Early intervention in CF: How to monitor the effect

Early and aggressive therapy already at the stage when no apparent signs of significant lung disease are detectable, may delay the development and progression of cystic fibrosis (CF). Identification of markers for early pulmonary disease in CF is crucial to monitor adherence to preventive therapy and determine its success. Currently several surrogate markers are available that are used in both the decision making and evaluation of the timing and success of early intervention namely, pulmonary function tests (PFT), microbial cultures, imaging techniques, inflammatory markers, serological markers, and several general signs such as exacerbation rate and nutritional status. This review will present the current status and discuss the significance of their application as well as their limitations for patients with CF and no apparent pulmonary disease.