A 5-year-old child with cystic fibrosis (CF) is being seen for a follow-up visit. The parents report that the child has difficulty gaining weight despite a high- calorie diet and frequently experiences loose, fatty stools. The healthcare provider prescribes pancreatic enzyme replacement therapy (PERT) to assist with digestion. Which of the following statements best explains why children with CF require digestive enzymes?
Explanation & Rationale
A. CF causes thickened mucus that obstructs the pancreas, preventing the release of digestive enzymes is correct because cystic fibrosis leads to thick, sticky secretions that block pancreatic ducts. This prevents digestive enzymes from reaching the small intestine, resulting in malabsorption of fats, proteins, and fat-soluble vitamins, which causes poor weight gain and steatorrhea (fatty stools). B. Children with CF have an overproduction of digestive enzymes, leading to malabsorption is incorrect because the problem in CF is insufficient delivery of enzymes to the intestine, not overproduction. C. Digestive enzymes are needed to break down fat, which children with CF can digest more efficiently than carbohydrates is incorrect because children with CF have difficulty digesting fats due to lack of pancreatic enzymes. They do not digest fat more efficiently; instead, fat malabsorption is a hallmark of the disease. D. Pancreatic enzyme replacement is only required when children develop diabetes, a common complication of CF is incorrect because PERT is required due to exocrine pancreatic insufficiency, not diabetes. Diabetes in CF results from endocrine pancreatic dysfunction and is unrelated to the need for digestive enzymes.