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Pediatric Pulmonology: The Remaining Barriers to Normalcy in Cystic Fibrosis

The Remaining Barriers to Normalcy in Cystic Fibrosis

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Improving growth in infants with CF

By Megan E. Gabel MD; Rachel E. Gaudio MD; Ala K. Shaikhkhalil MD

Cystic fibrosis (CF) results in chronic pulmonary infections, inflammation, pancreatic insufficiency, and multiple gastrointestinal manifestations. Malnutrition and poor growth are hallmarks of CF, and strongly associated with poor outcomes. Through newborn screening, many infants can be diagnosed within a few days of life, which allows for early initiation of nutritional counseling and close clinical follow-up. Obstacles to growth for infants with CF start in utero, as newborns with CF can have a lower birth weight than the general population. Improving infant growth has been linked to improved clinical outcomes and survival. It remains a top priority and challenge for caregivers and healthcare teams. An interdisciplinary approach, including registered dietitian and social work support, is essential to optimize health for infants with CF. Remaining barriers to normalcy include deficits in linear growth, lack of accurate nutrition biomarkers, persistence of inequities related to social determinant of health, particularly in the global CF community.

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Journal Issue: Remaining Barriers to Normalcy in Cystic Fibrosis: Advances in Gastroenterology, Hepatology, and Nutrition

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