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Pancreatic Enzyme Replacement Therapies (PERT) for Cystic Fibrosis

With cystic fibrosis, the same thick mucus that fills the lungs also keeps the pancreas from releasing enzymes that the body needs to digest food. This condition is called pancreatic insufficiency. Almost all people with CF need to take extra enzymes to make up for what their pancreas can’t do.

What are pancreatic enzyme replacement therapies?

These are called pancreatic enzyme replacement therapies (PERT). There are several brands and formulations. The enzymes a person needs change depending on age, gender and diet.1,2

Among people with CF, 97.5% of those with a class I, II, and III mutations are prescribed pancreatic enzymes, while 36.1% of those with class IV or V mutations take PERT.3

How do pancreatic enzyme replacements work?

Pancreatic enzyme replacements break down complex carbohydrates, fats, and proteins. The side of the enzyme bottle will list the amount of lipase (to digest fat), protease (to digest protein), and amylase (to digest starch). The number after the brand name is usually the amount of lipase in each capsule. Because fat is the hardest nutrient for your body to process, the number of enzymes you are prescribed is usually based on the amount of lipase you will need.

Things to know about taking enzymes

Most enzymes come in a capsule (pill) and begin working about 45 minutes to 1 hour after swallowed. Enzymes do not have a taste and should be taken with every meal and snack.1 You may be prescribed a fixed-dose to take with each meal and a smaller amount with each snack. You may be told to take additional enzymes if eating a high-fat meal.

Infants and small children who cannot yet swallow pills need to have their enzymes mixed into acidic food, such as applesauce.2

Side effects are rare, but the most common ones reported are headache, dizziness, abdominal pain, and flatulence. People taking very high doses of PERT are more likely to develop a serious condition called fibrosing colonopathy.2

Why is it recommended for people with cystic fibrosis to take enzymes?

Pancreatic enzymes help your body break down the food you eat so that you can get the calories, vitamins, and minerals you need to gain and maintain weight and stay healthy. Without enzymes, your body cannot digest fat, proteins, or starch very well. This leads to gas, pain, gassiness, constipation, or loose, greasy, frequent stools. In older children and adults, distal intestinal obstruction syndrome (DIOS) may develop.

It can also be very hard to maintain a healthy weight without enzymes. Studies have proven that people with CF who maintain a healthy body weight have better lung function.If you have trouble paying for your enzymes, the CF Foundation’s Compass program can help. You can reach Compass at 844-COMPASS (844-266-7277) Monday through Friday, 9 a.m. to 7 p.m. Eastern Time, or email [email protected]

Tips for taking enzymes

There are many tips and tricks that help make pancreatic enzymes work best. These include:

  • Take enzymes every time you eat any food, unless that food is pure sugar (ice pops, hard candy, or juice).
  • Swallow the capsules whole, washing them down with a liquid.
  • If you can’t swallow a capsule, sprinkle the beads from the capsule in an acidic food such as applesauce. Don’t crush the beads because this breaks the coating that helps the chemical release at the right stage during digestion.
  • If a meal lasts more than 30 minutes, split the enzyme dose, taking half at the beginning of the meal and half partway through. One study found that digestion of fat was better when enzymes were taken during or after meals.2
  • Carry extra enzymes to avoid missing a dose when eating away from home.
  • Keep enzymes in convenient places to make it easy to remember to take them. On the kitchen counter, the table where you eat, bedside table, your purse, and backpack are all good places.
  • Leave extra enzymes at relative’s and friend’s houses.
  • Pack enzymes in your lunch bag or box.
  • Store enzymes at room temperature (59°-86°F).
  • If you are going into the hospital, ask if your enzymes are available through the hospital pharmacy. If not, bring your own from home.

Taking too many enzymes can damage your intestines and taking too few can keep you from absorbing the nutrients you need. That’s why it’s important to talk to your CF team before changing doses.

Written by: Jessica Johns Pool | Last reviewed: September 2019
  1. Cystic Fibrosis Foundation. Enzymes. Available at: https://www.cff.org/Life-With-CF/Daily-Life/Fitness-and-Nutrition/Nutrition/Taking-Care-of-Your-Digestive-System/Enzymes. Accessed 4/26/2019.
  2. Fieker A, Philpott J, Armand M. Enzyme replacement therapy for pancreatic insufficiency: present and future. Clin Exp Gastroenterol. 2011;4:55–73. doi:10.2147/CEG.S17634.
  3. Cystic Fibrosis Foundation. Patient Registry Annual Data Report 2017. Available at: https://www.cff.org/Search.aspx?topic=230. Accessed 4/26/2019.