Effect of Mechanical Insufflation-exsufflation on Respiratory Parameters in Two Neuromuscular Populations.

Study Overview
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Brief Summary
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A single session of Mechanical Insufflation-Exsufflation (with Cough AssistÒ) may improve airway flow and gas exchange in neuromuscular atrophy patients. The goal of this study is to confirm the beneficial effects of this treatment in a larger neuromuscular population, to study its mechanisms of action and to assess whether the effects observed persist over time. A better knowledge of this treatment should help to define its position in the respiratory management of neuromuscular patients.

Detailed Description
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30 SMA and DMD adult patients will be recruited in the home ventilation unit of the intensive care department of Raymond Poincaré Hospital (Assistance Publique-Hôpitaux de Paris,Garches, France). Respiratory parameters and comfort will be evaluated before and after (5min, 1h, 3h) a MI-E session. Vital capacity, maximal inspiratory and expiratory pressures (PImax and PEmax), peak cough flow and peak expiratory flows will be measured. Breathing pattern will be recorded allowing the measurements of respiratory frequency, tidal volume, Inspiratory time (Ti),total breath (Ttot) during tidal breathing and the calculation of tension-time index (TT0.1). Gas exchange will be evaluated by measuring end tidal CO2, transcutaneous CO2 and pulse oxymetry. Respiratory comfort will be assessed with a visual analog scale. Optoelectronic plethysmography will be use to perform regional ventilation evaluation. Using chest wall motion analysis we will compute respiratory participation of upper, lower thorax and abdomen, right and left side. Results should allow to analyse the effects of MI-E on regional ventilation. The mechanisms of action of MI-E on respiration and chest-wall motion will be analysed and the persistence of the beneficial effect of MI-E will be assessed.

Official Title
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Effect of Mechanical Insufflation-exsufflation on Respiratory Parameters in Two Neuromuscular Populations.

Conditions
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Mechanical Insufflation-exsufflation Session ( With Cough Assist)

Intervention / Treatment
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* Device: Plethysmography opto electronic
* Procedure: respiratory parameters


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Adults aged more than 18 years Written consents Neuromuscular disorders Hemodynamic stability Exclusion Criteria: Pulmonary disorder Acute respiratory failure Cognitive deficit

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 70 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Non-Randomized
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: cough assist session<br>Mechanical insufflation-exsufflation contains 15 cycles durea 2-3 seconds. pressure level fixed +/-30 cm H2O. | Device: Plethysmography opto electronic<br> <br> Procedure: respiratory parameters<br> <br> |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| maximal inspiratory pressures | measure of maximal inspiratory pressures before mechanical insufflation-exsufflation session, just after this session and an hour after. | 2 hours | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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neuromuscular disorders, mechanical insufflation-exsufflation, respiratory parameters, plethysmography opto electronic, Vital capacity