Nutritional Therapy, Pancreatic Exocrine Insufficiency, and Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis

Cystic fibrosis (CF) results from poorly functional, nonfunctional, or absent cystic fibrosis transmembrane regulator protein. Early diagnosis and dietary intervention, particularly increasing fat and calorie intake, improves growth potential and clinical outcomes; early CF nutritional support is associated with a reduction in the rate of pulmonary function decline later in life. In patients with CF, pancreatic exocrine insufficiency (PEI) is often assumed based on genetic testing at diagnosis and confirmed with fecal elastase-1 measurement. One of the therapeutic mainstays for most patients with CF is targeted to reducing fat malabsorption with pancreatic enzyme replacement therapy (PERT). No generic products on the US market are recommended for the treatment of PEI in CF or any other pancreatic diseases. In the United States, PERT dosing is individualized for each patient with CF and PEI based on the CF Foundation guidelines for enzyme starting doses and range of daily doses per day.