Enzymes

Just as the lungs produce thick, sticky mucus, the pancreas also makes thick mucus that blocks the release of enzymes needed for digestion. Most people with cystic fibrosis need to take enzymes before they eat.

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Summary
  • Taken by mouth, the enzymes go to work in the intestines so you can digest food and absorb the nutrients to keep your body healthy.
  • It is important that you take the right amount of enzymes, so check with your doctor, nurse or dietitian on your CF care team for the exact amount of enzymes to take.

What Are Enzymes and How Do They Work?

Most pancreatic enzyme supplements come in capsule form. Inside each capsule are many small beads that contain the digestive enzymes. Each bead is covered with a special enteric coating. This coating allows the beads to dissolve in the small intestine. The digestive enzymes are then released in the small intestine to help digest food. Enzymes work for about 45 to 60 minutes after taking them.

Enzymes work by helping you to:

  • Digest carbohydrates, proteins and fats (the three nutrients in food that supply calories).
  • Gain and maintain a healthy weight.
  • Absorb essential nutrients such as vitamins and minerals.

In the short term, not taking your enzymes or not taking the proper dose can lead to poorly digested fat, protein or starch. The poorly digested food sits in your intestines, which causes gas, pain and unpleasant smells. It can also cause problems ranging from constipation and DIOS to loose, floating, greasy, frequent stools.

In the long term, better lung function is associated with higher body weight, so it is very important to take enzymes with all meals and snacks. If you have trouble paying for enzymes or have questions about coverage of them, please contact CF Foundation Compass, which can connect you to resources that can assist you.

To contact Compass, call 844-COMPASS (844-266-7277) Monday through Friday, 9 a.m. until 7 p.m. ET, or email compass@cff.org.

A 2016 study showed that if a parent caregiver has depression, their children with CF are less likely to take their enzymes. If you have a child with CF and you are experiencing symptoms of depression, please talk to your health care provider about treatment.

How to Take Enzymes

Most people with CF need to take pancreatic enzyme capsules before every meal and snack so their bodies can digest the nutrients. Meals and snacks include breast milk, formula, milk and nutritional supplements. People with CF should take enzymes with any food, unless it is pure sugar (such as a clear Popsicle, hard candy or fruit juice).

Older children and adults should take the capsules with liquid and swallow them whole. If you or your child are not able to swallow the capsules, open them and sprinkle the beads on a small amount of an acidic food, such as applesauce, that you can swallow. Don't crush or chew the enzyme beads. They won't work as well.

If meals are longer than 30 minutes, some people split their enzyme dose, taking half at the start of the meal and the other half partway through the meal.

The side of your enzyme bottle lists the amount of lipase (to digest fat), protease (to digest protein) and amylase (to digest starch) in the enzyme. The number that comes after the name of your enzyme capsule is the amount of lipase units per capsule. Because fat is the hardest thing for the body to digest, dosing guidelines primarily take into account the amount of lipase in the enzyme.

Many people have a fixed dose -- for example, a certain number of capsules with each meal and a smaller number with a snack. Others increase their dose if they eat a meal that has lots of fat in it, such as a pepperoni pizza or chicken wings in blue cheese sauce. Tell your CF dietitian or care provider if high-fat foods cause problems. 

As you get older or start to eat more, you will have to increase the amount of enzymes. If you have any questions about how many enzymes to take, talk to the dietitian, doctor or nurse on your care team. Taking too many enzyme supplements can actually damage your intestines, but taking too few can keep you from absorbing the nutrients you need. Do not change the dose without talking with your CF care team.

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Tips:

  • Enzyme brands are not interchangeable. The products vary slightly in the amounts of enzymes and the way the coating on the enzyme beads dissolves.
  • Avoid skipping enzymes. Keep a supply of enzymes with you in case you want to eat meals or snacks while away from home.
  • If you're supplementing your diet with tube feedings, talk to your health care team about the best way to incorporate enzymes into your tube feeding plan, as there are many different approaches to take.

Storing Your Enzymes

Get your enzymes ready once a week rather than as needed. This is also a great way to monitor whether you've taken them and if you need a refill soon.

https://www.instagram.com/p/BCssu7Jvxjy/
If necessary, use three or more pill cases (morning, afternoon, evening) and keep them where you will remember to take your meds -- for example, on the bedside table for morning and on the kitchen counter for evening.

Pour the enzymes you think you need for the week in larger containers and keep them in accessible places (on the dinner table or in your purse or book bag). Consider leaving a bottle at a relative's or friend's house.

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When brown-bagging it for lunch, include enzymes in the bag so no effort is needed to search for them. If your child is still in school, talk to the school nurse about how he or she can best keep and take enzymes, as some schools do not allow children to carry them on their own.

If you are anticipating a hospital stay, ask your care team how enzymes will be handled during your stay. If the enzymes are not on the hospital's formulary (internal stock), you may need to bring your enzymes from home.

  • Keep enzymes at room temperature (59˚ to 86˚F). Keep enzymes away from heat. Don't store them in places such as on top of a toaster oven or inside a hot car. Heat will destroy the activity of the enzymes.
  • Do not refrigerate enzymes.
  • Enzymes have an expiration date. Check the expiration date on each bottle to make sure they are fresh.
  • Always keep the lid tightly sealed on the bottle of enzymes.
  • Ask the pharmacist to give you the enzymes in the original, sealed, unopened bottle from the manufacturer.

Helping Infants and Young Children Take Their Enzymes

For infants and small children who need the capsules opened up, mix the beads with a soft, acidic food, such as applesauce. Avoid mixing enzymes with milk-based foods, like pudding, because they may damage the coating on the beads.

Some very young babies may spit out the beads. If this happens, gently scoop the bead mixture back into the baby's mouth until the entire dose of enzymes has been given. It may be helpful to offer breast milk or formula just after giving the beads.

Toddlers may refuse to take enzymes as they become more independent. If this occurs, try giving your toddler acceptable choices such as, “Would you like to take your enzymes in applesauce or pears today?” Do not allow them to eat if they refuse to take enzymes, as this will lead to bad eating habits.

It may take time for your child to learn how to swallow beads because of the new texture. Enzymes do not have a taste. Be patient, calm and reassuring to your child. Call your CF dietitian on your care team if your baby is having trouble taking enzymes.

You can learn more about enzymes at DailyMed, which is a service from the National Library of Medicine that provides information about drugs, including possible side effects. You can learn more about specific enzymes by searching for the medication from the main DailyMed page. Please note that information about dosages apply to the general public and not necessarily to people with CF. For specific dosing information, talk to your CF care team.

Signs of Malabsorption

Between 85 to 90 percent of individuals with CF have pancreatic insufficiency, which means that digestive enzymes are getting stuck in thick mucus in the pancreas and can't make it into the small intestine.1 With no enzymes to break down food, much of the protein, fat and carbohydrate in food is not absorbed for use in the body. This is called malabsorption.

Your or your child's CF care team can determine if you or your child has pancreatic insufficiency by using a fecal elastase test. In this test, doctors analyze a stool sample to see whether the pancreas is producing an enzyme to break down proteins.

Malabsorption of proteins and fats can lead to poor growth and malnutrition. Proteins are needed for growth and body tissue repair or healing. Fats are calorie-rich food sources and give the energy needed for growth and development, and to stay healthy. Fat is also needed for absorption of some vitamins and minerals.

For some people with CF, the mucus that lubricates the intestines is so thick and sticky that it may block the intestines. A blocked intestine needs special treatment, which is why it is important to talk to your care team if you notice symptoms of bloating, pain, gas or malabsorption.

People with CF who have not yet started taking enzymes may have any or all of the following symptoms of malabsorption:

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

You'll usually notice improvements once you start taking enzymes. If you take enzymes and still experience some of these symptoms, it could mean the dose or type of enzymes you've been prescribed needs to be adjusted. Do not increase or decrease the dose of enzymes without talking to your CF dietitian or care provider.

Foods That Do Not Need Enzymes

Pancreatic enzyme replacements contain enzymes that digest fat, protein and complex carbohydrates. Some foods and drinks do not require enzymes because they contain only simple carbohydrates that are digested and absorbed easily.

Here are examples of foods and drinks that do not require enzymes:

  • Fruits.
  • Juice, juice drinks.
  • Soft drinks, sports drinks.
  • Infant rehydrating formulas (like Pedialyte®).
  • Tea, coffee (without cream).
  • Hard candy (like lollipops).
  • Fruit snacks.
  • Jelly beans.
  • Gum.
  • Popsicles, freezer pops, flavored ice.

Some young children still need to take enzymes when eating these foods so they stay in the habit of always taking enzymes with food. If they learn that certain foods don't require enzymes, they may only want to eat those foods.

 

Except for fruits and some fruit juices, there is little nutritional value in most of the foods and drinks listed above. Thus, it is not recommended to consume these regularly or in large amounts. Ask your CF dietitian or care provider if you are unsure whether enzymes need to be taken with a certain food, or if your child is refusing to take enzymes.

***

Reference to any specific product, process or service does not necessarily constitute or imply its endorsement, recommendation or favoring by the Cystic Fibrosis Foundation. The appearance of external hyperlinks does not constitute endorsement by the Cystic Fibrosis Foundation of the linked websites, or the information, products or services contained therein.

Information contained on this site does not cover all possible uses, actions, precautions, side effects or interactions. This site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.

FDA-approved drug information is available at www.dailymed.nlm.nih.gov/dailymed.

REFERENCES

Quick, Virginia, Byrd-Bredbenner, Carol. Disordered Eating and Body Image in Cystic Fibrosis. In: Diet and Exercise in Cystic Fibrosis. San Diego, CA: Academic Press; 2015:11-12.

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