Neuromuscular Diseases
Conditions
Keywords
Noninvasive Ventilation, Physical activity
Brief summary
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
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.
Interventions
DEVICENIV
Sponsors
Universitaire Ziekenhuizen KU Leuven
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
16 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Average daily walking time | Measured before initiation of NIV | Average daily walking time will be measured by the McRoberts physical activity monitor |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Change in functional exercise capacity | Before NIV initiation | Six minute walking test |
| Change in muscle strength | Before NIV initiation | 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 |
| Change in quadriceps fatiguability | Before NIV intiation | Use of magnetic stimulation before and after performing fatigue test of the quadriceps muscle |
| Change in sleep structure and quality of sleep | Before NIV intiation | Full polysomnography will be used to get insight in sleep improvement |
Countries
Belgium
Outcome results
None listed