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Non-Invasive Ventilation (NIV) is an established treatment to manage respiratory muscles dysfunction in neuromuscular disease, preventing the progression of respiratory failure to intubation and/or a tracheotomy. NIV is commonly needed at first during the night, but when the disease worsens, it is required during the day. It is provided via nasal or oronasal masks, causing discomfort and/or aesthetic issues that result in poor compliance.
Intermittent Abdominal Pressure Ventilation (IAPV) is a valid, though unconventional, alternative to daytime NIV: it consists of a portable ventilator with an internal battery and a corset as interface. The IAPV corset is lightweight, comfortable and, thanks to velcro fasteners, easier and better fitting than a face mask. Cyclical inflation of a rubber bladder inside the corset moves the diaphragm upwards like a pneumobelt causing air to enter in the lungs via the upper airways as gravity draws the diaphragm back to its resting position.
IAPV is indicated in neuromuscular disease and has already been tested in few preliminary studies and case reports. This study wants to verify the hypothesis of its application in population of neuromuscular patients.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Intermittent Abdominal Pressure Ventilation | Experimental | Patients in experimental group will be adapted to Intermittent Abdominal Pressure Ventilation (IAPV). Abdominal ventilation replaces part of usual ventilation (non invasive ventilation with mouthpiece or nasal-pillow) |
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| Usual ventilation | Active Comparator | Patients in control group continue with usual ventilation (NIV through mouthpiece or nasal-pillow) |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Intermittent Abdominal Pressure Ventilation | Device | Patients of experimental group will use IAPV in daytime ventilation. Intermittent abdominal pressure ventilation (IAPV) is a portable ventilator with an internal battery and PneumoBelt corset as an interface. |
| Measure | Description | Time Frame |
|---|---|---|
| Blood Gas Analysis | Changes of respiratory parameters in terms of normalization of oxyemia (Arterial Pressure of oxygen 80-100 mmHg) and normalization of capnia (Arterial Pressure of carbon dioxide 35-45 mmHg), assessed by blood gas analysis | 12 months |
| Measure | Description | Time Frame |
|---|---|---|
| Therapy adherence | Adherence to IAPV tested with Beliefs about Ventilation Questionnaire (BVQ). BVQ is a qualitative questionnaire: it consist of 25 items, where higher scores mean a better outcome. | 12 months |
| Patients satisfaction to IAPV |
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Inclusion Criteria:
Exclusion Criteria:
- Diagnosis of kyphoscoliosis
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Paolo Banfi, MD | Contact | 00390240308812 | pabanfi@dongnocchi.it | |
| Valeria Volpi, PT | Contact | vvolpi@dongnocchi.it |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| IRCCS S. Maria Nascente - Fondazione Don Carlo Gnocchi | Recruiting | Milan | 20146 | Italy |
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| ID | Term |
|---|---|
| D009468 | Neuromuscular Diseases |
| ID | Term |
|---|---|
| D009422 | Nervous System Diseases |
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| Usual ventilation | Device | NIV |
|
Satisfaction to IAPV tested with Visual Analog Scale (VAS), with a score that ranges between 0 and 10, where a higher score means a worse outcome.
| 12 months |
| Caregivers satisfaction to IAPV | Satisfaction to IAPV tested with Visual Analog Scale (VAS), with a score that ranges between 0 and 10, where a higher score means a worse outcome. | 12 months |
| Amelioration of Quality of life | Improvement of quality of life tested with World Health Organization Quality of Life-Brief (WHOQOL-Brief), with a score ranging from 0 to 100 for each of the four domain and where a higher score means a better outcome | 12 months |