Effect of Non-Invasive Ventilation in Cystic Fibrosis Patient With Chronic Respiratory Failure.

Study Overview
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Brief Summary
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There is no randomised controlled trials to determine the clinical effects of long term Non-Invasive Ventilation in Cystic Fibrosis patients.

Detailed Description
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The effects of long term mechanical ventilation for the treatment of respiratory failure in patients with neuromuscular diseases is well established. Long term non-invasive ventilation is commonly used in the treatment of Cystic Fibrosis patients with respiratory failure. However, the benefits of this treatment have never been studied in randomized control trials and are unknown. The goal of this multicenter study is to evaluate the effects of the addition of non-invasive ventilation to the standard therapy in stable Cystic Fibrosis patients with chronic hypercapnia. Two groups of patients will be followed: The first group will receive standard treatment for advanced Cystic Fibrosis according to international guidelines, the second group will receive the same treatment plus non-invasive ventilation (4H/day). The follow up time will be one year for each patient.

Official Title
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Evaluation of the Effects of Long Term Non-Invasive Ventilation in Patient With Cystic Fibrosis and Chronic Hypercapnic Respiratory Failure.

Conditions
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Cystic Fibrosis, Chronic Respiratory Failure

Intervention / Treatment
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* Device: pressure support ventilator


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Confirmed diagnosis of Cystic fibrosis Chronic hypercapnia on room air in stable condition (PaCO2 between 45 and 55mmHg in two occasions separated by more than 4 weeks) Informed consent Exclusion Criteria: Current treatment with non-invasive ventilation PaCO2 more than 55 mmHg Recent pneumothorax (within past 3 months)

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 |
| --- | --- |
| No Intervention: Usual medical therapy<br>Usual CF care | |
| Experimental: Non-invasive ventilation<br>Pressure support ventilator (SAIME,AIROX) | Device: pressure support ventilator<br>* Non invasive ventilation 4h/d 5 days a week<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| The effect of non-invasive ventilation on room air PaCO2 at one year: Hypothesis is PaCO2 stabilisation in the non-invasive ventilation group compared to a increase in PaCO2 in the control group (standard treatment) |  | ended 2011 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Effects of non-invasive ventilation on dyspnea index, functional status (6 minutes walking test, spirometric values), pulmonary exacerbations and body mass index (BMI) compared to standard treatment. |  | ended 2011 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Hypercapnia, Non-Invasive Ventilation