Enzymes and CF

Nutritional management is an important part of CF Care. Good nutrition in CF is associated with better lung function and survival. Pancreatic enzyme replacement is different for everyone, sometimes it’s tricky to get the right balance.

The increased energy requirements are a result of increased work of breathing, repeated bouts of infection and inflammation, as well as malabsorption. Malabsorption results in the loss of fat and fat soluble vitamins in bowel motions which can lead to malnutrition and poor growth.  This makes good nutrition a challenge for people with CF.

Digestion and the pancreas

In a normal situation the pancreas produces digestive juices (enzymes) that reach the intestine through small tubes called ducts. Enzymes break down the food so that it can be absorbed into our blood and transported to our organs, like muscle. Our body has different enzymes to break down different parts of the food we eat, including fat, protein and carbohydrate.

So, in summary, the functions of the pancreas is to:

• Produce enzymes that help break down the food we eat
• Control the amount of sugar in our blood by producing insulin

How does CF affect my pancreas?

In approximately 85% of people with CF the pancreas, and/or the duct is blocked with mucus, preventing normal food digestion.

This is referred to as pancreatic insufficiency. This situation prevents the enzymes from working on the food in the intestine, and means those who are pancreatic insufficient will require enzyme supplements to assist with the digestion of food. Without enzyme supplements poor nutrient absorption leads to poor growth, vitamin deficiency and weight loss.

Enzymes

People with CF who are pancreatic insufficient need to take enzymes (granules or capsules) to absorb important nutrients, mainly fat and fat-soluble vitamins, but also protein and carbohydrate.

In children the dose of enzymes will change as they grow and as their diet changes. Check regularly with the dietitian to review enzyme doses, including babies who are breast fed. It is important to manage enzyme replacement well so your child grows normally.

The most commonly used enzyme supplement in Australia for babies, children and adults with CF is Creon®, which comes in microsphere form for babies or as capsules. Panzytrat® is also available and works well for some individuals.

Enzyme Dose

The dose required will vary from person to person, as people with CF vary in their degree of pancreatic insufficiency. The Australian Guidelines recommend a dose for enzymes as a range between 4,000 – 500 units of lipase per gram of fat. So this range is between a very low dose and a very high dose, as can be seen on the right of the table below;

Although you may need to adjust your dose, it is recommended not to increase the dose without first discussing this with your doctor or dietitian for guidance. This is partly because of the need to avoid exceeding the ‘Safe Upper limit’. This limit was introduced after very high doses of enzymes used in children in the 1980’s was suspected to be involved with damage to the bowel called fibrosing colonopathy. As a result the ‘safe upper limit’ was introduced and since that time the problem has been avoided. The safe upper limit is 10,000 units per kilogram of body weight per day. Ask your dietitian if you are exceeding this amount.

Enzyme doses need to be monitored regularly and adjusted based on the different amounts of food and fluid consumed. Capsules and beads should not be crushed or chewed. Beads should be mixed with an acidic food (e.g. fruit puree, fruit gel, jam or tomato sauce) until capsules can be swallowed.

How do you know if there are digestion/malabsorption problems?

It is important to learn the warning signs that may indicate that you or your child has problems digesting their food.

Changes that you might notice or should monitor:

• Tummy pain
• Discomfort
• Excessive smelly wind
• Greasy, fatty poos that are sometimes difficult to flush
• Diarrhoea and/or constipation
• Hunger despite eating lots of food
• Poor weight gain, poor growth

Your dietitian and doctor will work together with you, to work out the best plan to improve your or your child’s absorption. This will help ensure optimal nutrition and weight gain.

Your dietitian will also be able to provide advice on the type of food to eat for a high energy diet that will promote normal nutrition, growth and weight gain.

Getting the balance right

Enzymes need to be taken before, or during eating and with nearly all food and fluids. They are not necessary for foods that contain mainly simple sugars. Some examples include:

• All fruit fresh, dried, canned
• Non – starchy salad vegetables
• Lollies, jellies, sorbet, Roll-ups
• Juice, cordial, soft drink, electrolyte replacement drinks, water

Remember that while enzymes are dosed according to how much fat is in food, enzymes work on fat, carbohydrate and protein. Therefore, they are still needed with foods that are lower in fat such as rice cakes, lean meat, baked beans and pasta. Sometimes enzymes may need to be spread out if it takes longer than half an hour to eat your meal e.g. when out to dinner or if your child regularly takes longer to finish their meal.

Talk to your CF dietitian if you have any digestion problems.

Article from RED Magazine, Edition 3, 2016.