Enzyme replacement therapy for cystic fibrosis

Examples

Generic Name	Brand Name
lipase	Cotazym, Creon, Pancrease, Pancrease MT, Ultrase

How It Works

Enzymes help a person who has cystic fibrosis digest food by replacing digestive enzymes that are normally released by the pancreas. Lipase is available in tablet, powder, or capsule form and is usually taken before eating. Capsules can be swallowed whole or be opened and sprinkled on food.

Why It Is Used

These enzymes are used by people who have cystic fibrosis whose pancreatic ducts are blocked. The blockage prevents digestive enzymes that are made by the pancreas from reaching the intestines, where they are needed for digestion.

How Well It Works

Enzyme supplements can replace natural enzymes so that fat and proteins can be absorbed properly, which improves nutrition and reduces fatty stools.

People with cystic fibrosis who receive enzyme replacement therapy can eat the same foods as anyone else.

Side Effects

• Taking too little medicine can cause nausea, stomach cramps, or diarrhea.
• Very strong enzymes or taking too many enzymes can lead to stomach irritation, constipation, intestinal scarring, or blockage of the intestines.

See Drug Reference for a full list of side effects. (Drug Reference is not available in all systems.)

What To Think About

• Most people who have cystic fibrosis need to take digestive enzymes with meals and snacks.
• Some products work better than others. Generic substitutes may not work as well as brand names. Be sure to talk to your doctor about which enzymes work best or before you switch products.
• Different people need different amounts of enzymes. Some people may have to take several enzymes with each meal, while others may need to take only a few.
• Enzymes may not work as well if you have too much acid in your stomach. Some people need to take another medicine to stop the stomach from making too much acid.
• Coated enzymes will not be destroyed by stomach acid before they reach the intestines.

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Author:	Debby Golonka, MPH Ralph Poore	Last Updated: August 20, 2007
Medical Review:	Michael J. Sexton, MD - Pediatrics Susanna McColley, MD - Pediatric Pulmonology Andrew Swan, MD, CCFP, FCFP - Family Medicine
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