Efficacy of mechanical insufflation-exsufflation in extubating unweanable subjects with restrictive pulmonary disorders.

BACKGROUND: Subjects who do not pass ventilator weaning parameters but whose ambient air oxyhemoglobin saturation can be normalized by mechanical insufflation-exsufflation (MIE) can be extubated to continuous noninvasive ventilatory support (CNVS) with MIE used to maintain extubation. Our aim was to study MIE-associated changes in breathing tolerance, pulse oximetry, and vital capacity (VC) for consecutive unweanable subjects. METHODS: A retrospective chart review was performed for consecutively referred intubated subjects with single-organ (respiratory muscle) failure. At presentation, CO2 was normalized by adjusting ventilator settings and VC was measured (point 1). Then, MIE was used via the tube up to every h until oximetry remained ≥ 95% on ambient air and VC was remeasured (point 2) immediately before extubation. Subjects who could not meet ventilator weaning criteria and had no ventilator-free breathing ability upon extubation to CNVS were enrolled. Post-extubation, the MIE was used to maintain oximetry ≥ 95% in room air. VC and breathing tolerance were remeasured within 3 weeks (point 3). RESULTS: Ninety-seven of 98 subjects were successfully extubated despite 45 having been CNVS-dependent for 4 months to 18 y before being intubated. Sixty-nine of the 98 were intubated for 24.9 ± 22 (range 1-158) d and failed 0-6 (mean 1.7) extubation attempts before being transferred and successfully extubated in 2.24 ± 1.78 (range < 1-8) d to CNVS. VC increased by 270% (P < .001) from points 1 to 3. Weaning from CNVS to part-time NVS was achieved by all 52 subjects who had not been CNVS-dependent before intubation. One subject underwent tracheotomy. CONCLUSIONS: Many unweanable subjects can be extubated to CNVS and MIE. The latter can normalize O2 saturation, increase VC, and facilitate extubation.

Decanulation of patients with severe respiratory muscle insufficiency: efficacy of mechanical insufflation-exsufflation.

OBJECTIVE: Ventilator dependent patients with neuromuscular disorders and high level spinal cord injury have been extubated and decanulated to continuous noninvasive intermittent positive pressure ventilatory support after mechanical insufflation-exsufflation was used to achieve specific criteria for tube removal. The purpose of this study is to report changes in extent of need for ventilator use and in vital capacity related to mechanical insufflation-exsufflation used via tracheostomy tubes and post-decanulation via oronasal interfaces. METHODS: Upon presentation patients were placed on fiO2 21% and CO2 was normalized by adjusting ventilator settings as needed. The vital capacity (1st data point) and h/day of ventilator dependence were noted. Then mechanical insufflation-exsufflation was used via the tubes up to every 2 h until ambient air oxyhemoglobin saturation (SpO2) baseline remained ≥ 95% and other decanulation criteria were achieved. The vital capacity was re-measured (2nd data point) and the patient decanulated to continuous noninvasive intermittent positive pressure ventilatory support in ambient air as care providers used mechanical insufflation-exsufflation up to every 30 min to maintain SpO2 ≥ 95%. The vital capacity (3rd data point) and minimum hours/day of noninvasive intermittent positive pressure ventilatory support requirement during the next 3 weeks were recorded. RESULTS: The vital capacities of 61 tracheostomized ventilator users, 36 of whom were continuously dependent, increased significantly (p < 0.001) from presentation to immediately pre-decanulation and in the 3 weeks post-decanulation and all except one were successfully decanulated. CONCLUSION: Many ventilator users can be decanulated in outpatient clinics to continuous noninvasive intermittent positive pressure ventilatory support with mechanical insufflation-exsufflation used to increase vital capacity, SpO2, and autonomous ability to breathe.

Long-term survival in Werdnig-Hoffmann disease.

OBJECTIVES: To report long-term survival of spinal muscular atrophy type 1 (SMA 1) and consequences on speech and ventilator dependence as a function of mode of ventilator use. DESIGN: A retrospective chart review of 106 consecutively referred SMA 1 patients, the 92 most severe of which were considered in three groups: untreated (group 1), tracheostomy managed (group 2), and noninvasively managed (group 3). RESULTS: The untreated patients died at 9.6 +/- 4.0 mos of age. The mean age of the 22 patients referred with tracheostomy tubes (group 2) was 70.5 +/- 43.3 mos (range 2-159 mos); five died at 66.2 +/- 114.2 mos (range 8-270 mos) of age. Six had comprehendible speech at the time of tracheotomy and retained some ability to vocalize afterward. None of the 21 patients who had not developed the ability to speak did so after tracheotomy. Twenty-five of the 27 total lost all autonomous breathing ability immediately, and definitively, after tracheotomy. The 47 patients who used noninvasive mechanical ventilation (NIV) (group 3) were extubated to it during episodes of acute respiratory failure. Thirty-nine of these were 65.2 +/- 45.8 mos (range 11-153 mos) of age, and eight died at 60.9 +/- 26.1 mos (range 36-111 mos) of age. There was no significant difference in longevity with or without tracheostomy, but the NIV patients had significantly fewer (P = 0.04) hospitalizations per year after age 5; 39 of the 47 could communicate verbally, and only nine were continuously dependent on NIV. CONCLUSIONS: NIV and tracheostomy can both prolong survival for SMA 1 patients, but the latter results in continuous ventilator dependence and speech does not develop.