Effect of Noninvasive Ventilation on Lung Function in Amyotrophic Lateral Sclerosis

Study Overview
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Brief Summary
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Amyotrophic Lateral Sclerosis (ALS), or Lou Gehrig's Disease, is a fatal disorder that causes progressive degeneration and weakening of the muscles of breathing, leading to breathing insufficiency and eventually breathing failure. This breathing insufficiency is commonly treated with a breathing assistance device, known as noninvasive positive pressure ventilation (NIPPV). While generally well tolerated and accepted, it is not clear whether or to what extent NIPPV in fact helps breathing function: some data suggest that NIPPV preserves breathing function over time, whereas other data suggest that it actually causes breathing function to decline more quickly. No studies have shown what the acute effect of NIPPV is on breathing muscle function in ALS patients. This study will test the hypothesis that the acute use of NIPPV, at pressure levels that are in common clinical use, will cause measurable changes in tests of breathing function, compared to baseline and to lower levels of NIPPV. We expect that the results of this study will help to clarify whether and to what extent NIPPV assists respiratory muscle function in patients with ALS.

Official Title
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Effect of Noninvasive Positive Pressure Ventilation on Pulmonary Function Testing in Amyotrophic Lateral Sclerosis

Conditions
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Amyotrophic Lateral Sclerosis, Motor Neuron Disease

Intervention / Treatment
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* Device: noninvasive positive pressure ventilation
* Device: noninvasive positive pressure ventilation


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: clinical diagnosis of amyotrophic lateral sclerosis clinical indication to start noninvasive ventilation (forced vital capacity < 50% predicted or signs/symptoms of respiratory insufficiency) age 18 to 80 years old Exclusion Criteria: prior institution of NIPPV inability to safely use NIPPV indications for tracheostomy assisted ventilation because of inability to clear secretions from the airway inability or unwillingness to perform pulmonary function testing presence of advanced dementia.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 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: Supportive Care
Allocation: Randomized
Intervention Model: Crossover Assignment
Masking: Single


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: High-level ventilation<br>Each subject will spend 2 hours receiving high-level noninvasive ventilation. | Device: noninvasive positive pressure ventilation<br>* Each subject will undergo 2 hours of high-level noninvasive positive pressure ventilation, with an inspiratory positive airway pressure of 12 cm H2O and an expiratory positive airway pressure of 3 cm H2O.<br>* Other names: BiPAP (Respironics), variable positive airway pressure (VPAP) (ResMed), bilevel positive airway pressure, noninvasive ventilation;|
| Active Comparator: Low-level ventilation<br>Each subject will receive 2 hours of low-level noninvasive positive pressure ventilation. | Device: noninvasive positive pressure ventilation<br>* Each subject will undergo 2 hours of low-level noninvasive positive pressure ventilation, with an inspiratory positive airway pressure of 6 cm H2O and an expiratory positive airway pressure of 3 cm H2O.<br>* Other names: BiPAP (Respironics), VPAP (ResMed), bilevel positive airway pressure, noninvasive ventilation;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Difference in Pulmonary Function and Respiratory Muscle Pressure Testing |  | 5 hours | 
| Difference in Gas Exchange |  | 5 hours | 
| Difference in Subjective Dyspnea Between Baseline and the Two Different Ventilator Modes |  | 5 hours | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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noninvasive ventilation