WS12.04Vitamin levels in children with cystic fibrosis – the effect of elexacaftor/tezacaftor/ivacaftor

Objectives

In cystic fibrosis (CF), pancreatic insufficiency leads to malabsorption of fat-soluble vitamins A, D, E and K, and extra supplementation with these vitamins is recommended. The introduction of effective modulator treatment in CF have improved nutritional status of children with CF (cwCF). Although pancreatic destruction cannot be reversed, gut health is improved due to a decrease in inflammation, and studies have shown improvement in vitamin levels. We speculated that blood levels of vitamins A, D and E might have improved to a level that the daily vitamin dose had been decreased after up to 2 years of treatment with Elexacaftor-Tezacaftor-Ivacaftor (ETI) in a cohort of cwCF in Southern Sweden.

Methods

All cwCF followed in our centre were evaluated for inclusion in our study. Diagnosis was based on classical clinical symptoms and genotype. Only children treated with ETI for more than 1 year were included. Vitamin levels were measured before start of ETI, after 1 year and 2 years, and dose of daily vitamin supplementation (DEKAs) was recorded at the same time points.

Results

Forty cwCF (29 homozygous F508, 10 heterozygous, 1 with other mutations) were included. Mean age was 11.2 years (range 4.6 – 16.3 y) when ETI was introduced. Levels of vitamins A and E increased steadily after introducing ETI. Vitamin D level increased slightly during the first year, then went back to baseline. Dose of vitamin supplementation did not change. (Table 1). We speculate that compliance might have decreased due to the cwCF feeling better in general when treated with ETI.

Conclusion

Introduction of ETI may improve vitamin levels in cwCF, but studies should include compliance levels.