Cystic Fibrosis (CF) is a life-limiting condition characterized by chronic, progressive pulmonary disease in childhood. Understanding longitudinal changes in infectious and functional measures of disease is necessary to better understand the pathophysiology of disease and determine the utility of novel measures in defining and managing CF lung disease. This year, we formally removed MRI testing from our protocol due to changes in availability of the hardware and software needed for testing. Our modified study protocol has received IRB approval, and recruitment/enrollment of subjects has begun.
In thirty children 3-20 years old with mild CF lung disease,
- Correlate longitudinal change in sputum microbial diversity and density, ventilation inhomogeneity (MBW) and lung function (MBW, spirometry) over a 12-month period in children aged 3-20 with mild to moderate CF lung disease (FEV1 ≥ 60%).
- Investigate differences in the microbial communities and lung function characteristics of subjects who do and do not experience pulmonary exacerbation during the study period.
- Determine the impact of antibiotic therapy on the stability and organism profile of the respiratory microbiota.
Impact on child health:
Better understanding the mechanisms and markers of pulmonary disease in CF may improve disease management; the combination of testing proposed may prove more sensitive than traditional measures for detecting small changes in lung diseease.
Kristine Wylie, Ph.D.