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About 90% of people with CF will also develop exocrine pancreatic insufficiency. EPI is a condition in which the pancreas can't digest food properly. The pancreas is an organ that plays a very important role in digestion. When the pancreas can't do its job—break down fats, proteins, and carbohydrates (carbs)—the body can't absorb the nutrients it needs for healthy growth and development. As a result, when the food isn't broken down into small enough molecules, it can stay in the small bowel, causing frequent diarrhea. This is one of the main symptoms of EPI. Other symptoms of EPI include1-4:

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The pancreas is about 6 inches long and shaped like a flat pear. It is surrounded by the stomach, small intestine, liver, spleen, and gallbladder.5

The information in this section is intended for informational purposes only and is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your qualified healthcare provider with any questions you may have regarding a medical condition.

How many people with CF also develop EPI?2

About 90% of people with CF also have EPI. Here is the breakdown:
  • Almost 50% of infants with CF have EPI at birth
  • Another 25% of infants with CF develop EPI within 6 months of being born
  • An additional 15% of people with CF develop EPI in late childhood or early adulthood

Testing and diagnosis6

EPI can be tough to test for and diagnose since it is linked with many other conditions, the most common being CF and chronic pancreatitis. The following tests are commonly used to help diagnose EPI:
  • Stool tests
  • Blood tests
  • Endoscopic retrograde cholangiopancreatography (ERCP). This test uses a flexible tube that goes through your mouth and into your stomach to take pictures of the pancreas to look for damage

Treatment for EPI2,4

EPI is most often treated with pancreatic enzyme replacement therapy (PERT). Pancreatic enzymes are needed to help properly digest proteins, fats, and carbs. In addition, pancreatic enzymes help people with EPI gain weight and absorb nutrients. For children and adults, pancreatic enzymes should be taken during each meal or snack, while infants should receive pancreatic enzymes prior to feeding. All pancreatic enzymes should be taken at a dose that is recommended by your CF care team.
References: 1. Managing EPI and cystic fibrosis. Everyday Health website. http://www.everydayhealth.com/hs/exocrine-pancreatic-insufficiency/managing-epi-cystic-fibrosis/. Accessed May 1, 2016. 2. Pancreatic exocrine insufficiency. Part 1 of 2: pathogenic and diagnostic considerations. Gastroenterology & Endoscopy News website. http://www.gastroendonews.com/download/Pancre_CaPart1GENSE12_WM.pdf. Accessed May 1, 2016. 3. What is exocrine pancreatic insufficiency? Everyday Health website. http://www.everydayhealth.com/health-report/exocrine-pancreatic-insufficiency/what-is-epi.aspx. Accessed May 1, 2016. 4. Nutrition: pancreatic enzyme replacement in people with cystic fibrosis. Cystic Fibrosis Foundation website. https://www.cff.org/PDF-Archive/Pancreatic-Enzyme-Replacement/. Accessed May 1, 2016. 5. What is the pancreas? Pancreatic Cancer Action Network website. http://www.pancan.org/section-facing-pancreatic-cancer/learn-about-pan-cancer/what-is-the-pancreas/. Accessed May 1, 2016. 6. Diagnosing exocrine pancreatic insufficiency. Everyday Health website. http://www.everydayhealth.com/health-report/exocrine-pancreatic-insufficiency/epi-diagnosis.aspx. Accessed May 1, 2016.

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