Pediatric Cystic Fibrosis and Fat-Soluble Vitamins

Cystic Fibrosis (CF) is a genetic disorder that affects approximately 70,000 people worldwide. CF is caused by a mutation in the Cystic Fibrosis conductance Transmembrane Regulator (CFTR) gene. Mutations in CFTR result in thick, sticky mucus within the lungs, pancreas, intestines, and liver. Thick secretions within the pancreas result in destruction of pancreatic exocrine cells that are responsible for the production of pancreatic enzymes essential to the breakdown of fat, protein, and carbohydrates into absorbable nutrients. This also affects the absorption of fat-soluble vitamins A, D, E, and K. These vitamins are integral to physiologic processes within the body. Deficiencies result in significant morbidity and mortality. Thus, it is critical that patients with CF are screened at least annually for fat-soluble vitamin deficiencies. Once deficiencies are identified, they should be treated with appropriate supplementation, and treatment should be monitored to ensure adequate levels and prevent toxicity.