Mechanical insufflation-exsufflation (MI-E) devices increase expiratory air flow and thereby promote increased cough peak flow (CPF) in conjunction with a cough. There is little research looking at long-term use of MI-E in subjects with neuromuscular disease (NMD), and no long-term study has reported CPF, MI-E device settings, and adherence.
METHODS:
We evaluated 181 patient records (130 adults, 51 children) of individuals who received a MI-E device from our center between February 2014 and February 2018. Median age (interquartile range [IQR]) was 27 (14–51) y. Duchenne muscular dystrophy (DMD), spinal muscular atrophy (SMA), and amyotrophic lateral sclerosis (ALS) were the 3 most common diagnoses.
RESULTS:
MI-E devices were provided to the weakest subjects with a CPF < 160 L/min. Median (IQR) settings were insufflation, 25 (23–30) cm H2O, exsufflation −35 (−30 to −40) cm H2O, insufflation time 1.5 (1.3–1.7) s, exsufflation time 1.8 (1.5–2.0) s, and pause 1.5 (1.3–2.0) s. The inspiratory flow profile was set to high in all subjects, and no subject used supplemental oxygen with the MI-E device. When comparing insufflation pressures to exsufflation pressures, a greater negative pressure was used relative to positive pressure (P < .001). When comparing insufflation to exsufflation time, there was a significantly longer exsufflation duration (P < .001). Median (IQR) CPF at the start of MI-E was 60 (10–100) L/min. There was no correlation between either insufflation or exsufflation pressures and CPF. Median (IQR) usage for the group was 60% (13.5–100%) of days for the total days. Subjects with tracheostomies or SMA type I had the greatest adherence to treatment. Median (IQR) duration of MI-E use was 17 (8.5–32) months. Ninety-six percent of subjects were receiving ventilatory support.
CONCLUSIONS:
Greater exsufflation pressures than insufflation pressures, together with a shorter insufflation time than exsufflation time, were used. Predicting good adherence among the subjects was difficult. Subjects who produced daily secretions were more likely to use MI-E every day.
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