The Effect of rhDNase on Ventilation Inhomogeneity in Patients With Cystic Fibrosis

• Status: Completed
• Trial end date: 2009-06-01
• Target enrollment: 0
• Participant gender: All

Summary

This study will assess whether rhDNase treatment improves ventilation inhomogeneity as assessed by lung clearance index (LCI) in patients with cystic fibrosis (CF).

• Phase: Phase 4
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: The Hospital for Sick Children

Criteria

Inclusion Criteria:

• Diagnosis of CF as defined by clinical features of CF and a documented sweat chloride > 60 mEq/L by quantitative pilocarpine iontophoresis test or a genotype showing two well characterized disease causing mutations

• Informed consent and verbal assent (as appropriate) has been provided by the subject's parent or legal guardian and the subject

• 6-18 years of age at enrolment

• Able to perform reproducible spirometry

• Clinically stable at enrolment

• Ability to comply with medication use, study visits and study procedures as judged by the site investigator

• FEV1 % predicted > 70 % as calculated by the Wang reference equations

Exclusion Criteria:

• Respiratory culture positive for:

• NTM within past year or AFB positive at screening (sputum only)

• B. cepacia complex within past year or at screening

• Use of intravenous antibiotics or oral quinolones within 14 days of screening

• Investigational drug use within 30 days of screening

• History of alcohol, illicit drug or medication abuse within 1 year of screening

• Other major organ dysfunction excluding pancreatic dysfunction

• History of lung transplantation or currently on lung transplant list

• Physical findings at screening that would compromise the safety of the participant or the quality of the study data