Supplement
Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis

https://doi.org/10.1016/j.jpeds.2009.09.001Get rights and content

Newborn screening for cystic fibrosis (CF) offers the opportunity for early medical and nutritional intervention that can lead to improved outcomes. Management of the asymptomatic infant diagnosed with CF through newborn screening, prenatal diagnosis, or sibling screening is different from treatment of the symptomatically diagnosed individual. The focus of management is on maintaining health by preventing nutritional and respiratory complications. The CF Foundation convened a committee to develop recommendations based on a systematic review of the evidence and expert opinion. These guidelines encompass monitoring and treatment recommendations for infants diagnosed with CF and are intended to help guide families, primary care providers, and specialty care centers in the care of infants with CF.

Section snippets

Methods

The CF Foundation convened a group of experts to identify issues in the care of infants with CF and commissioned an evidence review from Johns Hopkins University. Details of this evidence review, including methods and results, are provided in the accompanying article.1 Committee members assessed this evidence in developing recommendation statements and, where possible, made evidence-based recommendations. Recommendations were graded using the United States Preventive Health Services Task Force

Conclusion

Newborn screening offers the opportunity to significantly improve the health of individuals with CF, enabling pre-symptomatic monitoring and treatment when needed to prevent or delay nutritional and pulmonary decline. Although infants with CF may appear well, subtle changes occur early in life. Rigorous and careful monitoring and timely medical intervention will permit the best possible outcome for individuals diagnosed with CF in infancy.

Author Disclosures

Drucy Borowitz, MD, has served as a consultant for Solvay Pharmaceuticals. Stephanie D. Davis, MD, has served as a consultant for Inspire Pharmaceuticals. Frank J. Accurso, MD, has received funding from Digestive Care, Inc., Axcan Pharma, and Yasoo Health, Inc. The following authors declare no financial arrangement or affiliation with a corporate organization or a manufacturer of a product discussed in this supplement: Philip M. Farrell, MD, PhD, Bruce C. Marshall, MD, Richard B. Parad, MD,

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