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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Patients with RLD initiating NIV | Other | Patients with RLD initiating NIV according to Belgian Health guidelines are followed-up before and after NIV initiation |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| NIV | Device |
|
| 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 |
| 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 |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Dries Testelmans, MD, PhD | Universitaire Ziekenhuizen KU Leuven | Principal Investigator |
| Bertien Buyse, MD,PhD | Universitaire Ziekenhuizen KU Leuven | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| UZ Leuven | Leuven | 3000 | Belgium |
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| ID | Term |
|---|---|
| D009468 | Neuromuscular Diseases |
| D009043 | Motor Activity |
| ID | Term |
|---|---|
| D009422 | Nervous System Diseases |
| D001519 | Behavior |
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| 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 |