title: Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough.
abstract: BACKGROUND
  Impaired cough secondary to weakness from neuromuscular disease (NMD) can cause serious respiratory complications, including atelectasis, pneumonia, small airway obstruction, and acidosis. The mechanical in-exsufflator (MI-E) delivers a positive-pressure insufflation followed by an expulsive exsufflation, thereby simulating a normal cough. Use of the MI-E in adults with impaired cough results in improved cough flows and enhanced airway clearance. However, only limited reports of MI-E use in children exist.
  
  
  OBJECTIVE
  To determine the safety, tolerance, and effectiveness of the MI-E in a pediatric population.
  
  
  METHODS
  Retrospective medical record review.
  
  
  METHODS
  Sixty-two patients (34 male patients) observed in a pediatric pulmonary program with NMD and impaired cough in whom MI-E therapy was initiated. Median age at initiation of MI-E use was 11.3 years (range, 3 months to 28.6 years). Diagnoses included the following: Duchenne muscular dystrophy (17 patients); spinal muscular atrophy, types I and II (21 patients); myopathy (12 patients); other nonspecific NMD (12 patients). Mechanical ventilation via tracheostomy was used in 29 patients, and 25 patients used noninvasive ventilation.
  
  
  RESULTS
  The median duration of use was 13.4 months (range, 0.5 to 45.5 months). One infant died before using MI-E at home. Five patients chose not to continue MI-E therapy. Complications were reported in two patients, but ultimately they used the MI-E device. Chronic atelectasis resolved in four patients after beginning MI-E therapy, and five patients experienced a reduction in the frequency of pneumonias.
  
  
  CONCLUSIONS
  In 90% of our study population, the use of an MI-E was safe, well-tolerated, and effective in preventing pulmonary complications.
authors.affiliation: Department of Nursing, University of Pennsylvania School of Medicine, The Children's Hospital of Philadelphia, Philadelphia 19104, USA. miske@email.chop.edu
authors.forename: Laura J
authors.initials: LJ
authors.lastname: Miske
authors.forename: Eileen M
authors.initials: EM
authors.lastname: Hickey
authors.forename: Susan M
authors.initials: SM
authors.lastname: Kolb
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Weiner
authors.forename: Howard B
authors.initials: HB
authors.lastname: Panitch
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003371:Cough / Q000503:physiopathology / Q000628:therapy*; D045853:Exhalation; D005260:Female; D006801:Humans; D007223:Infant; D007327:Insufflation / Q000295:instrumentation*; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000628:therapy; D020388:Muscular Dystrophy, Duchenne / Q000628:therapy; D009468:Neuromuscular Diseases / Q000628:therapy*; D011014:Pneumonia / Q000517:prevention & control; D011175:Positive-Pressure Respiration / Q000295:instrumentation*; D012189:Retrospective Studies; D012449:Safety