What is the recommended dose of Creon (pancrelipase) for an infant with cystic fibrosis?

Creon Dosing for Infants with Cystic Fibrosis

For infants with cystic fibrosis up to 12 months of age, start Creon at 2,000-4,000 units of lipase per 120 mL of formula or estimated breast milk intake, and approximately 2,000 units of lipase per gram of dietary fat in solid foods. 1

Initial Dosing Strategy

• Begin pancreatic enzyme replacement therapy (PERT) immediately upon diagnosis of pancreatic insufficiency, which occurs in 85-90% of CF patients and often evolves during the first year of life 1
• The recommended starting dose is 2,000-4,000 units of lipase per 120 mL of formula or breast milk for liquid feeds 1
• For solid foods introduced at the appropriate age, use approximately 2,000 units of lipase per gram of dietary fat 1
• Never exceed 10,000 units of lipase/kg per day as the maximum dose to avoid fibrosing colonopathy 1, 2

Administration Technique

• Open the capsule and mix the enteric-coated microspheres with a small amount of expressed breast milk or formula on a spoon 1
• If the infant refuses microspheres from a spoon, administration with an acidic puree (e.g., applesauce) may be successful 1
• Never add pancreatic enzymes directly to the infant's feed bottle as this can inactivate the enzymes 1
• If microspheres are still refused, unprotected powder enzymes may temporarily be considered with addition of a proton pump inhibitor to prevent gastric acid destruction 1

Monitoring and Dose Titration

• Monitor growth and nutritional status at every clinic visit for infants to determine adequacy of PERT 1
• Titrate the dose upward as needed based on clinical response, including stool consistency, frequency, and weight gain 1
• Research demonstrates that 500 units of lipase/kg/meal resulted in a coefficient of fat absorption of approximately 89% in infants 6-30 months old, and higher doses did not significantly improve absorption 3
• Enteric-coated microspheres are more effective than enteric-coated tablets and are preferred by parents 1

Critical Safety Considerations

• Avoid doses exceeding 10,000 units of lipase/kg per day due to the strong association with fibrosing colonopathy in young children 1, 2
• The risk of fibrosing colonopathy increases dramatically with doses above 24,000 units/kg/day, with a relative risk of 199.5 for doses exceeding 50,000 units/kg/day 2
• Smaller enzyme preparations specifically designed for infants (such as Creon for Children with 5,000 lipase units per scoop) improve ease of dosing and are preferred by caregivers 4

Common Pitfalls to Avoid

• Do not delay PERT initiation even in breastfed infants, as up to 80% of dietary fat may be excreted without enzyme supplementation 1, 4
• Do not use high-energy or hydrolyzed formulas routinely unless there is poor growth or malabsorption unrelated to CF 1
• Do not assume adequate dosing without monitoring—some infants may have suboptimal fat absorption despite enzyme therapy and require dose adjustment 4, 3
• If gastrointestinal symptoms persist despite appropriate PERT, consider adding a proton pump inhibitor to improve enzyme effectiveness rather than simply increasing the enzyme dose 1