Italian and North American dietary intake after ivacaftor treatment for Cystic Fibrosis Gating Mutations

J Cyst Fibros. 2019 Jan;18(1):135-143. doi: 10.1016/j.jcf.2018.06.004. Epub 2018 Jul 6.

Abstract

Background: In patients with cystic fibrosis (CF), ivacaftor treatment results in significant weight gain and the impact on diet has not been explored.

Methods: A study in 22 subjects (6.1-61.6 years) compared diet, energy balance, weight gain, and body composition, before and after three months of treatment in Italians and North Americans with CFTR gating mutations.

Results: With no differences between groups in energy or macronutrient intake at baseline, fat intake increased in all subjects, and both fat and energy intake increased in Italians. Height, weight, BMI, lean and fat mass, and % body fat increased and resting energy expenditure decreased after treatment. Weight gain was associated with energy and fat intake.

Conclusions: Fat intake increased with treatment, possibly due to the recommendation to take ivacaftor with high fat meals. Increased energy and fat intake correlated with weight gain. Regional dietary patterns differed.

Trial registration: ClinicalTrials.gov NCT02141464.

Keywords: Cystic fibrosis; Diet; Ivacaftor.

Publication types

  • Multicenter Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aminophenols / therapeutic use*
  • Child
  • Child, Preschool
  • Chloride Channel Agonists / therapeutic use
  • Cystic Fibrosis / epidemiology
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / therapy*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism
  • DNA / genetics
  • DNA Mutational Analysis
  • Diet / methods*
  • Dietary Supplements*
  • Energy Intake
  • Energy Metabolism / physiology*
  • Female
  • Humans
  • Italy / epidemiology
  • Male
  • Middle Aged
  • Morbidity / trends
  • Mutation*
  • North America / epidemiology
  • Quinolones / therapeutic use*
  • Young Adult

Substances

  • Aminophenols
  • Chloride Channel Agonists
  • Quinolones
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • ivacaftor
  • DNA

Associated data

  • ClinicalTrials.gov/NCT02141464