Pediatric exocrine pancreatic insufficiency (EPI)

What is pediatric exocrine pancreatic insufficiency (EPI)?

When a child’s pancreas does not make enough enzymes, it’s called exocrine pancreatic insufficiency (EPI) or pancreatic insufficiency. Enzymes are important proteins that help regulate how cells work.

Your child needs a specific type of enzyme, called digestive enzymes, to digest food, absorb nutrients and grow. Since children with EPI do not have enough of these vital enzymes, they might struggle to gain weight or maintain a healthy weight for their age.

Risk factors

Pancreatic insufficiency is not common in children. However, it is the most common nutrition-related complications associated with cystic fibrosis (CF).

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

• Abdominal pain
• Bloated stomach
• Frequent diarrhea
• Frequent gas
• Greasy stools
• Poor weight gain
• Unintended weight loss

How is pediatric exocrine pancreatic insufficiency (EPI) diagnosed?

Our team will likely perform some or all of the following tests:

• Physical examination - Your child’s doctor will check for visible signs and symptoms of pancreatic insufficiency, such as stomach bloating, pain or being underweight.
• Blood work - Taking a sample of your child’s blood and sending it to a lab for testing can show your child’s doctors whether your child has a fat-soluble vitamin deficiency.

These vitamins (A, D, E, and K) are important in vision, bone health and more. If these vitamin levels are low, your child’s care team will prescribe vitamin supplements and monitor their levels in the blood on an ongoing basis.

• Stool test - Doctors will check your child’s stool for certain enzymes that help with digestion. This information can help them detect pancreatic insufficiency.
• Endoscopic pancreatic function test (ePFT) - For an ePFT, your child will be under anesthesia. Your child’s doctor will insert a narrow, bendable tube with a built-in light and camera, called an endoscope, into your child’s mouth and feed it down to the small intestine. Your child’s doctor will collect fluid in the small intestine that they can analyze.

What causes pediatric exocrine pancreatic insufficiency (EPI)?

One of the most common causes of pediatric pancreatic insufficiency is cystic fibrosis (CF), a rare genetic condition that disrupts the body’s mucus production across the body, including the pancreas.

Other genetic conditions in which EPI is seen include:

• Shwachman (Diamond) syndrome
• Pearson’s syndrome
• Johanson-Blizzard syndrome
• Isolated enzyme deficiencies
• Pancreatic agenesis
• Hypoplasia of the pancreas
• Chronic pancreatitis

How is pediatric exocrine pancreatic insufficiency (EPI) treated?

Treating your child’s pancreatic insufficiency is a team effort. Our team of specialists will work closely with your family to create a treatment plan that reflects your child’s preferences, habits, lifestyle and overall health.

That team includes:

• Pediatric gastroenterologists
• Dietitians
• Psychologists

Most of the time, children with pancreatic insufficiency can manage the condition with:

• Oral medications and supplements - These include enzyme replacements and fat-soluble vitamins. This helps offset the shortages in your child’s pancreas. Your child can take these medications and supplements with each meal.
• Eating more calories - Eating foods with more calories helps your child gain weight.
• Dietician support - Our dieticians are highly trained in helping children with pancreatic insufficiency eat foods that will help them gain weight. We will also prioritize including foods in your child’s nutrition plan that they like. We also know the best types of vitamins to supplement with to correct any fat-soluble vitamin deficiencies.

In addition to prioritizing what works best for your child, our experts are also a part of a network of centers across the country conducting ongoing studies into pediatric pancreatic health. We know what the latest research has found because we’ve helped contribute to it. We’ll incorporate those findings into your child’s care so you can trust our decision-making is rooted in the latest scientific advances.