How cystic fibrosis affects the pancreas and the digestive system

Cystic fibrosis often affects the pancreas and digestive system because the mucus in these areas becomes thick and sticky. If this occurs, the mucus blocks normal digestive function as well as harbors infection.

The digestive system is responsible for breaking down food, absorbing nutrients from the food, and ridding the body of waste products. The pancreas is an organ that is located in the upper abdomen, behind the stomach and close to the spine. It produces digestive enzymes, which travel to the intestines and break down (digest) food so that nutrients can be absorbed by the body.

Cystic fibrosis and digestion

In cystic fibrosis, mucus clogs the pancreas, and digestive enzymes are not able to get to the intestine. Therefore, food is not properly digested, particularly fats and proteins. About 90% of children experience these problems within their first year; about 59% are affected during infancy.¹ A related symptom is large, greasy, smelly stools.

Lack of nutrition prevents normal growth and development in babies and children. Unhealthy weight loss and difficulty gaining or maintaining weight are common problems for many people of all ages who have cystic fibrosis.

Gastroesophageal reflux disease (GERD), which is more common in people who have cystic fibrosis, sometimes makes lung disease worse.¹

Cystic fibrosis and insulin

In addition to enzymes, the pancreas also produces insulin, a hormone that helps control blood sugar. Over time, the abnormal buildup of enzymes starts to break down pancreatic tissue. This slows and may eventually stop insulin production, resulting in diabetes.

Other problems

Cystic fibrosis may also affect the bowel and liver in the following ways:

• Large stools can cause irritation, swelling, or blockage of the bowels, resulting in the inability to pass stool; vomiting; bulging of the lower part of the large intestine (rectum) through the anus; and collapse of the bowel into itself like a closing telescope.
• Damage to the bile ducts results in eventual cirrhosis and liver disease.
• About 10% of people who have cystic fibrosis develop distal intestinal obstruction syndrome, usually in late childhood or thereafter.¹ This is an obstruction in the small bowel that causes abdominal pain, cramping, and constipation.
• Pancreatitis, an inflammation of the pancreas, may result when thick mucus blocks the tube (duct) that leads from the pancreas to the beginning of the small intestine (duodenum).

Citations

1. Orenstein DM, et al. (2002). Cystic fibrosis: A 2002 update. Journal of Pediatrics, 140: 156–164.