Energy cost of activity and exercise in children and adolescents with cystic fibrosis

Mark R. Johnson, Thomas W. Ferkol, Ross W. Shepherd

Research output: Contribution to journalArticlepeer-review

9 Scopus citations


In cystic fibrosis (CF), perturbations of total daily energy expenditure (TDEE) may be a major determinant of altered nutrition and growth. Measurement of TDEE is problematic, though the flex-heart rate method (FHRM) provides a close estimation of TDEE, as compared to the cost-prohibitive, gold standard, the double-labeled water method, and permits estimates of the energy cost of daily activities (ECA) above resting energy expenditure (REE). We hypothesize that alterations in ECA affects TDEE in CF. Purpose: To measure components of TDEE in adolescents with CF and normal lung function compared with controls, and to determine whether ECA can be improved by diet and exercise. Methods: Clinically stable CF subjects (aged 9-13, n=12) and age-and gender-matched controls (n=13) had repeated measurements of TDEE by FHRM, REE, and maximal cardiopulmonary exercise testing (CPET) during a 6-week exercise and diet program. Results: While the mean REE was similar in both groups, ECA was significantly lower in CF adolescents as compared to controls (p=0.02). During CPET, maximal exercise in CF was characterized by hyperventilation, which was unrelated to ventilation-perfusion mismatching. There were no changes in REE after dietary intervention. Conclusion: ECA in CF adolescents with normal lung function is lower when compared to healthy controls. These findings support the hypothesis that clinically stable patients with CF have inefficient energy metabolism or alternatively conserve energy during activities of daily living.

Original languageEnglish
Pages (from-to)53-58
Number of pages6
JournalJournal of Cystic Fibrosis
Issue number1
StatePublished - Jan 2006


  • Cardiopulmonary exercise testing
  • Cystic fibrosis
  • Energy expenditure


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