Long Term Significance (Survival) of LCI in Patients With Cystic Fibrosis

Does Lung Clearance Index (LCI) Predict the Future Lung Disease Course in Patients With Cystic Fibrosis

The current dream in CF research is to discover safe drugs that correct the basic defect and prevent lung disease, allowing patients without significant lung damage to live nearly normal lives with a dramatic increase in life expectancy and without the burden of current treatment. The compound VX-770 (Ivacaftor Ò) is hoped to be the first milestone along this way. Progression of lung disease is now so gradual in many centres that sensitive indicators of early lung disease (small airways disease) are critically needed to assess the effects of such new treatments. In this context, assessment of ventilation inhomogeneity by the measurement called Lung clearance index (LCI) seems to be the most promising tool. However, to get approval by health authorities, new measures used in drug evaluation need to fulfill strict criteria. For LCI, the investigators still need to prove its long term significance: How well does the LCI measurement predict the long term lung disease course? Therefore, in this study the investigators want to measure LCI at baseline in a large patient cohort and establish how well it predicts the patients' disease course over the next 2 years.

Study Overview

• Status: Withdrawn
• Conditions: Cystic Fibrosis (CF)
• Intervention / Treatment: Device: EasyOne Pro and Ecomedics

Detailed Description

Patients with CF ( 6 years and older and FEV1 above 40% predicted) will be invited to participate in this prospective non interventional study in 2 CF centers ( UCL and UZ Leuven). Jointly we take care of more than 40 % of the Belgian CF population. Median FEV1 of children (6 - <18 y, n >120) from these CTN centers is high ( around 100% pred), defining a study group of great interest in the current context.

To assess the predictive value of LCI over 2 years, the time to next pulmonary exacerbation will be evaluated in the entire cohort. To assess the predictive value of LCI over 2 years, the time to next pulmonary exacerbation will be evaluated in the entire cohort.

Repeat LCI measurements will be done at subsequent patient visits.

2 devices measuring the LCI will be tested and compared : Ecomedics and NDD.

LCI and spirometry will be measured in 120 healthy Belgian children and young adults. These subjects must be free of respiratory symptoms for at least two weeks and will not have any chronic or recurrent chest problem.

• Study Type: Observational

Contacts and Locations

Study Locations

• Belgium
  • Bruxelles
    • Woluwé-Saint-Lambert, Bruxelles, Belgium, 1200
      • Cliniques Universitaires Saint-Luc (UCL)

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 65 years (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients with Cystic Fibrosis ( 6 years and older and FEV1 above 40% predicted) will be invited to participate in this prospective non interventional study in 2 CF centers (UCL and UZ Leuven)

Description

Inclusion Criteria:

• FEV1(% predicted) > 40%

Exclusion Criteria:

• FEV1(% predicted) < 40%
• Exacerbation during baseline measurement

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Cystic fibrosis (Ecomedics vs NDD); No treatment, observational	Device: EasyOne Pro and Ecomedics; LCI will be assessed with 2 different devices : EasyOne Pro and Ecomedics
Healthy (Ecomedics vs NDD); Free of respiratory symptoms for at least two weeks and will not have any chronic or recurrent chest problem. No treatment, observational	Device: EasyOne Pro and Ecomedics; LCI will be assessed with 2 different devices : EasyOne Pro and Ecomedics

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Lung Clearance Index (Predictive value and change of)	Evaluation of LCI is repeated at 1 week and 3 months to assess short and intermediate repeatability. LCI and spirometry will be repeated after 22-24 months after the baseline measurement. This will allow to evaluate tracking of LCI as well as FEV1 % predicted in the total patient cohort as well as in the patients with FEV1 above 80% predicted.	Baseline, 1 week, 3 months and 2 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
FEV1 (Predictive value and change of)	LCI and spirometry will be repeated after 22-24 months after the baseline measurement. This will allow to evaluate tracking of LCI as well as FEV1 % predicted in the total patient cohort as well as in the patients with FEV1 above 80% predicted.	baseline and 2 years

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
Symptom score	Symptom score is calculated at 1 week and 3 months to ensure a stable disease of the patient to avoid bias during the calculation of LCI repeatability.	1 week, 3 months

Collaborators and Investigators

• Sponsor: University Hospital St Luc, Brussels
• Collaborators: KU Leuven
• Investigators: Study Director: Lebecque Patrick, PhD, Cliniques Universitaires Saint-Luc; Principal Investigator: Poncin William, Cliniques Universitaires Saint-Luc

Study record dates

Study Major Dates

• Study Start: January 1, 2013
• Primary Completion (Anticipated): May 1, 2015
• Study Completion (Anticipated): May 1, 2017

Study Registration Dates

• First Submitted: March 14, 2013
• First Submitted That Met QC Criteria: September 24, 2013
• First Posted (Estimate): September 27, 2013

Study Record Updates

• Last Update Posted (Estimate): January 15, 2015
• Last Update Submitted That Met QC Criteria: January 14, 2015
• Last Verified: November 1, 2013

More Information

Terms related to this study

• Keywords: Lung clearance index (LCI)
• Additional Relevant MeSH Terms: Digestive System Diseases; Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Infant, Newborn, Diseases; Genetic Diseases, Inborn; Pancreatic Diseases; Fibrosis; Cystic Fibrosis
• Other Study ID Numbers: LCI-01-St-Luc