Improving the Care of Infants Identified through Cystic Fibrosis Newborn Screening / The Journal of Pediatrics

On behalf of the Cystic Fibrosis Foundation, we are pleased to share the enclosed clinical practice guidelines for the care of infants identified through cystic fibrosis (CF) newborn screening (NBS). The widespread implementation of NBS for CF throughout the USA presents an incredible opportunity to improve health outcomes for this patient population. The evidence documenting the magnitude of the opportunity is growing.1,2Weare entering a new
era in which the devastating symptomatic presentations of CF such as failure to thrive will become a thing of the past.


Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis / The Journal of Pediatrics

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.


Cystic Fibrosis Pulmonary Guidelines: Treatment of Pulmonary Exacerbations


The natural history of cystic fibrosis lung disease is one of chronic progression with intermittent episodes of acute worsening of symptoms, frequently called acute pulmonary exacerbations. These exacerbations typically warrant medical intervention. It is important that appropriate therapies are recommended on the basis of available evidence of efficacy and safety. The Cystic Fibrosis Foundation therefore established a committee to define the key questions related to pulmonary exacerbations, review the clinical evidence using an evidence-based methodology, and provide recommendations to clinicians. It is hoped that these guidelines will be helpful to clinicians in the treatment of individuals with cystic fibrosis.

Key words: aminoglycosides, IV antibiotics, drug synergism, Pseudomonas, respiratory therapy