None listed
Conditions
Brief summary
The purpose of the study is to further improve outcomes in CF by developing a better understanding of the reasons for the decline in health status and lung function during adolescence, which is one of the key challenges facing clinicians. We hope to do this by looking at such things as the 1) pathology involved in CF lung disease, 2) long term risks of emerging organisms, 3)potential effects of early therapeutic interventions, such as P. aeruginosa eradication, 4) metagenomic profile identifying bacteria in respiratory samples, 5) psychosocial factors, and 6) relationship between early life events and outcomes between 9 - 17 years of age.
Interventions
Two study visits will occur from 12 months up to 3 years depending on the timing of the initial visit. At each visit clinical data will be recorded including: current symptoms, physical examination, weight, height, concomitant medications, spirometry, induced sputum collection and urine for matrix breakdown products and biomarkers of neutrophilic inflammation. Blood is collected routinely for review tests and will be collected and stored for inflammatory mediators. Health Related Quality of Life questionnaires and other standard measures of psychosocial health are completed and a bone density test (a DEXA), and a low dose chest CT scan will be done at the start and end of the study (again this is depending on the timing of the initial visit).
Sponsors
Eligibility
Inclusion criteria
All children with cystic fibrosis who completed the ACFBAL Study (ACTRN12605000665639) CE Wainwright et al. Effect of Bronchoalveolar lavage-Directed Therapy on Pseudomonas aeruginosa Infection and Structural Lung Injury in Children with Cystic Fibrosis A Randomized Trial. JAMA 13 July 2011; 306(2): 163-171.
Exclusion criteria
Children who did not not complete the ACFBAL study