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Efficacy of Mechanical Insufflation-Exsufflation in Medically Stable Patients With Amyotrophic Lateral Sclerosis^*

^* From the Department of Respiratory Medicine, Hospital Clínico Universitario, Universitat de València, Valencia, Spain.

Correspondence to: Emilio Servera MD, FCCP, Avda Blasco Ibáñez 84, E 46021 Valencia, Spain; e-mail: emilio.servera{at}uv.es

Objective: To determine under what circumstances the use of mechanical insufflation-exsufflation (MI-E) can generate clinically effective expiratory flows for airway clearance (> 2.7 L/s) for clinically stable patients with amyotrophic lateral sclerosis (ALS).

Materials and method: Twenty-six consecutive patients with ALS were studied, 15 with severe bulbar dysfunction. Using a pneumotachograph and with the aid of an oronasal mask, we measured FVC, FEV₁, peak cough flow (PCF), maximum insufflation capacity (MIC), PCF generated from a maximum insufflation MIC (PCFMIC), and PCF generated by MI-E (PCFMI-E). MI-E was delivered at ± 40 cm H₂O. Maximum inspiratory pressure (PImax) and maximum expiratory pressure (PEmax) at the mouth were also measured.

Results: Although both groups had a similar time from ALS symptom onset to diagnosis, statistical differences (p < 0.05) were found between nonbulbar and bulbar patients in lung function and cough capacity parameters: FVC, 2.58 ± 1.24 L vs 1.62 ± 0.74 L; FEV₁, 2.26 ± 1.18 L vs 1.54 ± 0.69 L; PImax, – 93.45 ± 47.47 cm H₂O vs – 3.64 ± 25.07 cm H₂O; PEmax, 140.45 ± 75.98 cm H₂O vs 69.93 ± 32.14 cm H₂O; MIC, 3.02 ± 1.22 L vs 1.97 ± 0.75 L; PCF, 5.91 ± 2.55 L/s vs 3.42 ± 1.44 L/s; PCFMIC, 6.68 ± 2.71 L/s vs 4.00 ± 1.48 L/s; and PCFMI-E, 4.34 ± 0.82 L/s vs 3.35 ± 0.77 L/s. Four patients with bulbar dysfunction and MIC > 1 L had PCFMI-E < 2.7 L/s. The receiver operating characteristic (ROC) curve analysis showed PCFMIC of 2.7 L/s predicting those patients with PCFMI-E < 2.7 L/s. The ROC curve analysis showed PCFMIC > 4 L/s predicting those patients with PCFMIC greater than PCFMI-E.

Conclusion: MI-E is able to generate clinically effective PCFMI-E (> 2.7 L/s) for stable patients with ALS, except for those with bulbar dysfunction who also have a MIC > 1 L and PCFMIC <2.7 L/s who probably have severe dynamic collapse of the upper airways during the exsufflation cycle. Clinically stable patients with mild respiratory dysfunction and PCFMIC > 4 L/s might not benefit from MI-E except during an acute respiratory illness.

Key Words: amyotrophic lateral sclerosis • cough capacity • lung function test • mechanical insufflation-exsufflation • neuromuscular disease • noninvasive respiratory aids • noninvasive ventilation • peak cough flow

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