Enzyme Replacement Therapy for Cystic Fibrosis

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Enzyme Replacement Therapy for Cystic Fibrosis


Generic NameBrand Name
pancrelipaseCreon, Pancreaze, Zenpep

How It Works

Enzymes help a person who has cystic fibrosis digest food by replacing digestive enzymes that are normally released by the pancreas. Pancrelipase is available in tablet, powder, or capsule form.

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

All medicines have side effects. But many people don't feel the side effects, or they are able to deal with them. Ask your pharmacist about the side effects of each medicine you take. Side effects are also listed in the information that comes with your medicine.

Here are some important things to think about:

  • Usually the benefits of the medicine are more important than any minor side effects.
  • Side effects may go away after you take the medicine for a while.
  • If side effects still bother you and you wonder if you should keep taking the medicine, call your doctor. He or she may be able to lower your dose or change your medicine. Do not suddenly quit taking your medicine unless your doctor tells you to.

Call 911 or other emergency services right away if you have:

  • Trouble breathing.
  • Swelling of your face, lips, tongue, or throat.

Call your doctor if you have:

  • Hives.

Common side effects with high doses of this medicine include:

  • Diarrhea.
  • Blocked intestine.
  • Nausea.
  • Stomach cramps or pain.

If you use the powder form of this medicine, do not breathe in the powder. You could have side effects such as stuffy nose, shortness of breath, or trouble breathing.

If you take the tablet form of this medicine, swallow it right away. It can irritate your mouth if you hold the tablet in your mouth.

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

What To Think About

Follow the eating plan your doctor gave you. This can help the medicine work properly and can help prevent indigestion.

Drink plenty of water while you are using this medicine.

One brand or dose form (tablet, capsule, liquid) may work better for you than another. Don't switch products before talking to your doctor first.

Enzymes may not work as well if you have too much acid in your stomach. Your doctor may suggest that you take another medicine to stop the stomach from making too much acid. Do not use antacids that contain calcium carbonate and/or magnesium hydroxate. They may not let the pancrelipase work properly.

Taking medicine

Medicine is one of the many tools your doctor has to treat a health problem. Taking medicine as your doctor suggests will improve your health and may prevent future problems. If you don't take your medicines properly, you may be putting your health (and perhaps your life) at risk.

There are many reasons why people have trouble taking their medicine. But in most cases, there is something you can do. For suggestions on how to work around common problems, see the topic Taking Medicines as Prescribed.

Advice for women

If you are pregnant, breast-feeding, or planning to get pregnant, do not use any medicines unless your doctor tells you to. Some medicines can harm your baby. This includes prescription and over-the-counter medicines, vitamins, herbs, and supplements. And make sure that all your doctors know that you are pregnant, breast-feeding, or planning to get pregnant.


Follow-up care is a key part of your treatment and safety. Be sure to make and go to all appointments, and call your doctor if you are having problems. It's also a good idea to know your test results and keep a list of the medicines you take.

Complete the new medication information form (PDF)(What is a PDF document?) to help you understand this medication.


ByHealthwise Staff
Primary Medical ReviewerJohn Pope, MD - Pediatrics
Specialist Medical ReviewerSusanna McColley, MD - Pediatric Pulmonology
Last RevisedMay 14, 2012

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