Exocrine pancreatic insufficiency occurs when the pancreas does not produce enough of the enzymes that aid digestion.
This condition can lead to poor absorption of nutrients, weight loss, and a shortage of vitamins.
Two of the main causes of exocrine pancreatic insufficiency (EPI) are cystic fibrosis and chronic pancreatitis. Symptoms are similar to other common diseases of the stomach and intestine, and doctors often underdiagnose EPI.
A healthcare provider can successfully treat EPI with prescription enzymes and lifestyle changes.
Fast facts on exocrine pancreatic insufficiency
- The pancreas is one of the organs that make up the gastrointestinal (GI) system.
- The pancreas produces amylase, protease, and lipase, three substances known as enzymes that are necessary for the normal digestion of food.
- Pancreatic enzyme replacement therapy (PERT) medications are made from the pancreas of a pig.
- It is possible for a person to live without a pancreas.
Exocrine pancreatic insufficiency is a digestive disorder involving the pancreas and the enzymes it produces. Enzymes are chemicals released to bring about a specific chemical reaction, and the pancreas secretes many different enzymes that serve different purposes.
Digesting food is a complicated process, starting in the mouth with chewing and the release of saliva. Once a person swallows food, acids in the stomach break it down.
After about 15 minutes, the broken-down food moves to the small intestine. Here, the pancreas provides the necessary enzymes to convert the food into smaller molecules. These molecules are then absorbed into the bloodstream and sent on to nourish the body.
The pancreas, along with the salivary and sweat glands, is one of the main exocrine glands. Exocrine glands release substances into another organ or the surface of the body through an opening called a duct.
Endocrine glands, such as the thyroid and pituitary, are different in that they release their hormones directly into the bloodstream. The pancreas serves as both. A correctly functioning pancreas enables effective digestion and absorption of important nutrients.
In EPI, the pancreas does not produce enough of the enzymes needed to digest food. Without this breakdown, an individual cannot absorb the necessary nutrients and vitamins. This leads to diarrhea, vitamin deficiency, and weight loss.
If a healthcare professional does not properly treat EPI, the condition can result in delayed or limited growth in infants and children, bone problems, a reduced life expectancy, and exposure to infections.
There are many causes of EPI.
Any condition that damages the pancreas and either stops or blocks the release of its enzymes can result in EPI. The two most common causes are cystic fibrosis and chronic pancreatitis.
Cystic fibrosis, a life-threatening genetic disorder that develops during childhood, produces thick, sticky mucus that affects both the lungs and digestive system. The mucus builds up and plugs the opening of the pancreas, preventing the natural release of enzymes during digestion.
In chronic pancreatitis, the pancreas becomes inflamed. Normal pancreatic tissue starts to become scar tissue. The build-up of this scar tissue prevents the digestive enzymes from leaving the duct.
Research has confirmed a link between smoking and the development of chronic pancreatitis.
Other medical conditions and diseases that can cause EPI are:
- pancreatic cancer
- surgical removal of the pancreas
- obstructions of the pancreatic duct
- celiac disease
- Crohn’s disease
- autoimmune pancreatitis
- Zollinger-Ellison Syndrome, in which a tumor of pancreatic cells leads to the production of too much gastric acid, eventually leading to gastric ulcers
- dumping syndrome, a collection of symptoms including weakness and rapid bowel movements that sometimes occurs following gastric surgery
- gastric bypass and other GI surgeries
EPI can be difficult to diagnose because it shares symptoms with other disorders of the digestive system.
The most frequent symptoms of EPI are diarrhea and weight loss.
People refer to the type of diarrhea in EPI as steatorrhea. This type of diarrhea produces stools that are:
- extremely foul smelling
These stools contain oily droplets. Because they consist of large quantities of undigested fat, they stick to the toilet bowl or float on top of the water, making them hard to flush.
Additional symptoms of EPI might also include:
A healthcare provider will often diagnose EPI based on the above symptoms, particularly when the individual describes fatty stools and weight loss and once the doctor rules out other, more common conditions.
The distinctive diarrhea of EPI does not occur until the pancreas loses 90 percent of enzyme production. This can make EPI difficult to diagnose.
Treatment rarely begins straight away, due to the slow-developing nature of the condition. There is no danger in waiting for a formal diagnosis before beginning treatment.
Tests to confirm the diagnosis include taking a stool sample for laboratory analysis and various blood tests to check for fat deposits and vitamin deficiencies. The doctor may also request a CT scan to examine the underlying causes of EPI.
Pancreatic enzyme replacement therapy (PERT) is the standard treatment for EPI.
PERT medications are only available on prescription. They are derived from the natural, intact pancreas of a pig and contain all three pancreatic enzymes.
This therapy takes over the role of the pancreas. The amount of medication given will differ from person to person and is based on both body weight and the amount of fat intake in the diet.
A person on a course of PERT must take these medications with all meals and snacks.
PERT is safe and has very few side effects. The U.S. Food and Drug Administration (FDA) have approved the following six PERT medications:
In addition to taking the replacement enzymes, individuals with EPI need to stop habits that can worsen both the health of the pancreas and overall quality of life.
These changes include:
- avoiding smoking and giving up if you already do
- eating a well-balanced diet, often with reduced fat
- reducing meal size but increasing frequency
- not consuming alcohol
- taking vitamin supplements, primarily for fat-soluble vitamins A, D, E, and K, under the guidance of a medical professional
Treatment can help people with the condition eat and digest their food normally, allowing them to absorb nutrients and enjoy a better quality of life.