Orkambi and fatty foods

Frage

Dear experts,

Our son (1 year old, homozygous f508del) has been taking Orkambi for 3 days. My husband and I were told that he needs to consume 10g of fat with the Orkambi. This is extremely difficult, especially in the evenings, because he simply doesn't have enough concentration to eat calmly. This, of course, causes him a lot of stress. I've tried to find the 10g fat requirement in the literature, but everywhere it just says "fatty food."

Does it really have to be 10g of fat?

Thank you for your help!

Antwort

Dear Family,

The current product information for Orkambi, as well as the approval data for children aged 1 to under 2 years, does not specify a minimum amount of 10 g of fat. It simply recommends taking Orkambi with a meal or snack containing fat.

This is because the absorption of the active ingredients lumacaftor and ivacaftor is significantly improved by fat compared to taking them on an empty stomach. However, pharmacokinetic studies do not support a specific minimum amount such as 10 g of fat.

A specific amount for this age group is recommended by the National CF Center at Oslo University Hospital in Norway. This is a clinical recommendation, not a minimum amount determined in a pharmacokinetic study. They state that approximately 5–8 g of fat is sufficient for children taking Orkambi.

Therefore, a tired one-year-old does not necessarily need to eat a full evening meal. A small, age-appropriate meal containing fat, or a suitable snack, is generally sufficient. Examples include 150 ml of whole milk, a full-fat natural yogurt, a small slice of whole-grain bread with butter, an egg, or a tablespoon of nut butter. The required amount depends on the fat content of the food.

If your CF center recommended 10 g of fat while taking your modulator, I would ask you to discuss this again with your healthcare team. It would be particularly helpful to know the source of this specific 10 g fat recommendation. This specific guideline is not included in the current prescribing information or the approval data for this age group.

Best regards,

Suzanne van Dullemen

12.10.2026