New Perspectives of Adaptation to NIV in ALS

Study Overview
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Brief Summary
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The purpose of this study is to demonstrate that the adaptation to the Non Invasive Ventilation (NIV) at home is not worse when compared with an adaptation performed in inpatient settings.

Official Title
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New Perspectives of Adaptation to Non-Invasive Ventilation (NIV) in Amyotrophic Lateral Sclerosis (ALS)

Conditions
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Amyotrophic Lateral Sclerosis (ALS)

Intervention / Treatment
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* Other: Adaptation and training to Non Invasive Ventilation (NIV)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: ALS diagnosis according to the criteria of El Escorial; Clinical indication to NIV (nocturnal hypoventilation and respiratory muscle weakness confirmed according to the criteria normally accepted); No respiratory infection within 3 months Severe disability (ALS-FRS <31); Age > 18 years; The voluntary participation to the Study. Exclusion Criteria: -- Previous episodes of pneumothorax; Comorbid cardiac and / or renal important Severe cognitive impairment; Refusal of the patient at the time of Informed Consent.

Ages Eligible for Study
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Minimum Age: 18 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
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Home Group<br>Adaptation and training to Non Invasive Ventilation (NIV) (not less than five sessions), at home | Other: Adaptation and training to Non Invasive Ventilation (NIV)<br>* Each person recruited after a respiratory evaluation (baseline spirometry and supine, maximal inspiratory and expiratory pressures (MIP / MEP) or sniff nasal inspiratory pressure (SNIP), 6 Minute Walking Test (6MWT) -if still ambulatory, arterial blood gas, nocturnal polygraphy) will be adapted to Non Invasive Ventilation (NIV). Moreover, the subject will be brought to the management of bronchial secretions (under the terms set out herein) during not less than five outpatient or house accesses, depending on who is randomly assigned to the experimental group or the control group. These accesses will take place weekly, for a period of two months, considering both the needs and the patient's clinical condition.<br>|
| Active Comparator: Outpatient Group<br>Adaptation and training to Non Invasive Ventilation (NIV) (not less than five sessions), at the outpatient clinic | Other: Adaptation and training to Non Invasive Ventilation (NIV)<br>* Each person recruited after a respiratory evaluation (baseline spirometry and supine, maximal inspiratory and expiratory pressures (MIP / MEP) or sniff nasal inspiratory pressure (SNIP), 6 Minute Walking Test (6MWT) -if still ambulatory, arterial blood gas, nocturnal polygraphy) will be adapted to Non Invasive Ventilation (NIV). Moreover, the subject will be brought to the management of bronchial secretions (under the terms set out herein) during not less than five outpatient or house accesses, depending on who is randomly assigned to the experimental group or the control group. These accesses will take place weekly, for a period of two months, considering both the needs and the patient's clinical condition.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change from baseline in adherence NIV in ALS patients at baseline, 2 and 4 months as measured by monthly means and standard deviations of hours of use of Non Invasive Ventilation (NIV) | The primary outcome the time of adherence to NIV defined as actual use of NIV for not less than 150 hours / month. | Baseline + after 2 and 4 months from the recruitment | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Non Invasive Ventilation (NIV), Amyotrophic Lateral sclerosis (ALS), adaptation, home care