About 90 percent of people with cystic fibrosis (CF) need to take enzymes before eating. The pancreas in people with CF produces a thick mucus that blocks the discharge of enzymes needed for digestion, called pancreatic insufficiency. Enzymes administered via pancreatic enzyme replacement therapy (PERT), helps ensure that nutrients are effectively absorbed from food.

The most effective test to confirm this insufficiency is to measure an enzyme, the fecal elastase, which is low in people with this condition.

How PERT works for CF patients

The digestive enzymes are taken by mouth in a capsule. They are distributed throughout the intestines and help digest food and absorb nutrients in order to keep the body healthy.

Each person needs a different amount of enzymes, so these levels must be checked and determined by a healthcare practitioner. The enzymes work for about 45 to 60 minutes after being taken.

There are several brands of enzymes available in different strengths, all of them containing three digestive enzymes – lipase, protease, and amylase. Lipase digests fat, protease digests protein, and amylase digests carbohydrates. Fat is the most difficult for the body to digest, so lipase is the enzyme that must be dosed most accurately.

Pancreatic enzymes should be taken with all meals, snacks, and drinks that contain fat. Some foods do not require enzyme supplements, such as fruit (except avocado) and vegetables, sugar, jam, honey and syrup, fruit juice, fizzy drinks, sorbet, jelly and boiled sweets, and juice-based supplements.

If the enzymes are not taken, or the dose is not adequate, it can lead in the short term to poorly digested food that causes gas, pain, and unpleasant smells, along with constipation or frequent stools. In the long term, a higher body weight is related to better lung function, further emphasizing the importance of taking the enzymes with all meals.

Taking too many enzyme supplements may cause damage to the intestines, but taking fewer than needed can keep nutrients from being fully absorbed. In very high doses, excess uric acid can occur, although this condition is rare. If excessively high doses are needed, the enzyme efficacy can be improved by using a proton pump inhibitor or H2 antagonist  to reduce gastric acid production.

Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.