Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening

Abstract

Background: Successful implementation of newborn screening (NBS) for cystic fibrosis (CF) depends on robust protocols, good communication and appropriate management of recognised infants. In response to current varied practice, the ECFS Neonatal Screening Working Group developed a consensus on the early management of these infants using the Delphi methodology.

Methods: Following detailed literature review, statements were generated by a core group of experts and then assessed by a larger group using modified Delphi methodology.

Results: Forty-one statements were written by the core group. Eighty-six CF specialists contributed to the modified Delphi process. During three rounds, extra statements were added and consensus achieved on 44 (one statement did not achieve consensus).

Conclusions: These statements will provide a framework for the management of screened infants in the first year of life. This process highlights the paucity of evidence on which to base management of these infants. To improve this situation, it is important that each infant with CF identified through NBS has opportunity to be included in a randomised controlled trial.