Guidelines for Management of Infants with CF
Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis Drucy Borowitz, MD, Karen A. Robinson, PhD, Margaret Rosenfeld, MD, MPH, Stephanie D. Davis, MD, Kathryn A. Sabadosa, MPH, Stephanie L. Spear, PhD, Suzanne H. Michel, MPH, RD, LDN, Richard B. Parad, MD, MPH, Terry B. White, PhD, Philip M. Farrell, MD, PhD, Bruce C. Marshall, MD, and Frank J. Accurso, MD 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 in- dividual. 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 ev- idence 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. (J Pediatr 2009;155:S73-93). ymptomatic diagnosis of cystic fibrosis (CF) is associated with short- and long-term complications including failure to thrive, stunting, wasting, vitamin and mineral deficiencies, recurrent pulmonary infections associated with decreased Slung function, and recurrent hospitalizations. Early identification of CF by newborn screening (NBS), prenatal diagnosis, or family history offers the opportunity to delay and potentially prevent many of these complications through early treatment. Although many treatment issues are the same for individuals with CF regardless of when they are diagnosed, some are unique to the population of newborn infants who may not have overt symptoms of the disease before referral to the CF care center.
[Show full text]