Exocrine pancreatic insufficiency (EPI) is a condition characterized by deficiency of the exocrine pancreatic enzymes, resulting in the inability to digest food properly, or maldigestion.

What causes exocrine pancreatic insufficiency (EPI)?

The pancreas is part of your digestive system. It makes enzymes that aid digestion and help your body absorb nutrients.

When you have EPI, you don’t have enough digestive enzymes. Foods pass through your intestines in a more complete (undigested) state. As a result, your body doesn’t get the nutrients it needs from foods.

The different types of pancreatic enzymes include:

Amylase, which breaks down carbohydrates.
Lipase, which breaks down fats.
Protease and elastase, which break down proteins.

What causes exocrine pancreatic insufficiency (EPI) in adults?

Chronic pancreatitis is the main cause of EPI in adults. As many as 8 in 10 adults with this disorder develop EPI. Pancreatitis causes inflammation and swelling of the pancreas. Over time, chronic inflammation can damage the pancreatic cells that make digestive enzymes.

Other causes of EPI in adults include:

What causes exocrine pancreatic insufficiency (EPI) in children?

Cystic fibrosis is the top cause of EPI in infants and children. Children inherit cystic fibrosis from a parent. It causes thick mucus to build up in the lungs, making breathing difficult. Mucus also collects in the pancreas, which keeps digestive enzymes from reaching the small intestine.

Nearly 9 in 10 infants with cystic fibrosis develop EPI within the first year. The rest are at risk for developing EPI during childhood or adulthood.

Another inherited condition, Shwachman-Diamond syndrome (SDS), also causes EPI in children. With SDS, the part of the pancreas that makes enzymes doesn’t work properly.

What are the symptoms of exocrine pancreatic insufficiency (EPI)?

People with EPI have a particularly difficult time absorbing fats from foods. This leads to uncomfortable digestive problems, such as:

Abdominal pain, gas and bloating.
Constipation.
Diarrhea.
Fatty stools (pale, oily, foul-smelling poop that floats).
Unexplained weight loss or failure to thrive in infants and children.

MANAGEMENT AND TREATMENT

What are the complications of exocrine pancreatic insufficiency (EPI)?

People with EPI can’t absorb enough fats, proteins and carbohydrates from foods. This problem is called malabsorption. Your body needs these nutrients for energy and to maintain organ function. Malabsorption of nutrients can lead to malnutrition.

Signs of malnutrition include:

Dry skin, brittle nails and hair loss.
Depression.
Edema (tissue swelling).
Fatigue or dizziness.
Feeling cold all the time.
Irritability.
Memory and concentration issues.
Muscle loss.

How is exocrine pancreatic insufficiency (EPI) managed or treated?

EPI is a lifelong condition. Treatments focus on getting your body the nutrients it needs to maintain good health. Treatments include:

Pancreatic enzyme replacement therapy (PERT): This prescription medication is a substitute for the missing digestive enzymes. You take PERT with meals to help your body break down nutrients in food.
High-calorie, high-fat diet: It’s important to get enough calories and fat with your meals. Fat helps your body absorb nutrients. You may benefit from working with a dietitian.
Vitamins: You may need to take prescription vitamins to help your body maintain healthy nutrient levels. Your provider may prescribe vitamins A, D, E and K. People with EPI have a harder time absorbing these fat-soluble nutrients from food.

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