Non-invasive Ventilation and Physical Activity

Study Overview
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Brief Summary
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As fatigue and daytime sleepiness are typical symptoms of alveolar hypoventilation in patients with neuromuscular disorders and thoracic deformations, we hope, by starting non-invasive ventilation and improving these symptoms, to increase their physical activity.

Detailed Description
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Patients with neuromuscular disorders and thoracic deformations develop alveolar hypoventilation during their disease progression. At that time, non-invasive ventilation(NIV) can be started as treatment. Fatigue and daytime sleepiness are typical symptoms of alveolar hypoventilation. NIV has the intention to improve these symptoms but no research has been performed whether these improvements also objectively improve physical activity. Further on, no research has been performed on muscle strength and muscle fatigue. Patients' physical activity, physical capacity and sleep quality will be measured before NIV initiation, after 3 months and 1 year of NIV use. To start NIV, patients will be admitted to our sleep laboratory for 5 days/4 nights. Polysomnography will be used during NIV titration. Physical activity will each time be measured by 2 activity monitors during 5 days.

Official Title
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Non-invasive Ventilation and Physical Activity in Patients With Neuromuscular Disorders and Thoracic Wall Disorders

Conditions
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Neuromuscular Diseases

Intervention / Treatment
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* Device: NIV


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: restrictive pulmonary function test AND symptoms of nocturnal alveolar hypoventilation or increased daytime pressure of arterial CO2 (PaCO2) (>45 mmHg) or >= 10 mmHg increase in PaCO2 during sleep in comparison to a normal awake supine value Exclusion Criteria: patients < 16 years severe cardiac arrythmias or conduction disturbances severe mental disturbances amyotrophic lateral sclerosis total wheelchair-dependent patients

Ages Eligible for Study
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Minimum Age: 16 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
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: Patients with RLD initiating NIV<br>Patients with RLD initiating NIV according to Belgian Health guidelines are followed-up before and after NIV initiation | Device: NIV<br> <br> |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Average daily walking time | Average daily walking time will be measured by the McRoberts physical activity monitor | Measured before initiation of NIV | 
| Average daily walking time | Average daily walking time will be measured by the McRoberts physical activity monitor | Measured after 3 months of NIV | 
| Average daily walking time | Average daily walking time will be measured by the McRoberts physical activity monitor | Measured after 1 year of NIV | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in functional exercise capacity | Six minute walking test | Before NIV initiation | 
| Change in muscle strength | Quadriceps muscle strength will be measured by magnetic stimulation. MicroFet measurements of wrist flexion/extension and ankle dorsiflexion/plantar flexion Respiratory muscle strength will be measured by maximal inspiratory pressure and sniff nasal inspiratory pressure | Before NIV initiation | 
| Change in quadriceps fatiguability | Use of magnetic stimulation before and after performing fatigue test of the quadriceps muscle | Before NIV intiation | 
| Change in sleep structure and quality of sleep | Full polysomnography will be used to get insight in sleep improvement | Before NIV intiation | 
| Change in functional exercise capacity | Six minute walking test | After 3 months of NIV use | 
| Change in functional exercise capacity | Six minute walking test | After 1 year NIV use | 
| Change in muscle strength | Quadriceps muscle strength will be measured by magnetic stimulation. MicroFet measurements of wrist flexion/extension and ankle dorsiflexion/plantar flexion Respiratory muscle strength will be measured by maximal inspiratory pressure and sniff nasal inspiratory pressure | After 3 months of NIV use | 
| Change in muscle strength | Quadriceps muscle strength will be measured by magnetic stimulation. MicroFet measurements of wrist flexion/extension and ankle dorsiflexion/plantar flexion Respiratory muscle strength will be measured by maximal inspiratory pressure and sniff nasal inspiratory pressure | After 1 year NIV use | 
| Change in quadriceps fatiguability | Use of magnetic stimulation before and after performing fatigue test of the quadriceps muscle | After 3 months of NIV use | 
| Change in quadriceps fatiguability | Use of magnetic stimulation before and after performing fatigue test of the quadriceps muscle | After 1 year NIV use | 
| Change in sleep structure and quality of sleep | Full polysomnography will be used to get insight in sleep improvement | After 3 months of NIV use | 
| Change in sleep structure and quality of sleep | Full polysomnography will be used to get insight in sleep improvement | After 1 year NIV use | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Noninvasive Ventilation, Physical activity