The pulmonary function of patients with cystic fibrosis (CF) is associated with nutritional status not only expressed as body mass index (BMI) but also as fat-free mass index (FFMI). This study evaluated the effect of a residential rehabilitation program on nutritional status (BMI, FFMI). The rehabilitation program provided supervised respiratory and nutritional treatment and daily physical activity for 3 weeks (median stay 20 (19-25) days). At the start and the end of the program, weight, height, pulmonary function, and body composition using dual-energy X-ray absorptiometry were prospectively collected. Supervised weighed food records were obtained, and physical activity intensity was measured using a SenseWear Pro3 Armband. CF-related complications were collected from the patient. Thirty-four patients (21 males, median age 18 years old (12-27)) were included. The diet contained a median of 30 EN% fat, 16 EN% protein, and 52 EN% of carbohydrates. A significant median weight gain (+1.45 kg (0.58; 2.6) (p < 0.0001) and a significant increase in BMI (+0.24 kg/m2 (0.11; 0.38)) (p < 0.0001), FFMI (+0.26 kg/m2 (0.01; 0.55)) (p < 0.0001), and FMI (+0.19 kg/m2 (0.04; 0.41)) (p < 0.0001) were obtained.Conclusion: A short rehabilitation program in individuals with CF between 6 and 40 years old is able to improve nutritional status and body composition.Trial registration: NCT04527796 What is Known: • Fat-free mass depletion is frequently present in CF. • In CF pulmonary function is associated with nutritional status measured as body mass index but also fat-free mass index. What is New: • Nutritional status and body composition improve significantly after a short-term rehabilitation program. • The rehabilitation program was able to improve nutritional outcome even with a diet containing less fat than currently advised in the guidelines.
Keywords: Body composition; Cystic fibrosis; Nutritional status; Physical exercise; Rehabilitation program.