Mucoviscidosis (CF)

Clinical picture

In patients with cystic fibrosis, the pancreas produces very few to no digestive enzymes. This means that the body cannot take in enough nutrients, vitamins and minerals. To remedy this deficiency, there are drugs available containing a mixture of different digestive enzymes that work to break down nutrients, lipids, carbohydrates and proteins. The dosage depends primarily on the fat content of the food consumed. This makes it essential to be aware of the fat content of these foods in order for the right dosage of digestive enzymes to be provided.


It has been proven that healthy, age-appropriate nutrition has a positive influence on the progression of cystic fibrosis in patients. For families with affected children, the subject of eating is often a major issue. However, it is essential for the parents to tackle this subject with their child every day. In time it will become routine.

Pancreatic enzyme preparations

Pancreatic enzyme preparations contain the three enzymes lipase, amylase and protease, which are required to break down the three nutrients fats, carbohydrates and proteins. The pancreatic enzyme preparation dosage depends solely on the lipase content. According to the ESPEN-ESPGHAN-ECFS guidelines, around 500 to 1,000 units of lipase are required on average for infants and about 2,000 to 4,000 units of lipase are required for children and adults in order to break down just 1 gram of fat. The maximum daily dose for all age groups is 10,000 lipase units per kilogram of body weight.

Enzyme preparations are available in different particle sizes and dosages:

Micro-pellets of 5,000 lipase units
Capsules of 10,000 lipase units
Capsules of 20,000 lipase units
Capsules of 25,000 lipase units
Capsules of 40,000 lipase units

You should discuss the dosage beforehand with the child’s doctor and nutritionist.

Tips on how to ensure your child receives the right nutrition

Despite how important it is and the care that you need to take, food should be fun, too. A natural, shared approach helps to reduce resistance.

Increase your child’s interest in what they are eating by putting a playful spin on the topic of nutrition.
Ask your child to help you in the kitchen.
Bringing up food as a topic for discussion doesn’t have to be out of the ordinary – many families do it.
Rules and rituals help to increase acceptance.
Do not cook just for your child and eat together as a family if possible.
Your child has to learn how to judge food for themselves. Give them responsibility in accordance with their age.
Praise positive behaviour.
From time to time, ask your child what they would really like to eat.