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

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

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

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.

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)