Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough.
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.
Miske
Laura J
LJ
Department of Nursing, University of Pennsylvania School of Medicine, The Children's Hospital of Philadelphia, Philadelphia 19104, USA. miske@email.chop.edu
Hickey
Eileen M
EM
Kolb
Susan M
SM
Weiner
Daniel J
DJ
Panitch
Howard B
HB
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