R. . Point: Is noninvasive ventilation always the most appropriate manner of long-term ventilation for infants with spinal muscular atrophy type 1? Yes, almost always. Chest. 2017;151:962-965 [PubMed]journal
 
Gonçalves MR, Bach JR, Ishikawa Y, Saporito L, Winck JC. Outcomes of noninvasive management of end-stage neuromuscular respiratory muscle failure: a multicenter retrospective analysis. Eur Respir J Monographs. In press.
 
Villanova M. .Bach J.R. . Allogeneic mesenchymal stem cell therapy outcomes for three patients with spinal muscular atrophy type 1. Am J Phys Med Rehabil. 2015;94:410-415 [PubMed]journal. [CrossRef] [PubMed]
 
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