Exocrine pancreatic insufficiency (EPI) definition and facts
- Exocrine pancreatic insufficiency (EPI) is a condition in which a person's pancreas is unable to produce and/or secrete adequate amounts of enzymes into the gastrointestinal track resulting in an inability to digest and thus absorb some fats, vitamins and minerals from food.
- Symptoms of exocrine pancreatic insufficiency may include:
- Causes of exocrine pancreatic insufficiency may be either pancreatic or non-pancreatic, for example, a pancreatic cause could be chronic pancreatitis while a non-pancreatic cause could be celiac disease.
- Exocrine pancreatic insufficiency may be difficult to diagnose since it symptoms mimic other problems; however, the patient's history, physical exam and blood tests to check on the levels of vitamins, pancreatic enzymes along with studies on fat absorption (fecal test), and results from CT, MRI and/or ultrasound studies of the pancreas can provide the diagnosis of exocrine pancreatic insufficiency.
- Treatments of exocrine pancreatic insufficiency may include pancreatic enzyme replacement therapy (PERT), dietary changes and vitamin supplements. PERT treatment plans are individualized according to the needs of the patient and the severity of EPI.
- Individuals can help manage exocrine pancreatic insufficiency at home by following their doctor's instructions for treating the underlying causes of exocrine pancreatic insufficiency-additionally, lifestyle modifications such as avoiding fatty foods, reducing or eliminating alcohol intake and eating a balanced diet with vitamin supplementation and/or carefully timing PERT therapy with meals according to your doctor's instructions can be very helpful in reducing symptoms.
- In some individuals, exocrine pancreatic insufficiency can be prevented or stopped from progressing if the underlying cause is treated; however, other causes (for example, genetic causes such as presence of the cystic fibrosis gene) are not preventable.
- The prognosis for an individual with exocrine pancreatic insufficiency depends on the underlying cause. Some individuals who have underlying causes that are treatable may have a good prognosis, but others may have only a fair to poor prognosis if they develop complete pancreatic insufficiency and do not respond well to enzyme treatment therapies or refuse to modify their diets.
What is 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 track 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).
What causes exocrine pancreatic insufficiency (EPI)?
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 are:
- Chronic pancreatitis (recurrent inflammation of the pancreatic tissue)
- Cystic fibrosis (inherited genetic disorder that effects mucous, digestive enzymes and secretions)
- Obstruction of the pancreatic duct
- Swachman-Diamond syndrome (rare congenital disorder that includes exocrine pancreatic insufficiency and other problems)
Non-pancreatic causes of EPI
Non-pancreatic causes of exocrine pancreatic insufficiency are:
- 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 effect the pancreas
What are the symptoms of exocrine pancreatic insufficiency?
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)
- 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 include:
Which specialties of doctors treat exocrine pancreatic insufficiency (EPI)?
Although primary care doctors can help the person with exocrine pancreatic insufficiency, other specialists that 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.
How is exocrine pancreatic insufficiency (EPI) diagnosed?
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 suggests 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 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.
What is the treatment for exocrine pancreatic insufficiency (EPI)?
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 US FDA to treat maldigestion and/or exocrine pancreatic insufficiency:
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).
How can I help manage exocrine pancreatic insufficiency at home?
In general, people with EPI can help manage it at home by treating 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 health-care professional to determine if the therapy is still effective or needs modification as exocrine pancreatic insufficiency can be a progressive disease.
Lifestyle modifications recommended by many health-care professionals include
- avoiding fatty foods, limiting or stopping alcohol intake,
- stopping smoking, and
- 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.
Can exocrine pancreatic insufficiency (EPI) be prevented?
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.
What is the prognosis for a person with exocrine pancreatic insufficiency (EPI)?
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.
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