EVALUATION OF PULMONARY FUNCTION TESTS FOR OUTCOME MEASURES IN INFANT WITH CF

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Recent studies have demonstrated that CF lung disease begins far earlier than previously understood, frequently in infancy and prior to the onset of respiratory symptoms. To date, research in this age range has been hindered by the lack of sensitive and reproducible measures of lung disease. Spirometry requires the voluntary generation of a maximal inspiratory effort followed by a rapid and maximal forced expiration. Because infants cannot perform such a maneuver, measurements in the first several years of life have been restricted to those obtained in the tidal volume (normal breathing) range, where they are highly variable and poorly reproducible. Recently, a new technique has been developed for measuring forced expiratory flows from raised lung volumes and fractional lung volumes in sedated infants, for the first time producing results comparable to those obtained by older subjects. These new techniques have the potential to dramatically improve the understanding of early CF lung disease, and to serve as reproducible outcome measures for clinical trials. Although these tests have been successfully employed in single-center studies utilizing investigator-fabricated devices, they have never been employed in a multi-center setting. The purpose of this study is to validate the Infant Pulmonary Lab device, manufactured by Collins, Inc., for use in future clinical trials and to determine whether infant pulmonary function testing with the IPL device is safe and well tolerated in infants with CF.