Exocrine Pancreatic Insufficiency

Introduction:

The pancreas is a gland located in the abdomen. It produces essential hormones and enzymes that perform various functions. The hormones produced by the pancreas include insulin and glucagon, which help regulate blood sugar levels. Enzymes are chemical substances produced by the digestive tract that aid in breaking down food particles into smaller pieces that the body can easily absorb. Amylase (an enzyme that breaks down carbohydrates), lipase (an enzyme that breaks down fat), protease and elastase (which breaks down proteins), trypsin, and chymotrypsin are the enzymes produced by the pancreas. These enzymes are then released into the small intestines, where they act on the food.

What Is Exocrine Pancreatic Insufficiency?

EPI is when the pancreas does not produce sufficient enzymes, or the enzymes do not act the way they should. Therefore, the body does not receive enough nutrients and vitamins, leading to malnutrition. Both adults and children can be affected by the condition.

What Are the Causes of Exocrine Pancreatic Insufficiency?

Different conditions that cause damage to the pancreatic cells can cause EPI. The conditions include the following:

• Chronic Pancreatitis: EPI is commonly caused due to chronic pancreatitis. Alcohol, infection, trauma, and certain medications cause long-standing inflammation that leads to chronic pancreatitis. The inflammation that lasts long damages the pancreatic cells that produce enzymes, thereby affecting digestion.

• Acute Pancreatitis: The chances of developing EPI in people with acute pancreatitis, a condition where the inflammation lasts only for a short period, are less. However, untreated acute pancreatitis that develops into chronic pancreatitis also increases the risk of developing EPI.

• Autoimmune Pancreatitis: Autoimmune pancreatitis develops when the body's cells destroy its pancreatic cells. Steroid therapy is helpful for people to boost enzyme production.

• Diabetes: Though the exact relationship between diabetes and EPI is unclear, a hormonal imbalance in diabetes can trigger EPI.

• Genetic Conditions: Genetic conditions like cystic fibrosis and Shwachman-Diamond syndrome can cause EPI. Cystic fibrosis is a genetic disorder in which the digestive system and lungs get blocked with thick mucus. Shwachman-Diamond syndrome is a rare genetic abnormality affecting bones, bone marrow, and pancreas.

• Surgery: Surgery of any part of the digestive system, like the stomach, intestines, or pancreas, can cause EPI, as the system gets disturbed due to surgery.

• Celiac Disease: Celiac disease is a reaction produced in the body due to the consumption of foods containing gluten, which causes symptoms like diarrhea, bloating, and weight loss. People may continue having diarrhea even when following a gluten-free diet, which can occur due to EPI.

• Pancreatic Cancer: Cancer cells replacing the normal cells of the pancreas causes a deficiency of digestive enzymes. Also, the tumor cells can block the enzymes from entering the digestive system. Sometimes, EPI can also occur as a complication of surgery done to treat pancreatic cancer.

• Zollinger-Ellison Syndrome: The stomach produces large amounts of acid, restricting the pancreatic enzymes from working correctly and causing EPI.

• Inflammatory Bowel Diseases: Diseases like ulcerative colitis and Crohn's disease can attack and inflame the digestive system, causing EPI.

What Are the Symptoms of Exocrine Pancreatic Insufficiency?

People with EPI have difficulty digesting foods, especially fats, which may cause uncomfortable digestive issues. The symptoms become noticeable when the enzymes that break down foods reduce by five to ten percent. The symptoms include:

• Abdominal pain.

• Gas and bloating.

• Fatty stools (oily, pale, foul-smelling feces, which float and are difficult to flush down the toilet).

• Diarrhea.

• Constipation.

• Unexplained loss of weight.

• Failure to thrive, especially in infants and children.

What Are the Complications of Exocrine Pancreatic Insufficiency?

People with EPI have a problem digesting carbohydrates, proteins, and fats, which causes a deficiency of nutrients. Inadequate nutrient absorption leads to malnutrition, the symptoms of which include:

• Dry skin.

• Hair loss.

• Brittle nails.

• Tiredness.

• Edema.

• Depression.

• Irritability.

• Difficult to concentrate.

• Poor memory.

• Muscle loss.

• Dizziness or fainting.

• A feeling of cold.

How to Diagnose Exocrine Pancreatic Insufficiency?

Doctors may advise various tests that help diagnose EPI, as many digestive system issues have similar symptoms as those of EPI. They may order the following pancreatic function tests, which include:

• The fecal elastase test (FE-1) helps check for elastase enzymes in stools. Elastase helps the body digest proteins. Therefore, little or no elastase in stools indicates EPI.

• The fecal fat test helps assess fat in stools. A high-fat level can mean EPI.

• The secretin pancreatic function test helps the doctor know how the pancreas works and releases enzymes in response to the hormone secretin. It is an invasive test done only in cases where the other tests indicate pancreatic insufficiency. The patient receives secretion through an (IV) intravenous route during the test, and endoscopic ultrasound collects the digestive fluid and checks it for enzymes.

• Imaging tests like CT (computerized tomography) and abdominal ultrasound help detect pancreatic problems that cause EPI.

How Is Exocrine Pancreatic Insufficiency Managed?

EPI is a life-long condition associated with chronic diseases of the pancreas. The treatment aims at providing the body with necessary nutrients, which help maintain good health. The various treatment options include:

• Pancreatic Enzyme Replacement Therapy (PERT): PERT is a medicine that acts as a replacement for missing pancreatic enzymes. It has to be taken along with food, helping the body break down nutrients in the food. The FDA (Food and drug administration) approved pancreatic enzymes that are available on prescription include:

1. Pancreaze.

2. Pertzye.

3. Creon.

4. Ultresa.

5. Zenpep.

6. Viokase.

• Dietary Changes: Foods high in fat and calories are advised, as fat helps the body absorb nutrients from the food. A dietitian can help to plan the diet accordingly.

• Vitamins: Doctors prescribe supplements containing vitamins A, D, E, and K. As these are fat-soluble vitamins, people with EPI have difficulty absorbing them through food. Replenishing the nutrients keeps the body healthy and maintains required nutrient levels.

• Treatment of Chronic Conditions: Chronic conditions like cystic fibrosis, chronic pancreatitis, and Shwachman-Diamond syndrome have to be treated accordingly by the healthcare team.

• Surgery: Surgery is preferred to open ducts blocked by gallstones. Decompression is a technique that is followed to widen a narrowed pancreatic duct, allowing enzyme-rich pancreatic juice to flow into the intestine.

How to Prevent Exocrine Pancreatic Insufficiency?

Doctors recommend people avoid smoking and drinking, as it can trigger chronic pancreatitis leading to EPI. Chronic conditions that are mostly inherited cannot reduce EPI risk; however, checking symptoms can help.

Conclusion:

Exocrine pancreatic insufficiency is a condition in which there is an inadequate release of pancreatic enzymes into the intestine, causing unpleasant symptoms. Though there is no cure for EPI, PERT can help the body with essential nutrients and reduce unfavorable symptoms. The doctor's recommendations have to be followed while taking PERT. Planning meals with a dietitian's advice can maintain a healthy balance of nutrients, fats, and minerals in the body.