American journal of physical medicine & rehabilitation · 2025
Continuous Ventilatory Support Without Hospitalization or Tracheotomies: A Retrospective Observational Study of Two Centers' Outcomes for Duchenne Muscular Dystrophy.
СтатьяМногоцентровое исследованиеНаблюдательное исследованиеПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Bach John R, Ishikawa Yuka, Herrero Maria Victoria, Saporito Louis R, Liendro Macarena, Miura Toshihiko
Аннотация
INTRODUCTION: Duchenne muscular dystrophy management often results in tracheostomies or palliative care deaths.
METHODS: Two centers, A in the US and B in Japan, report 486 with Duchenne muscular dystrophy, including 263 dependent on continuous noninvasive ventilatory support, and mechanical in-exsufflation. When needing intubation, all were extubated to continuous noninvasive ventilatory support and mechanical in-exsufflation whether ventilator weanable or not.
RESULTS: Of 320 noninvasive ventilatory support users, 263 extended it to continuous noninvasive ventilatory support without hospitalization or acute respiratory failure. Another 57 were extubated to, and 15 decannulated to continuous noninvasive ventilatory support. Of 223 noninvasive ventilatory support users in Center A, 197 eventually required continuous noninvasive ventilatory support over 10.1 ± 6.3 yrs with 91 surviving to at least age 35.4 ± 9.0 (21-58) yrs; 106 died or were lost to follow-up at 34.3 ± 9.0 (18-58) yrs of age. Center B had 97 noninvasive ventilatory support/continuous noninvasive ventilatory support users; 31 died at 36.4 ± 8.1 (17-46) yrs of age. Sixty-six became continuous noninvasive ventilatory support dependent from age 24.3 with 35 now 38.2 ± 6.7 (29-51) yrs of age. The centers had 14 over age 50, 57 over age 40. Three underwent tracheotomies for chronic lung disease.
CONCLUSIONS: The facts that 263 became continuous noninvasive ventilatory support dependent without hospitalization, that 15 using trach ventilations were decannulated to continuous noninvasive ventilatory support, and that only three of 486 apparently required tracheotomies suggest that airway tubes are unnecessary for these patients without severe chronic lung disease.
METHODS: Two centers, A in the US and B in Japan, report 486 with Duchenne muscular dystrophy, including 263 dependent on continuous noninvasive ventilatory support, and mechanical in-exsufflation. When needing intubation, all were extubated to continuous noninvasive ventilatory support and mechanical in-exsufflation whether ventilator weanable or not.
RESULTS: Of 320 noninvasive ventilatory support users, 263 extended it to continuous noninvasive ventilatory support without hospitalization or acute respiratory failure. Another 57 were extubated to, and 15 decannulated to continuous noninvasive ventilatory support. Of 223 noninvasive ventilatory support users in Center A, 197 eventually required continuous noninvasive ventilatory support over 10.1 ± 6.3 yrs with 91 surviving to at least age 35.4 ± 9.0 (21-58) yrs; 106 died or were lost to follow-up at 34.3 ± 9.0 (18-58) yrs of age. Center B had 97 noninvasive ventilatory support/continuous noninvasive ventilatory support users; 31 died at 36.4 ± 8.1 (17-46) yrs of age. Sixty-six became continuous noninvasive ventilatory support dependent from age 24.3 with 35 now 38.2 ± 6.7 (29-51) yrs of age. The centers had 14 over age 50, 57 over age 40. Three underwent tracheotomies for chronic lung disease.
CONCLUSIONS: The facts that 263 became continuous noninvasive ventilatory support dependent without hospitalization, that 15 using trach ventilations were decannulated to continuous noninvasive ventilatory support, and that only three of 486 apparently required tracheotomies suggest that airway tubes are unnecessary for these patients without severe chronic lung disease.