Exocrine Pancreatic Insufficiency (EPI)

Exocrine pancreatic insufficiency or EPI is a term that describes a condition in which a person's pancreas is unable to produce and/or secrete normal levels of enzymes into the gastrointestinal tract resulting in the inability of the person to digest and thus absorb some fats, vitamins, and minerals from foods (maldigestion).

The exocrine (secreted) enzymes made by the pancreas are mainly amylase, protease, and lipase. Any malfunction in the production of one or more of these enzymes can result in exocrine pancreatic insufficiency because these enzymes are needed to help the body to modify and/or absorb foods.

EPI has multiple possible causes and occurs more frequently in men than in women (8 vs. 2/100,000 population).

Most researchers and doctors classify the causes of exocrine pancreatic insufficiency into either pancreatic or non-pancreatic causes, with the most common cause being chronic pancreatitis because that leads to the damage or loss of pancreatic exocrine cells.

Pancreatic causes of EPI

Pancreatic causes of exocrine pancreatic insufficiency include:

• Chronic pancreatitis (recurrent inflammation of the pancreatic tissue)
• Cystic fibrosis (an inherited genetic disorder that affects mucous, digestive enzymes, and secretions)
• Obstruction of the pancreatic duct
• Swachman-Diamond syndrome (a rare congenital disorder that includes exocrine pancreatic insufficiency and other problems)

Non-pancreatic causes of EPI

Non-pancreatic causes of exocrine pancreatic insufficiency include:

• Celiac disease (autoimmune disorder resulting in problems with gluten digestion)
• Crohn's disease (inflammatory bowel disease)
• Autoimmune pancreatitis
• Zollinger-Ellison syndrome (overproduction of gastric acid with recurrent peptic ulcers)
• Surgical procedures that may directly or indirectly affect the pancreas

The symptoms of exocrine pancreatic insufficiency or EPI and their severity vary with the underlying causes of the disease but when symptoms develop, they generally may include:

• Abdominal pain or tenderness
• Gas (increased production)
• Diarrhea
• Bowel movements that have a very bad smell
• A full or bloated feeling
• Weight loss

Other symptoms that may develop due to the severity of the disease or its underlying cause occur somewhat less frequently than those listed above including:

• Chronic abdominal pain
• Bleeding disorders
• Bone pain
• Intestinal blockage

Although primary care doctors can help the person with exocrine pancreatic insufficiency, other specialists may be consulted for the diagnosis and/or treatment. Such specialists may include gastroenterologists, immunologists, surgeons, and/or specialists with training in treating cystic fibrosis and other diseases listed above that can lead to exocrine pancreatic insufficiency.

Although dietitians and not doctors can play a major role in treating exocrine pancreatic insufficiency by providing dietary suggestions that may help to minimize the symptoms.

Exocrine pancreatic insufficiency is diagnosed by the patient's history, physical exam, and certain tests that help distinguish exocrine pancreatic insufficiency from other problems. For example, the history of abdominal pain, bad-smelling bowel movements, and weight loss clinically suggest a diagnosis of exocrine pancreatic insufficiency.

Blood tests may be ordered to check vitamin levels and pancreatic enzymes. A three-day "fecal test" that requires the collection of bowel movement samples is often ordered to determine if fats are being digested appropriately. This test includes a test for fecal elastase 1 to see if the pancreas is making enough of this digestive enzyme.

Other tests may include a CT scan, MRI, and/or endoscopic ultrasound to determine if the pancreas is inflamed or has other changes.

The treatment for exocrine pancreatic insufficiency is termed pancreatic enzyme replacement therapy (PERT). This therapy involves taking prescription drugs that replace the enzyme(s) that your pancreas either produces in insufficient amounts or simply does not produce any enzyme(s).

The following six pancreatic enzyme products (PEPs) have been approved by the U.S. FDA to treat maldigestion and/or exocrine pancreatic insufficiency:

• Creon, Zenpep (pancrelipase enteric)
• Pancreaze, Ultrase, Viokace, Pertzye (pancrelipase)

Your doctors can prescribe those drugs that are most likely to benefit you as an individual. In addition to those PEPs listed above, you may require additional medications such as antacids, protein pump inhibitors (PPIs), and possibly some pain medications like Tylenol (acetaminophen).

In general, people with EPI can help manage it at home by treating the causes of diseases according to their treatments. In addition, to obtain good results from PERT therapy at home, patients are advised that PEPs should be administered together with meals and/or snacks. If this is not done, the PERT therapy may become far less effective. Patients should follow up with their healthcare professional to determine if the therapy is still effective or needs modification as exocrine pancreatic insufficiency can be a progressive disease.

Lifestyle modifications

Lifestyle modifications recommended by many healthcare professionals include:

• Avoiding fatty foods, limiting or stopping alcohol intake
• Stopping smoking
• Eating a well-balanced diet with vitamin supplementation (especially the fat-soluble vitamins A, D, E, and K)

Eating smaller meals more frequently (for example, six small meals per day) also is recommended.

The prognosis of patients with exocrine pancreatic insufficiency depends upon the underlying cause. Individuals with a cause such as drinking alcohol can have a good prognosis if they stop drinking. These people may recover from exocrine pancreatic insufficiency, or at least will likely halt the progression of EPI. However, individuals with autoimmune pancreatitis or cystic fibrosis may continue to progress to almost complete pancreatic insufficiency with a more guarded prognosis; however, even these individuals may do reasonably well if they respond to the enzyme treatment therapies and modify their diets.

If the underlying cause of exocrine pancreatic insufficiency can be prevented, then exocrine pancreatic insufficiency can be prevented (for example, some causes of chronic pancreatitis such as excessive alcohol intake can be prevented). Other causes that are genetic (for example, patients that have the cystic fibrosis gene) or causes that are unknown are not preventable.