Managing Exocrine Pancreatic Insufficiency and Cystic Fibrosis

If you have cystic fibrosis, there is a high likelihood that you will also develop exocrine pancreatic insufficiency, or EPI. In people with EPI, the pancreas can’t release enough of the digestive enzymes needed to properly break down food. Up to 90 percent of people with cystic fibrosis will develop EPI, according to a review published in Journal of Clinical Medicine in December 2021.

Because cystic fibrosis is a genetic disease, it is often diagnosed shortly after birth — and most by age 2, according to the Cystic Fibrosis Foundation. This also means a likelihood of developing EPI at an early age, which, left untreated, could restrict the ability to grow and cause weight loss, abdominal bloating, and more.

“In EPI, the pancreas doesn’t have enough enzymes to digest food, and then what you do digest may not be absorbed due to cystic fibrosis,” says Steven D. Freedman, MD, PhD, a professor of medicine at Harvard Medical School and the director of The Pancreas Center at Beth Israel Deaconess Medical Center in Boston.

How Cystic Fibrosis Interferes With Digestion

Cystic fibrosis is characterized by a buildup of mucus in the pancreas and other organs. These secretions, which are normally thin and slippery, become thick and sticky in people with EPI. The mucus then blocks the tubes that carry key enzymes for digestion from your pancreas to your intestines.

“These thick secretions plug the pancreatic duct system and lead to scarring of the pancreas and loss of exocrine pancreatic function,” says Dr. Freedman. When this occurs, the pancreas can’t effectively deliver the enzymes needed to digest fat, protein, and carbohydrates. The result is one of the hallmark symptoms of EPI: fatty, oily, foul-smelling stools.

Additionally, although lung function can vary greatly, Freedman notes that there’s a clear correlation between the severity of the cystic fibrosis gene mutation and pancreatic function: “The more severe the mutations of the cystic fibrosis gene a person has, the more disease and scarring there is in the pancreas,” he says.

Once you’ve been diagnosed with EPI, you’ll begin treatment with pancreatic digestive enzyme replacement therapy (PERT). These enzymes are taken with each meal to help with digestion and prevent common symptoms such as fatty stools, diarrhea, and stomach pain.

A Diet for EPI and Cystic Fibrosis

Notably, you may need to eat twice as many calories as people without the cystic fibrosis, according to the Cystic Fibrosis Foundation. Not only is it harder to digest food, but you also need to consume more energy to breathe normally and fight lung infections. Children with cystic fibrosis also have higher nutritional needs to ensure healthy growth and development.

“All together, it’s so much harder for people with cystic fibrosis to get the proper nutrition,” Freedman says.

Due to varying calorie and nutrition needs from person to person, it’s important to consult your doctor or a dietitian who works with people with cystic fibrosis to develop a personalized diet for you.

You may need to go on a high-fat diet to ensure proper daily energy and take additional digestive enzymes to tolerate the extra fat, says Freedman. People with cystic fibrosis and EPI are also more likely to have low levels of the fat-soluble vitamins A, D, E, and K, so you may need to take these dietary supplements.

How to Maintain Your Lung Health

“Good nutrition leads to better lung function,” says Freedman. “So taking the right dose of digestive enzymes for EPI and consuming enough calories will help with cystic fibrosis treatment.”

In addition to digestive enzyme replacement, there are newer medications available that may be able to improve your nutritional health. In particular, cystic fibrosis transmembrane conductance regulator (CFTR) modulators may help restore the flow of pancreatic enzymes in some people with cystic fibrosis, which can improve digestion, according to a review published in February 2020 in the journal Annals of the American Thoracic Society. The review found that in younger children, the medication can also reverse some of the accumulated damage in the pancreas. A study published in August 2021 in Pediatrics International also shows a promising and inexpensive new way to detect early pancreatic malfunction in children with an ultrasound imaging technique called point shear-wave elastography.

Living with these conditions can be challenging, but treating EPI can help you better manage cystic fibrosis. Schedule regular checkups with your doctors and arrange an immediate appointment if you experience worsening symptoms.