Evaluation of Vital Capacity

Study Overview
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Brief Summary
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Respiratory failure is the most common cause of death in neuromuscular diseases. The aim of this research is to evaluate the thoracopulmonary recruitment by comparison of inspiratory vital capacity (VC ins)and inspiratory/expiratory vital capacity (VC ins-ex) with the vital capacity (VC) according to pathology and severity of pulmonary damage of patients with neuromuscular diseases. The methodology consists in realize VC by support of inspiratory/expiratory pressures, while the patient is passive and realizing a maximal inspiratory/expiratory effort simultaneously, with the aid of mechanical insufflation/exsufflation device (Cough Assist®). The evaluation will last for 30 minutes and the non-inclusion criteria are those for an insufflation. The study hypothesis is that long term insufflation/exsufflation device use could improve thoracopulmonary mobility.

Official Title
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Measurement of Assisted Vital Capacity to Appreciate the Level of Recruitment in Neuromuscular Diseases

Conditions
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Respiration Disorders, Neuromuscular Disease

Intervention / Treatment
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* Device: measures of assisted vital capacity by a mechanical insufflation/exsufflation (Cough Assist®)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: consent form signature Men and women aged over or equal to 18 years old Medical examination before the research Negative results of tests of pregnancy for the women at age to procreate or taking an effective contraceptive Patients with neuromuscular pathologies without tracheostmy - Vital Capacity < 80% of theoretical value Exclusion Criteria: Refuse to participate at the research Patient under guardianship or under trusteeship pregnant Women Patients unable to cooperate Patients without a security social scheme (as beneficiary or bearer of rights) Usual contra-indications for an insufflation (haemoptise, recent pneumothorax, pulmonary emphysema, pleuresy, nausea, chronic obstructive pulmonary diseasee or asthma, recent lobectomy, intracranial hypertension, troubles of consciousness, unable to talk)

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Supportive Care
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: measure of assisted vital capacity by mechanical insufflation<br>measure of assisted vital capacity by a mechanical insufflation/exsufflation | Device: measures of assisted vital capacity by a mechanical insufflation/exsufflation (Cough Assist®)<br>* measures of assisted vital capacity by a mechanical insufflation/exsufflation (Cough Assist®)<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Difference between spontaneous vital capacity and supported vital capacity at the inspiration and at inspiration/expiration at the same manoeuver. |  | 30 minutes |