Exocrine pancreatic insufficiency occurs when there is a deficiency of pancreatic enzymes due to diseases affecting the pancreas or GI tract. It results in maldigestion and malabsorption. Patients with conditions like cystic fibrosis, chronic pancreatitis, or who have undergone pancreatic surgery are prone to EPI. Diagnostic tests like fecal elastase can indicate EPI. Symptoms include poor growth, loose stools, and abdominal issues. Treatment involves replacing missing enzymes with pancreatic enzyme replacement therapy pills taken with meals and snacks. Dosing is based on lipase units and a patient's caloric needs. Acid reducers are often needed to help the enzymes work properly.

1. EXOCRINE PANCREATIC INSUFFICIENCY
ANDTHE ROLE OF
PANCREATIC ENZYMES
Phoebe Gulling
Dietetic Intern 2015

2. Exocrine Pancreatic Insufficiency (EPI)
• Deficiency of exocrine pancreatic enzymes (lipase, amylase, and protease) which
results in maldigestion and malabsorption.
• Occurs in diseases which effect the pancreas or GI system
• Cystic Fibrosis
• Chronic Pancreatitis
• PancreaticCancer
• Whipple Surgery (duodenopancreatectomy)
• Total Pancreatectomy

3. Testing for Pancreatic Insufficiency
• Fecal Elastase: indirect test of exocrine pancreatic function.
• If <200 ug/g  treat w/ enzymes
• C-Mixed Triglyceride BreathTest (C-MTG): breath sample collection to measure maldigestion
2/2 gastroduodenal and pancreatic surgery.
• Indicates lipase activity w/ CO2 excretion
• <29% indicates EPI
• 72-h fecal fat test (FFQ): measures total stool fat content to determine coefficient of fat
retention (%) and degree of steatorrhea (g fat/72h)
• Pt consumes 100 g fat/day for 72-h prior to start of test and throughout collection
• Normal: >95% coefficient of fat retention or 5g fat/24-h
• Abnormal: steatorrhea and occurs w/ low coefficient of fat retention.
• Immuno-reactive Trypsinogen (IRT): measures amt of trypsinogen in blood.
• Increased w/ acute pancreatitis and CF
• Low levels after 8yrs w/ pancreatic insufficiency

4. Indications of Malabsorption
• Poor weight gain or failure to thrive
• Frequent, loose, large stools
• Mucus or oil in stools (steatorrhea)
• Excessive gas or stomach pain
• Abdominal distention or bloating
• Exceptionally foul smelling stool

5. Cystic Fibrosis
• Genetic disease caused by defective gene
• Causes thick build-up of mucus in lungs,
pancreas, and other organs.
• Lungs: clogs airway, traps bacteria,
respiratory failure.
• Pancreas: prevents release of digestive
enzymes which allow breakdown of food
and absorption.

6. CFTreatment plan
• Daily: airway clearance of mucus, Inhalants (nebulizer), pancreatic enzymes.
• Airway clearance by coughing, huffing, percussion (clapping), or vibrations to loosen mucus.
• Inhalants/Mucolytics used to break up mucus or make it thinner.
• Pancreatic enzymes if pancreatic insufficiency (87% of CF pts have EPI)
• PICC or ports for easy medication application (antibiotics)
• CFTR protein to regulate flow of Cl and Na.
• Kalydeco and orkambi approved for CF patients 2 and older w/ certain CF mutations.
• Thins mucus to ease coughing out.
• Lung transplant

7. RDTreatment Plan for CF
• High calorie, high protein, high sodium diet
• AquaADEK if pancreatic insufficient
• May require extra supplementation of fat soluble vitamins, Ca, zinc, and/or iron.
• Check fat-soluble vitamin levels yearly – more often if abnormal.
• Pancreatic enzymes

8. What are Pancreatic Enzymes?
• Healthy pancreas produces enzymes lipase, protease, and amylase to
digest fat, protein, and carbohydrates.
• If pancreatic insufficient, enzymes are not produced.
• Capsules which contain small beads with special enteric coating to allow
absorption in small intestine.
• Taken right before a meal or snack.Work up to 30-60 minutes after taking.
• Higher dose required for high fat meals.
• Labeled and dosed based on amount of Lipase in each capsule.

9. Types of PERT
(Pancreatic Enzyme ReplacementTherapy)
• Pancreaze (pancrelipase)
• 4,200
• 10,500
• 16,800
• 21,000
• Creon
• 3,000
• 6,000
• 12,000
• 24,000
• 36,000
• Ultresa
• 13,800
• 20,700
• 23,000
• Zenpep
• 3,000
• 5,000
• 15,000
• 20,000
• 25,000
• Viokace: no enteric coating. Okay for enteral feeds
• 10,440
• 20,880
• Pertzye
• 8,000
• 16,000

10. Example: Creon 24,000

11. Dosing
• Infant dosing: 2,000-4,000 units lipase per 120mL human milk or formula
• >1 y/o (>14kg): 500-2,500 units lipase per kg per meal
• Begin w/ 1,000 units lipase/kg/meal advance as needed
• >4 y/o and adults (>28kg): 500-2,500 units lipase per kg per meal
• Begin w/ 500 units lipase/kg/meal advance as needed
• Based on fat intake: 500-4,000 units lipase per g fat
• DON’T exceed 10,000 units lipase per kg per day
• Can lead to fibrosing colonopathy

12. Example
• 150 lb (68 kg) CF patient
• Needs ~2600 kcal/day (35-40 kcal/kg)
• ZenPep 20,000
• 5 tablets at each meal (100,000 lipase units)  1,470 units lipase/kg/meal
• 2 tablets at each snack (40,000 lipase units)  588 units lipase/kg/meal
• Total for day: 380,000 units lipase
• 5,588 units lipase/kg/day

13. PERT and tube feedings
• Calculate lipase dose ofTF based on grams fat in mL formula
• UseViokace – no enteric coating, easily crushed and mixed in formula
• CrushViokace tab, dissolve in warm water, then add to 4-h feeding volume for 20-30
minutes prior to feeding. Repeat q4h.
• Take ZenPep or Creon orally -- ½ dose prior to feeding and ½ dose after
tube feeding.
• Dissolve Creon in sodium bicarbonate and flush through tube before tube
feeding.

14. Example: Nutren 1.5
• Viokace 20,880
• 3 tablets in AM and at Noon
• 2,088 units lipase per g fat per feed
• 5 tablets in PM
• 2,320 units lipase per g fat
• Total for day = 229,680 units lipase
• 3,377 units lipase per kg per day
• 150 lb (68 kg) CF pt.
• Needs ~2600 kcal/d (35-40 kcal/kg/day)
• 7 cans Nutren 1.5 per day
• AM = 2 cans (30 g fat)
• Noon = 2 cans (30 g fat)
• PM = 3 cans (45 g fat)

15. Suggestions for Chronic
Pancreatitis,Whipple, or GI surgery

16. Need for Acid Reducers
• Proton Pump Inhibitor (Prilosec, Prevacid, Nexium)
• H2-Receptor Site Blocker (Pepcid, Zantac)
• Decreased bicarbonate secretion from pancreas.
• Acidic intestinal environment
• Ineffective dissolution of enzyme’s enteric coating.
• Subsequent maldigestion & malabsorption.
• PPI and H2 Blocker reduce acid and allow proper release of enzymes in duodenum

17. References
• Nutrition Care Manual
• Grande L. Pancreatic Enzyme Products:What’s New. ICAN: Infant, Child, &
Adolescent Nutrition. 2011; 3(2): 87-91.
• Cystic Fibrosis Foundation (cff.org)
• Dominguez-Munoz JE, Iglesias-Garcia J, Iglesias-Rey M,Vilarino-Insua M.
Optimising the therapy of exocrine pancreatic insufficiency by the association of a
proton pump inhibitor to enteric coated pancreatic extracts. PMCID:PMC 1856339.
2006; 55(7): 1056-1057.
• Dominguez-Munoz JE. Pancreatic Enzyme ReplacementTherapy: Exocrine
Pancreatic Insufficiency after Gastrointestinal Surgery. International Hepato-
Pancreato-Biliary Association. 2009; 11(3): 3-6.

18. Pictures
• Slide 2: http://animatedpancreaspatient.com/en/exocrine-pancreatic-insufficiency-epi-
slide-show.phtml
• Slide 5: http://www.nhlbi.nih.gov/health/health-topics/topics/cf/signs
• Slide 8: http://royaldrugstore1.beep.com/
• Slide 10: http://www.iodine.com/drug/creon/fda-package-insert
• Slide 12: https://pancreaticcanceraction.org/about-pancreatic-cancer/diet-and-
nutrition/pancreatic-enzyme-replacement-therapy-pert/
• Slide 14: https://www.nestlehealthscience.us/brands/nutren/nutren-1-5
• Slide 15: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2798169/
• Slide 18: http://cysticfibrosismbm.blogspot.com/

19. QUESTIONS???