ABSTRACT
Improvements in outcomes for those with cystic brosis (CF) that are evident by school-age point to the period of early life as crucial in determining long-term outcomes (Figure 32.1). is is unsurprising given that the early years are a time of rapid lung development. Measurement of lung function is a central part of the clinical assessment of older children and adults with CF. Serial tests provide longitudinal information about the extent of abnormality, progression of disease, and individual response to treatment. As evidence accumulates that chronic pulmonary disease commences in infancy, the need to evaluate lung function early is self-evident. Neutrophil-dominated inammation similar to that seen in older subjects has been identied in the lungs of aected infants1,2 and understanding of how airway pathology evolves during the rst years of life is improving. Recent studies add to knowledge as to how useful lung function testing in the preschool years could be, and also the nature of functional changes in CF. e limiting factors, however, remain that infant lung function is technically dif-cult to perform, expensive, time consuming, and requires sedation, which makes it dicult to recruit healthy subjects as controls. Furthermore, between infancy and school age, toddlers are too old to sedate and too young to cooperate with most testing.
