Swedish study tracks gastrointestinal changes in children receiving ETI for cystic fibrosis

This prospective study at three Swedish paediatric cystic fibrosis centres assessed children aged 6–17 years who started ETI. Sixty-two participants, with a mean age of 11.7 years, completed assessments at baseline, six months and 12 months. Outcomes included gastrointestinal symptoms, digestive health-related quality of life, nutritional status, pancreatic enzyme replacement therapy (PERT) use and faecal elastase.

The mean combined number of reported gastrointestinal symptoms fell from 1.5 at baseline to 0.9 at 12 months (p=0.005). No individual symptom changed significantly. The CFQ-R Digestive score improved by 7.8 points (p=0.002), and PERT dose decreased by 1.3 × 10³ units/kg/day (p<0.001). BMI z-scores and faecal elastase did not change.

The findings describe improvements in reported digestive symptoms and quality of life during the first year of ETI, alongside lower PERT use. However, unchanged faecal elastase and growth measures indicate that the study did not demonstrate improved pancreatic function or nutritional status. The results are limited to children treated at three Swedish centres and do not establish whether, or how, PERT should be adjusted in individual patients.