Cystic fibrosis (CF) may lead to exocrine pancreatic insufficiency (EPI). This is because CF causes the body to produce sticky mucus that can block the pancreatic duct, leading to various symptoms.

EPI affects how the body digests food. The pancreas produces enzymes that aid digestion. EPI can occur when the body does not produce enough digestive enzymes or these enzymes are not functioning correctly. This can result in the small intestine being unable to digest food properly or extract certain nutrients.

CF is a genetic condition that affects a certain protein in the body. In people with CF, the abnormal protein affects how the body produces mucus, causing it to become thick and sticky. This sticky mucus can build up and lead to blockages, infections, and damage in major organs, including the pancreas.

This article explores the link between EPI and CF. It also discusses EPI’s symptoms, diagnosis, and treatment. Finally, it advises when a person may want to speak with a doctor.

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When the pancreas functions typically, it produces digestive enzymes and bicarbonate, which enter the small intestine and aid digestion.

When a person has CF, the mucus produced is typically thick and sticky. This sticky mucus can build up and block the main passage that runs from the pancreas into the small intestine. This is the pancreatic duct.

When CF causes a blockage in the pancreatic duct, it can prevent an adequate amount of digestive enzymes and bicarbonate from entering the small intestine. This can lead to EPI.

Research included in a 2023 review suggests CF is one of the most common causes of EPI, and approximately 85% of people with CF also have EPI. CF can also cause pancreatitis which can worsen EPI.

When a person with CF has EPI, the body cannot fully digest the fats, carbohydrates, and proteins from food. This can lead to several symptoms a person can look out for, including:

If a person with CF is experiencing symptoms of EPI, a doctor will typically ask for a full medical and family history. They may also want to carry out a physical examination and perform various tests.

A physical examination for EPI may involve a doctor checking the abdominal area for any swelling, tenderness, or pain. It may also involve examining the person for any signs of weight loss or malnutrition and listening to the abdominal area for any abnormal sounds.

There are two types of testing methods for EPI. Indirect testing assesses if there are any signs of malnutrition from the maldigestion of nutrients. Direct testing looks specifically at how the pancreas functions.

A doctor may ask a person to provide a stool sample to test for EPI. The fecal elastase-1 stool test is the most common type of indirect test for EPI.

Direct tests examine the pancreas’s functioning by collecting fluid from the pancreas and measuring the levels of digestive enzymes and bicarbonate.

Direct tests are typically the most sensitive type of diagnostic test for EPI. However, direct tests can also be invasive, time-consuming, and expensive. Therefore, indirect tests are a more common diagnostic tool for EPI.

A doctor may also want to perform blood tests to check if a person has signs of malnutrition, such as low levels of fat-soluble vitamins and minerals.

To treat EPI, a doctor may prescribe pancreatic enzyme replacement therapy (PERT). PERT describes a type of medication that contains a mixture of digestive enzymes. It can help a person absorb more nutrients from their food.

If a person with CF has EPI, a doctor may also recommend acid-suppressive medications. These medications can improve how PERT works and reduce EPI symptoms in a person with CF.

Alongside PERT, a doctor may recommend a person make some healthy diet and lifestyle changes, including:

  • following a balanced diet that includes an adequate amount of healthy fats
  • eating smaller amounts regularly
  • taking supplements, such as vitamins A, D, E, and K
  • limiting alcohol
  • quitting smoking

If a person with CF experiences symptoms of EPI, they should speak with a doctor as soon as they can.

Additionally, people with CF should speak with a doctor immediately if they experience any worsening CF symptoms, new symptoms, or problems with their blood sugar levels.

People should call 911 immediately if they experience symptoms of respiratory failure, which include:

A person with cystic fibrosis (CF) can be more vulnerable to developing exocrine pancreatic insufficiency (EPI) due to the risk of sticky mucus blocking the pancreatic duct. This can prevent the small intestine from digesting food properly, which can prevent the body from absorbing vital nutrients from the food.

EPI can lead to symptoms, such as pain and discomfort in the abdomen, diarrhea, and unexplained weight loss. Treatment for EPI can include pancreatic enzyme replacement therapy (PERT) and lifestyle changes.

A person with CF should speak with a doctor as soon as possible if they experience symptoms of EPI, develop new CF symptoms, or if their current CF symptoms worsen. They should call 911 immediately if they experience symptoms of respiratory failure.