What is Exocrine Pancreatic Insufficiency (EPI)?

Exocrine Pancreatic Insufficiency (EPI) occurs when the pancreas does not secrete enough digestive enzymes and is common in people with chronic pancreatitis. This is because as the pancreas gets scarred, the part of the pancreas that generates enzymes is destroyed. As a result, the pancreas is unable to produce these enzymes that are essential to digest food. If you suffer from EPI, your body has difficulties breaking down food.

Leading causes of EPI:

  • Chronic pancreatitis: 15-30% prevalence
  • Pancreatic restriction/older age: 15-40% prevalence
  • Cystic fibrosis: 80-90% of CF patients
  • All infants up to 6 months of age!

Listen to Dr. Steve Freedman of Harvard Medical School explain:

  • What exocrine pancreatic insufficiency (EPI) is
  • Who’s at risk of developing EPI
  • Symptoms of EPI
  • Treating EPI

What happens if you have Exocrine Pancreatic Insufficiency or EPI?

EPI causes 4 main problems:

  1. Carbohydrates are not properly absorbed
  2. Proteins are not properly absorbed
  3. Fats are not properly absorbed
  4. This can lead to malnutrition and weight loss

Signs and symptoms of Exocrine Pancreatic Insufficiency

  • Steatorrhea: Pale oily stools and “sticking” phenomenon.
  • Abdominal Symptoms: Like diarrhea, bloating, abdominal pain, and gas.
  • Fat-soluble vitamin deficiency (A, D, E, K):
    • Rashes and night blindness → Vitamin A deficiency.
    • Metabolic bone disease → Vitamin D deficiency.
    • Hemolytic anemia, neuropathy, retinopathy → Vitamin E deficiency.
    • Bruising/bleeding →Vitamin K deficiency.

How do doctors diagnose Exocrine Pancreatic Insufficiency or EPI?

Doctors usually diagnose EPI using a fecal elastase test. Elastase is an enzyme made by the pancreas that helps break down essential nutrients like fats, proteins, and carbohydrates after you eat. If little or no elastase is found in the stool, it might mean the enzyme is not working properly. In this case, your doctor might diagnose you with EPI. In adults, pancreatic insufficiency is often a sign of chronic pancreatitis.

Sometimes a doctor can prescribe Pancreatic Enzyme Replacement Therapy (PERT) and see if you respond to them. If you do, that can help them diagnose you.

Listen to Dr. Steve Freedman of Harvard Medical School explain:

  • What tests are used to diagnose EPI
  • What EPI diagnostic tests involve
  • Requirements for EPI diagnostic tests
  • And more

What are pancreatic enzymes? What do they do?

Pancreatic Enzymes (also called Pancreatic Enzyme Replacement Therapy or PERT) are extracts from pig pancreas. They are given as a pill that you take with your meal. They can be enteric-coated or “non-enteric-coated.” These enzymes can improve protein and fat absorption. This can reduce digestive symptoms in children and adults with EPI. The enzymes work in the digestive system. Their goal is to mimic the function of the pancreas during the process of digestion.

Risks associated with PERTs 

High-dose PERT can lead to fibrosing colonopathy. A theoretical viral transmission, i.e. if the pig from which the pancreas was extracted was infected with a virus and infected the patient, is unlikely. PERTs contain purines, which can be a risk for patients with gout, renal impairment, and hyperuricemia. Current research is focused on developing microbial/recombinant-based PERTs.

How do you take PERTs?

Determine the correct amount

Infants should take around 4,000 units per every 4 oz formula or breast milk

Children less than 4 years old should take 1,000 units per kg per every meal, or 500 units per kg per snack.

Adult and/or Child 4 years and older: 500 units per kg per meal, 250 units per kg per snack

Dosing should not exceed 2,500 lipase units per kg of body weight per meal, or 10,000 lipase units per kg of body weight per day. For example, for a 60 kg (132-pound) adult, 6 Creon capsules (24,000 units) per meal is recommended. This table is a general guide to PERT dosing based on weight, which is how the FDA approved it. Pancreatologists suggest that dosing should consider exocrine pancreatic function and the amount of fat consumed. Consult with your pancreatic specialist for the dose that is right for you.

Examples of Weight-Based Dosing of PERT

When to take enzymes

Take the enzymes with the first bite of a meal. Consider adding more during and towards the end of the meal, especially if the meal is longer than twenty minutes. The goal is to mimic the effects of your pancreas in relation to the quantity of food and type of fats. The dose should be adjusted to the fat content and size of the meal.

Hydration/Alkaline pH

Make sure to drink enough water and stay hydrated. Patients with Cystic Fibrosis have trouble producing fluids, which makes this even more necessary. Consider adding an acid blocker.

Side effects

If the PERT is not absorbed properly, it will produce minimal side effects. Older PERT formulations can make uric acid levels rise (gout) in Cystic Fibrosis patients.

Challenges to taking enzymes correctly

There are many barriers to taking PERT therapy. It is a lifelong  therapy, and it means people with EPI need to take many capsules throughout their lifetime. Additionally, some patients might forget to time their doses with a meal. Some patients struggle with associated psychological disorders like depression and inadequate coping mechanisms which make adherence harder. Teenagers or young adults often find it even more difficult to correctly follow PERT therapy. Young women, in particular, may resist taking full doses due to fear of gaining weight and distorted body image.

It is important to understand that this is a required lifelong therapy. To make sure you follow the treatment correctly, you can take notes on how many capsules you take. This is called self-monitoring If possible, follow the CF Foundation recommendations.

Care providers and healthcare professionals  should understand that it is important to talk to patients who are going through PERT therapy. With respectful and forthright conversations, care providers should help their patients with following the therapy correctly. A multidisciplinary approach (i.e. involving dietitians) will yield the best results.

Use of PERTs for chronic pancreatitis pain

Only Viokace, which exists only in quick release form, may help reduce chronic pancreatitis pain. Other PERTs have shown NOT to be effective for pain, although they are effective for enzyme insufficiency.

Viokace has worked best in young women without a dilated pancreatic duct on CT or MRI scans. A normal dose is three tablets (20,880 lipase units) with each meal with an acid blocker. 

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