Treatment Options

Pancreatic Enzyme Replacement

Functioning of the pancreas can be compromised in CF if the pancreas ducts become clogged, blocking the delivery of the digestive enzymes that the pancreas generates and secretes. If the enzymes are blocked, they do not reach the food in the small intestines, which can lead to poor digestion and absorption, as well as problems with weight gain.

Fortunately, pancreatic enzymes replacements or "enzymes" are available to help people with CF digest and absorb their food. Replacement enzymes generally come in capsules containing beads with a special "enteric coating" that allows them to dissolve in the small intestine, where they promote nutrient absorption and proper digestion.

Enzyme capsules should be taken just before meals and snacks. Adults and older children can swallow the capsules whole with liquid, but for small children and infants, capsules should be opened and the beads mixed with soft food, such as applesauce.

If a baby spits out the beads, continue feeding the bead mixture until it is all swallowed. The enzymes do not have a taste, but the child may take time to adjust to the texture. If your baby continues to struggle with the enzymes, you can call your CF dietitian or care center.

Signs of Enzyme Maldigestion and Malabsorption
The following are symptoms of inadequate enzyme activity. Patients who receive enzyme replacement and experience these symptoms may need to adjust their dose or the type of enzymes they use.

• Poor weight gain despite a good (sometimes ravenous) appetite
• Frequent, loose and/or large bowel movements
• Foul-smelling bowel movements
• Mucus or oil in the bowel movement
• Excessive gas and/or stomach pain
• Distention or bloating

Do not increase or decrease the dose of enzymes without talking to your CF dietitian or care provider.

General Information About Enzymes

The FDA has required all pancreatic enzyme manufactures to undergo testing and approval for their formulations, and set April 28, 2010 as the deadline. So far, three brand pancreatic enzymes have met the new rigorous requirements set by the FDA and have been approved. These are Creon, Zenpep and Panceaze. For more information on pancreatic enzyme treatment, click here.

• Enzymes should be taken before eating.
• Enzymes are needed with all meals and snacks, milk, breast milk, formula and nutritional supplements.
• Avoid skipping enzyme doses. Keep a supply with you when eating away from home.
• Enzymes work for about an hour after taking them.
• Always take the right dose.
• Slightly higher doses may be needed with foods that are high in fat.
• If the enzyme capsules are opened, do not crush or chew the enzyme beads, because they won't work as well.
• Do not mix enzymes into food ahead of time.
• Keep enzymes at room temperature. Do not store them over toaster or inside a hot car, and do not refrigerate them.
• Keep the container tightly sealed when not in use.
• For persons who supplement their diet with tube feedings, enzymes may be taken by mouth before starting tube feeding.
• Generic enzymes are less effective than name-brand enzymes. If you receive generic enzymes, alert your CF dietitian or care provider.

Foods That Do Not Require Enzymes

Foods that contain only simple carbohydrates do not require replacement enzymes to digest. You can consume any of the following foods without enzymes:

• Fruits
• Juice and juice drinks
• Soft drinks and sport drinks
• Infant rehydrating formulas
• Tea or coffee without cream
• Hard candy
• Fruit snacks
• Jelly beans
• Gum
• Popsicles, freezer pops, flavored ice