Chest
Clinical Investigations in Critical CareSpinal Muscular Atrophy Type 1: A Noninvasive Respiratory Management Approach
Section snippets
Materials and Methods
Eleven consecutively referred SMA type 1 children in respiratory failure were managed as per a protocol (Table 1) that was approved by our Institutional Review Board. All 11 patients had severe skeletal and bulbar muscle weakness to the extent that none had functional extremity movements or ability to take any nutrition by mouth. Three have not developed the ability to verbalize. All of the parents of the 11 had refused tracheostomies on multiple occasions.
Nine of the 11 patients have required
Results
In all, 11 consecutively referred patients were treated by the respiratory aid protocol. The demographic data and the results of management are summarized in Table 2 . They had 28 distinct episodes of respiratory compromise necessitating hospitalizations: 2 postoperative, 2 associated with insidiously progressive inspiratory muscle dysfunction, and 24 sudden episodes mostly due to chest colds. These resulted in a total of 48 intubations. Nonprotocol therapy and extubation were attempted 20
Discussion
This study suggests that it may be possible for infants with SMA type 1 to avoid tracheostomy long enough to be able to cooperate with the use of respiratory muscle aids and possibly safely avoid tracheostomy indefinitely.6, 9 This is important because the parents of children with neuromuscular disease often refuse tracheostomy but want their children to survive.
Hypercapnia can cause oxyhemoglobin desaturation. We have noted that patients with neuromuscular disease tend to become symptomatic
ACKNOWLEDGMENT
We thank the University Hospital respiratory therapists, nurses, patients, and their parents, without whose support this study would not have been possible.
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Cited by (0)
This work was performed at the University Hospital of the University of Medicine and Dentistry of New Jersey-New Jersey Medical School in Newark, NJ.