Exocrine Pancreatic Insufficiency: Absorbing the Important Facts

Abdominal pain, excessive gassiness, bloating/abdominal distention and loose stool: these are symptoms that all children experience at some point. But as a parent, when are these symptoms something to be worried about? TV and radio ads might have you thinking everyone has exocrine pancreatic insufficiency, or EPI, but fortunately there are specific things you can discuss with your doctor to see if testing for EPI is right for your child.

What Is EPI?

EPI occurs when your intestine can no longer digest food and absorb nutrients due to decreased production of digestive enzymes by the pancreas. Although the pancreas has a role in digesting all macronutrients, it is mostly involved in the digestion of fats.

For many patients, symptoms are non-specific and include crampy abdominal pain, excessive gassiness and/or bloating and loose stool. In children with unrecognized EPI, poor growth, malnutrition or even poor bone health may be present. Genetic causes, such as cystic fibrosis and Shwachman-Diamond syndrome, are the most common causes of EPI among children, but EPI may also be caused by other conditions such as chronic pancreatitis, pancreatic surgery and/or malnutrition.

When Is EPI Considered?

Diagnosing EPI in children starts with a good history. Your doctor will ask you and/or your child about their stool, specifically about the presence of loose stool and fat in the stool, known as steatorrhea. They will also ask about symptoms such as bloating and the relationship to food, especially high fat meals or snacks. Children with steatorrhea, GI symptoms that are worse after a high fat meal, or prolonged history of loose stool with growth or nutritional issues are most likely to need additional testing for EPI.

Testing for EPI

The first test for most patients will be a stool test known as fecal elastase. Although this is the most common test performed to test for EPI, several factors, including the consistency of the stool and how long the sample is stored, can impact the results and must be interpreted by a trained gastrointestinal specialist.

It is also common to obtain blood work to screen for low levels of fat-soluble vitamins, specifically vitamins A, D, E and K. For some children, more advanced testing by either magnetic resonance imaging (MRI) or endoscopic evaluation may be recommended. These tests are measuring the pancreatic response to secretin (a hormone produced by the body), either indirectly by MRI, or directly by endoscopic pancreatic function testing.

Treating EPI

The treatment for EPI is pancreatic enzyme replacement therapy (PERT). In patients with EPI, it is necessary to take PERT with every meal and most snacks. Your child’s dose will be based on their weight and/or amount of fat in their diet. Dosing may be altered by symptom response or as the child grows. In some children, PERT may be started without additional testing and monitored for symptom improvement. In any child, regardless of results of testing, if PERT doesn’t cause improvement, additional testing to evaluate for other causes of the child’s symptoms is necessary. Additionally, most children with EPI will also require a multivitamin that contains appropriate levels of fat-soluble vitamins.

Long-term Expectations

Children diagnosed with EPI should consider this a life-long diagnosis and anticipate taking PERT for life. However, in a small subset of patients, especially those with malnutrition-induced EPI, this can be a temporary condition that improves once nutrition improves. Although less common, in some genetic conditions, there is some evidence that EPI “resolves” with age, likely reflecting a decreased caloric and fat requirement rather than improvement in pancreas function.

The Bottom Line

If your child experiences steatorrhea or prolonged GI symptoms as discussed here, please talk to your doctor to determine if a workup for EPI is appropriate.