About EPI in cystic fibrosis

What is cystic fibrosis?

Cystic fibrosis (CF) is a genetic disease that affects about 30,000 people in the United States, including 1 out of every 3,500 babies. CF causes the mucus that coats and protects the lungs and digestive system to become thick and sticky.

CF affects 30,000 people in the United States including 1 out of every
3,500 babies

In the lungs

  • The mucus causes coughing and trouble breathing. It often causes lung infections because microbes get stuck in the mucus. These infections can cause damage to the lungs that gets worse over time

In the digestive system

  • The mucus can clog up an organ called the pancreas. The pancreas secretes substances that help digest food in the intestines. Without these substances, the body can’t break down food to be used as nutrients. When these substances can’t reach the intestines, it results in exocrine pancreatic insufficiency (EPI)

What happens in EPI?

The problem in EPI is often thought to be the blockage of pancreatic ducts that secrete digestive substances called pancreatic enzymes.

  • Pancreatic enzymes help break down food into nutrients in the duodenum after the food has been partially digested in the stomach. The intestines then absorb the nutrients into the body where they are used as energy

However, other substances that aid in digestion are also disrupted in EPI. Another important substance is bicarbonate.

  • Bicarbonate helps pancreatic enzymes work efficiently to digest food in the duodenum

Without the digestive action of pancreatic enzymes and the aid of bicarbonate (as well as other digestive substances), people with EPI can’t get enough nutrition from the food they eat. As a result, they have trouble growing or maintaining a healthy weight. They may also have symptoms like gas, bloating, and having lots of bowel movements that seem to be oily.

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