How Is the Pancreas Affected by Cystic Fibrosis?

The pancreas, found in the center-left of the abdomen behind the stomach, is connected to the small intestines. A healthy pancreas is a helper organ that releases chemicals that help digest, or process, food and utilizes the energy in carbohydrates.

The purpose of the pancreas

The pancreas assists with digestion by sending a liquid with enzymes and bicarbonate into the intestines. This helps break down food so that nutrients and calories can be moved everywhere in the body and used for growth and energy. The pancreas also makes hormones, including insulin, that regulate blood sugar levels.

The history of cystic fibrosis and the pancreas

Problems with the pancreas gave cystic fibrosis (CF) its name. In 1938, Dr. Dorothy Andersen, a doctor at Columbia University, first described what she called “cystic fibrosis of the pancreas” in children who had died of malnutrition. She identified the unique combination of CF symptoms, including meconium ileus, respiratory and gastrointestinal complications and pancreatic issues in 49 patients at her hospital and others.¹

CF and pancreatic complications

Today, much more is known about cystic fibrosis and the role the pancreas plays in the disease. As with all complications of CF, how well the pancreas works -- or doesn’t -- depends greatly on the type of CF mutation the individual has.

About 80-90% of people with cystic fibrosis have pancreatic insufficiency. This means that the ducts (tubes) in the pancreas become filled with sticky mucus.² This makes it difficult for the intestines to break down and use the nutrients in food, and this undigested food can cause pain, cramping, gas, and either loose, greasy stools or constipation. This is also why it can be difficult for people with CF to get enough nutrients and maintain a healthy weight.

The pancreas also does not produce enough bicarbonate to neutralize stomach acid in every person with CF. This also contributes to pain, cramping, gas, and constipation.

CF-related diabetes (CFRD)

The other important function of the pancreas, producing insulin, can be hindered by CF, either not creating enough insulin, or the body not processing insulin correctly. This leads to CF-related diabetes (CFRD). Unlike Type-2 diabetes, CFRD is not caused by diet or lack of exercise and cannot be prevented.²

Pancreatitis, an inflammation of the pancreas, is a rare complication of cystic fibrosis, occurring in less than 1% of children with CF and 1.6% of adults with CF.³ Interestingly, people with CF who have normal pancreatic function are more likely to develop pancreatitis. Pancreatitis can cause severe pain, vomiting, and diarrhea.⁴

Treating pancreatic issues

Infants with two CFTR mutations associated with pancreatic insufficiency and other signs of malabsorption of nutrients should be prescribed pancreatic enzyme replacement therapies (PERT).³

PERT is prescribed for older people with CF too; although, its use may decrease as people age, perhaps because those that live the longest have a milder form of the disease. Acid blockers are also commonly prescribed for people with CF to treat acid reflux and to help PERT work better. Vitamins A, D, E and K may also be given.³

Other gastrointestinal conditions that may result from the pancreas working poorly in people with cystic fibrosis include:

• Small bowel bacterial overgrowth (from repeated antibiotics)
• Gastric paresis (the stomach emptying too slowly)
• Gastroesophageal reflux disease (GERD) or acid reflux¹