Use of the Mechanical In-Exsufflator in Pediatric Patients With Neuromuscular Disease and Impaired Cough*

Miske, Laura J. MSN
Hickey, Eileen M. CRNP
Kolb, Susan M. CRNP
Weiner, Daniel J. MD, FCCP
Panitch, Howard B. MD, FCCP

*From the Departments of Nursing (Mss. Miske, Hickey, and Kolb) and Pediatrics (Drs. Weiner and Panitch), University of Pennsylvania School of Medicine, The Children’s Hospital of Philadelphia, Philadelphia, PA.

Manuscript received July 9, 2003; revision accepted November 12, 2003.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail: [email protected]).

Correspondence to: Laura J. Miske, MSN, Pulmonary Division, Fifth Floor, Wood Building, Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104; e-mail: [email protected]

Chest 125(4):p 1406-1412, April 2004.

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.

Method:

Retrospective medical record review.

Participants:

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.

Conclusion:

In 90% of our study population, the use of an MI-E was safe, well-tolerated, and effective in preventing pulmonary complications.

Abbreviations: ANOVA = analysis of variance; CPF = cough peak flow; DMD = Duchenne muscular dystrophy; MI-E = mechanical in-exsufflator; MYO = congenital myopathy; NMD = neuromuscular disease; NS-NMD = nonspecific neuromuscular disease; Pemax = maximal expiratory pressure; Pimax = maximal inspiratory pressure; SMA = spinal muscle atrophy