Clinical nutrition ESPEN · 2025
Assessment of dietary intake in adults with cystic fibrosis pre and post commencement of Elexacaftor/Tezacaftor/Ivacaftor.
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Loel Felicity, Keith Megan, Oliver Wenonah, Herd Karen, Hanna Katherine, Matson Angela
Аннотация
BACKGROUND & AIMS: Elexacaftor, Tezacaftor, Ivacaftor has enhanced clinical outcomes, expected lifespan and quality of life for people with cystic fibrosis. Increased body mass index post- Elexacaftor, Tezacaftor, Ivacaftor is well documented, suggesting high-energy, high-fat recommendations may no longer be appropriate. This study aims to identify changes in dietary intake and nutritional parameters post gene modulator therapy Elexacaftor, Tezacaftor, Ivacaftor.
METHODS: A retrospective cohort study assessed diet and nutritional parameters of adults with cystic fibrosis (n = 40) pre- and post- Elexacaftor, Tezacaftor, Ivacaftor (9 ± 3 months). Dietary intake was analysed (24hr recalls), for total energy intake, macronutrient distribution, sodium, core food groups, and discretionary intakes being compared to Nutrition Guidelines for people with cystic fibrosis, and general population guidelines.
RESULTS: Total energy intake decreased from 139.3 to 116.6 kJ/kg/day (p = 0.012) and was below the estimated energy requirement for people with cystic fibrosis by 4457.3 kJ (p < 0.001), despite body mass index increasing (p=<0.001). Mean dietary sodium decreased from 3921.1 mg ± 1364.5 mg to 3517.1 mg ± 1251.7 mg (p = 0.028). Intake of protein was above, and total fat intake met the higher end of guidelines for people with cystic fibrosis. Saturated fat intake did not change (p = 0.403) however exceeded general guidelines of <10 % total energy intake in line with general population intake of 15.9 %. A decrease in discretionary foods (5.1-4.5 serves/d, p = 0.038) and increase in vegetable intake (interquartile range 1.0-2.9 to 1.5-3.7 serves/d, p = 0.036) was noted. Serum retinol levels increased from 1.7 μmol/L to 2.0 μmol/L (p = 0.003).
CONCLUSION: Dietary patterns of people with cystic fibrosis change following Elexacaftor, Tezacaftor, Ivacaftor commencement. While total energy intake decreased and vegetable intake increased, saturated fat and discretionary intake remained high, increasing risk of metabolic disease. An individualised approach is needed with modulator therapy and recognition that changing diet is a modifiable risk factor for chronic disease.
METHODS: A retrospective cohort study assessed diet and nutritional parameters of adults with cystic fibrosis (n = 40) pre- and post- Elexacaftor, Tezacaftor, Ivacaftor (9 ± 3 months). Dietary intake was analysed (24hr recalls), for total energy intake, macronutrient distribution, sodium, core food groups, and discretionary intakes being compared to Nutrition Guidelines for people with cystic fibrosis, and general population guidelines.
RESULTS: Total energy intake decreased from 139.3 to 116.6 kJ/kg/day (p = 0.012) and was below the estimated energy requirement for people with cystic fibrosis by 4457.3 kJ (p < 0.001), despite body mass index increasing (p=<0.001). Mean dietary sodium decreased from 3921.1 mg ± 1364.5 mg to 3517.1 mg ± 1251.7 mg (p = 0.028). Intake of protein was above, and total fat intake met the higher end of guidelines for people with cystic fibrosis. Saturated fat intake did not change (p = 0.403) however exceeded general guidelines of <10 % total energy intake in line with general population intake of 15.9 %. A decrease in discretionary foods (5.1-4.5 serves/d, p = 0.038) and increase in vegetable intake (interquartile range 1.0-2.9 to 1.5-3.7 serves/d, p = 0.036) was noted. Serum retinol levels increased from 1.7 μmol/L to 2.0 μmol/L (p = 0.003).
CONCLUSION: Dietary patterns of people with cystic fibrosis change following Elexacaftor, Tezacaftor, Ivacaftor commencement. While total energy intake decreased and vegetable intake increased, saturated fat and discretionary intake remained high, increasing risk of metabolic disease. An individualised approach is needed with modulator therapy and recognition that changing diet is a modifiable risk factor for chronic disease.