Correlates of Pancreatic Enzyme Replacement Therapy Intake in Adults with Cystic Fibrosis: Results of a Cross-Sectional Study

Abstract

Most people with cystic fibrosis (pwCF) develop pancreatic insufficiency and are treated with pancreatic enzyme replacement therapy (PERT). We aimed to describe the use of PERT and assess the correlates of PERT dose in adult pwCF. In a cross-sectional study at the Copenhagen CF Centre, the participants reported PERT intake, gastrointestinal (GI) symptoms and the use of concomitant treatments. Demographic and clinical characteristics were extracted from the Danish CF Registry. We used linear regression to assess the correlates of PERT dose per kg bodyweight (U-lipase/kg). We included 120 pwCF with a median age of 32.9 years, 46% women and 72% F508delta homozygote. The PERT dose ranged from 0 to 6160 U-lipase/kg per main meal (mean 1828; SD 1115). The PERT dose was associated with participants’ sex (men vs. women: 661; 95% CI: 302; 1020 U-lipase/kg), age (−16; 95% CI: −31; −1 U-lipase/kg per year) and weight (−45; 95% CI: −58; −31 U-lipase/kg per kg). Having less frequent constipation and being lung transplanted were also associated with a higher PERT dose. A third of participants did not take PERT for snacks, and this was associated with the frequency of diarrhoea. These findings indicate that PERT intake may be improved to reduce GI symptoms.

Diagnosis and Management of Gastrointestinal Manifestations in Children with Cystic Fibrosis

“…Effective enzyme-replacement therapy may enhance lipid absorption [74,88]. Despite treatment, lipid malabsorption persists in some cases, with absorption coefficients below 85-90% of dietary lipid intake [79].…”