Initiating Pancreatic Enzyme Replacement Therapy (PERT) — PERT Dosing Recommendations — Clinical Pathway: All Settings

Clinical Pathway for Pancreatic Enzyme Replacement Therapy (PERT) in Children with or at Risk for Exocrine Pancreatic Insufficiency (EPI)

PERT Dosing Recommendations

Dosing Guidance for Oral, Bolus, and Continuous Tube Feeds

Consider consult with Nutrition or PERT expert when dosing enzymes to determine the best method for use and for dose titration recommendations. PERT Formulations

PERT for Oral Feeds – Use Enteric Coated Formulation

Child's Age Age-based Dosing Recommendations Focused Guidance About Administration Titration
Premature and
Full-term Infants ≤ 12 mos
  • Initiate When Taking > 60 mL per Feed (Formula/Breast Milk)
  • Starting dose: 3000 lipase units/feed
  • Range: 1000-2500 lipase units/kg/feed
  • Max: 10,000 lipase units/kg/day
  • Open capsule, sprinkle enzyme beads on a small amount of applesauce
  • Administer at the start of feed
  • Give by mouth even if portion of feed is enteral
  • Never give enteric coated beads via feeding tube, will clog tube
  • Check infant’s mouth for retained beads and mucosal irritation
  • Increase by 1 capsule per dose based on clinical symptoms of malabsorption and/or poor weight gain
  • Max dose may transiently exceed 10,000 lipase units per kg/day due to frequency of infant feedings
Children and Adolescents
  • Starting dose
  • 1-4 yrs
    1000 lipase units/kg/meal
    Titrate to max 2500 lipase units/kg/meal
  • ≥ 4 yrs
    500 lipase units/kg/meal
    Titrate to max 2500 lipase units/kg/meal
  • Range: 500-2500 lipase units/kg/meal
  • Max: 10,000 lipase units/kg/day
  • Snack dose: Half of the meal dose
  • Give capsule by mouth
    Give by mouth even if portion of feed is enteral
     
  • If unable to swallow capsule: open capsule, sprinkle enzyme beads on a small amount of applesauce
     
  • For meals lasting longer than 30 mins, split dose and administer half way through meal
  • Increase by 1 capsule per dose based on clinical symptoms of malabsorption and/or poor weight gain
     
  • Consult CF RD or other PERT expert and dose as needed

PERT for Tube Feeding – Bolus Feeds

Enteric Coated Enzyme
  • Weight Based
    Starting dose
  • Children: 500-1000 lipase units/kg/feed
  • Range: 500-2500 lipase units/kg/feed
  • Max: 10,000 lipase units/kg/day
  • Use Only if Child is Able to Take Enzymes by Mouth, Give at Start of Feed
  • Give at start of feed
  • Start at lower end of dosing range
  • Initiate when taking ≥ 60 mL per feed
  • Never crush or chew enteric coated beads
  • Fat Gram Dosing Based
  • Typical dose: 1800-2200 lipase units/g of fat
  • Range: 500-4000 lipase units/g of fat
  • Dose enzymes based on total grams of fat in the formula per
    RD recommendations
  • Non-CF: start at lower end of dosing range
Non-enteric Coated Enzyme (Viokace)
  • Fat Gram Dosing Based
  • Typical dose: 1800-2200 lipase units/g of fat
  • Range: 500-4000 lipase units/g of fat
  • Use Only if Child is Unable to Take Enteric Coated Enzymes by Mouth
  • Crush Viokace and add to formula; Viokace Patient Instructions
  • Lipase units that come from Viokace (non-enteric coated enzyme) do not count towards total max dose per day of 10,000 units lipase/kg/day
  • Round to the nearest 1/2 tablet of Viokace; dosage options are either 10,440 or 20,880 lipase units per tablet
  • Non-CF: start at lower end of dosing range
RELiZORB
  • Child is ≥ 2 yrs of Age
  • Starting Dose: 1 cartridge per 500 mL of formula
  • Max: 6 cartridges/24 hr period
  • Optimal rate of feed: 24-120 mL/hr
  • 10-400 mL/hr (when using single cartridge)
  • 24-150 mL/hr (when using 2 cartridges with a tandem configuration)
  • Avoid fiber-containing and blenderized formulas as they can clog cartridge
  • Refer to RELiZORB for compatible formulas and feeding pumps
  • Minimum tube feeding rate is 10 mL/hr
  • Consult RD if rate < 10 mL/hr
  • Consult RD if child < 2 yrs of age or formula is not on list of compatible formulas
  • RELiZORB (Immobilized Lipase) Cartridge Patient Instructions

PERT for Tube Feeding - Continuous/Overnight Feeds

RELiZORB
  • Child is ≥ 2 yrs of Age
  • Starting Dose: 1 cartridge per 500 mL of formula
  • Max: 6 cartridges/24 hr period
  • Optimal rate of feed: 24-120 mL/hr
  • 10-400 mL/hr (when using single cartridge)
  • 24-150 mL/hr (when using 2 cartridges with a tandem configuration)
  • Preferred Method for Continuous Tube Feeds
  • Avoid fiber-containing and blenderized formulas as they can clog cartridge
  • Refer to RELiZORB for compatible formulas and feeding pumps
  • Minimum tube feeding rate is 10 mL/hr
  • Consult RD if rate < 10 mL/hr
  • Consult RD if child < 2 yrs of age or formula is not on list of compatible formulas
Non-enteric Coated Enzyme (Viokace)
  • Grams of Fat Based
  • Starting dose: 1800-2200 lipase units/g of fat
  • Range: 500-4000 lipase units/g of fat
  • If unable to use RELiZORB, this method provides the next
    best option
  • Lipase units that come from Viokace (non-enteric coated enzyme) do not count towards total max dose per day of 10,000 units lipase/kg/day
  • Always round to the nearest 1/2 tablet of Viokace
  • Dosing options for Viokace are either 10,440 or 20,880 lipase units per tablet
  • Non-CF: start at lower end of dosing range
Enteric Coated Enzyme
  • Given Orally Only
    Dose based on weight or grams of fat in enteral formula
  • Weight Based
    Range: 500-2500 lipase units/kg/feed
    Max: 10,000 lipase units/kg/day
  • Fat Gram Based
    Typical dose: 1800-2200 lipase units/g of fat
    Range: 500-4000 lipase units/g of fat
  • This option is for children > 2 yrs on overnight feeds who can take oral enzymes when RELiZORB or Viokace are unavailable. Use a meal dose of enzymes at start of tube feeds. Children may need an additional half dose at the end of
    the feed.
  • Do not use for 24 hr continuous feeds
  • Initiate when receiving ≥ 15 mL per hr (formula/breast milk)
  • Do not recommend enteric coated enzyme beads through any enteral tube due to risk for clogging
  • Non-CF: start at lower end of dosing range