Duchenne muscular dystrophy: Prolongation of life by noninvasive ventilation and mechanically assisted coughing

Elia Gomez-Merino, John Bach

Research output: Contribution to journalArticle

190 Citations (Scopus)

Abstract

Objective: To quantitate prolongation of survival for patients with Duchenne muscular dystrophy with the use of noninvasive intermittent positive-pressure ventilation (IPPV) with and without access to a protocol involving mechanically assisted coughing. Design: In this retrospective review of all patients with Duchenne muscular dystrophy visiting a neuromuscular disease clinic, patients were trained to use mouth piece and nasal IPPV and mechanically assisted coughing to maintain oxyhemoglobin saturation >94% (protocol). Survival was considered prolonged when noninvasive IPPV was required full time. Results: Ninety-one of 125 patients used noninvasive IPPV part time for 1.9 ± 1.3 yr, and 51 went on to require it full time for 6.3 ± 4.6 yr. Of the 31 noninvasive IPPV users who died without access to the protocol, 20 died from respiratory causes and seven died from cardiac causes. None of the 34 full-time noninvasive IPPV users with access to the protocol underwent tracheotomy or died from respiratory complications during a period of 5.4 ± 4.0 yr, whereas three died from heart failure. Five patients with no breathing tolerance were extubated or decannulated to continuous noninvasive IPPV. Conclusions: Noninvasive respiratory aids can prolong survival and permit extubation or decannulation of patients with Duchenne muscular dystrophy with no breathing tolerance.

Original languageEnglish (US)
Pages (from-to)411-415
Number of pages5
JournalAmerican Journal of Physical Medicine and Rehabilitation
Volume81
Issue number6
DOIs
StatePublished - Jun 6 2002

Fingerprint

Life Support Care
Intermittent Positive-Pressure Ventilation
Noninvasive Ventilation
Duchenne Muscular Dystrophy
Survival
Respiration
Neuromuscular Diseases
Tracheotomy
Oxyhemoglobins
Nose
Mouth
Heart Failure

All Science Journal Classification (ASJC) codes

  • Rehabilitation
  • Physical Therapy, Sports Therapy and Rehabilitation

Keywords

  • Cough
  • Duchenne Muscular Dystrophy
  • Exsufflation
  • Intermittent Positive- Pressure Ventilation
  • Noninvasive Mechanical Ventilation
  • Respiratory Paralysis
  • Respiratory Therapy

Cite this

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abstract = "Objective: To quantitate prolongation of survival for patients with Duchenne muscular dystrophy with the use of noninvasive intermittent positive-pressure ventilation (IPPV) with and without access to a protocol involving mechanically assisted coughing. Design: In this retrospective review of all patients with Duchenne muscular dystrophy visiting a neuromuscular disease clinic, patients were trained to use mouth piece and nasal IPPV and mechanically assisted coughing to maintain oxyhemoglobin saturation >94{\%} (protocol). Survival was considered prolonged when noninvasive IPPV was required full time. Results: Ninety-one of 125 patients used noninvasive IPPV part time for 1.9 ± 1.3 yr, and 51 went on to require it full time for 6.3 ± 4.6 yr. Of the 31 noninvasive IPPV users who died without access to the protocol, 20 died from respiratory causes and seven died from cardiac causes. None of the 34 full-time noninvasive IPPV users with access to the protocol underwent tracheotomy or died from respiratory complications during a period of 5.4 ± 4.0 yr, whereas three died from heart failure. Five patients with no breathing tolerance were extubated or decannulated to continuous noninvasive IPPV. Conclusions: Noninvasive respiratory aids can prolong survival and permit extubation or decannulation of patients with Duchenne muscular dystrophy with no breathing tolerance.",
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