Clinical trial NCT00208078: Effect of Non-Invasive Ventilation in Cystic Fibrosis Patient With Chronic Respiratory Failure.
Official title: Evaluation of the Effects of Long Term Non-Invasive Ventilation in Patient With Cystic Fibrosis and Chronic Hypercapnic Respiratory Failure.
Study type: INTERVENTIONAL
Overall status: TERMINATED
Phase: PHASE4
Conditions: Cystic Fibrosis
Chronic Respiratory Failure
Keywords: Hypercapnia
Non-Invasive Ventilation
Lead sponsor: Cochin Hospital, India
Sponsor class: OTHER
First posted: 2005-09-21
Enrollment: 36
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: RANDOMIZED
Intervention model: PARALLEL
Primary purpose: TREATMENT

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

Detailed description: 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.

Eligibility criteria: 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)

Primary outcomes:
measure: 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)
timeFrame: ended 2011
Secondary outcomes:
measure: 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.
timeFrame: ended 2011
Other outcomes:
Locations:
city: Paris
country: France
facility: Department of pulmonary disease of Cochin Hospital (AP-HP)
Study officials:
affiliation: Department of pulmonary disease of Cochin Hospital (AP-HP)
name: Pierre-Régis Burgel
role: PRINCIPAL_INVESTIGATOR
References:
citation: Ellaffi M, Vinsonneau C, Coste J, Hubert D, Burgel PR, Dhainaut JF, Dusser D. One-year outcome after severe pulmonary exacerbation in adults with cystic fibrosis. Am J Respir Crit Care Med. 2005 Jan 15;171(2):158-64. doi: 10.1164/rccm.200405-667OC. Epub 2004 Oct 22.
citation: Moran F, Bradley J. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. 2003;(2):CD002769. doi: 10.1002/14651858.CD002769.