Enzymes in cystic fibrosis 

Digestive enzymes are made in the pancreas. The fat, protein and carbohydrate in food is broken down by the enzymes to release nutrients. „„In cystic fibrosis (CF) sticky mucus blocks the passages from the pancreas to the small intestine which stops the enzymes working, so the food cannot be digested or absorbed by the body. This is called malabsorption and causes loose or oily stools, wind, stomach ache and poor weight gain.

How do enzymes work in CF?

„„There are several brands of enzymes which come in a capsule for example, Creon® and Nutrizym® „„Beads inside the capsule contain digestive enzymes. The outer capsule dissolves in the stomach. The beads then move along into the small intestine where the coating dissolves releasing the enzymes. Food is then broken down and the nutrients are absorbed by the body.

When should enzymes be taken?

„„All food and drinks containing fat, including nutritional supplements, require enzymes. „„Enzymes need to be in the stomach at the same time as food. „„Enzymes should be taken at the start of a meal or snack. If you are unsure how much you will eat or the meal has several courses, the dose can be split „„If you forget to take enzymes at the start of a meal, it is not too late to take them at the end of the meal.

Compiled by: 
The Cystic Fibrosis Team in collaboration with the Child and Family Information Group
Last review date: 
February 2014
Ref: 
2013F1415

Disclaimer

Please read this information sheet from GOSH alongside the patient information leaflet (PIL) provided by the manufacturer. If you do not have a copy of the manufacturer’s patient information leaflet please talk to your pharmacist. A few products do not have a marketing authorisation (licence) as a medicine and therefore there is no PIL.

For children in particular, there may be conflicts of information between the manufacturer’s patient information leaflet (PIL) and guidance provided by GOSH and other healthcare providers. For example, some manufacturers may recommend, in the patient information leaflet, that a medicine is not given to children aged under 12 years. In most cases, this is because the manufacturer will recruit adults to clinical trials in the first instance and therefore the initial marketing authorisation (licence) only covers adults and older children.  

For new medicines, the manufacturer then has to recruit children and newborns into trials (unless the medicine is not going to be used in children and newborns) and subsequently amend the PIL with the approved information. Older medicines may have been used effectively for many years in children without problems but the manufacturer has not been required to collect data and amend the licence. This does not mean that it is unsafe for children and young people to be prescribed such a medicine ‘off-licence/off-label’. However, if you are concerned about any conflicts of information, please discuss with your doctor, nurse or pharmacist.