Association of In-Line Digestive Enzyme Cartridge with Enteral Feeds on Anthropometrics among Pediatric Patients with Cystic Fibrosis

Introduction: In pediatric patients with cystic fibrosis (CF), adequate caloric intake is associated with better lung function and decreased mortality. Sufficient caloric intake can be difficult as CF results in exocrine pancreatic insufficiency (EPI). Pancreatic enzyme replacement therapy (PERT) can be taken with meals to ameliorate this issue. However, for the 9% of people with CF who use continuous nighttime enteral tube feeds to reach calorie goals, oral PERT is insufficient as it only lasts about 45 minutes. An in-line lipase cartridge (ILC) is available to continuously deliver PERT with enteral tube feedings. ILC outcomes are limited in literature. This project evaluated our single-center experience to add to the literature describing the benefits and limitations of ILC among pediatric patients with CF. Methods: Retrospective chart review was performed on 29 pediatric patients with CF and EPI who received supplemental tube feedings and used ILC for a minimum of 3 months from 2015 to 2019. Anthropometrics were evaluated 12 months before and after initiation of ILC. Results: Weight, height, and BMI z-score changed over time based on multivariable longitudinal regression models after adjusting for relevant variables identified a priori, including age at start of ILC, sex, PERT dose with GT feeds prior to ILC, PERT with GT feeds after starting ILC, and CF gene mutations. Compared to mean height z-score at 6-months-before-ILC, mean height z-score at 6-months-after-ILC (adjusted mean difference=0.2540; 95% CI=[0.0487, 0.4592]; p=0.0153) and mean height z-score at 12-months-after-Relizorb (adjusted mean difference = 0.2684; 95% CI=[0.0203, 0.5166]; p=0.0340) were significantly higher. Mean weight z-score at 12-months-after-ILC neared statistical significance when compared to 6-months-before-ILC (adjusted mean difference=0.2816; 95% CI=[-0.0003, 0.5634]; p=0.0502) when excluding 7 patients with the most advanced lung disease (FEV1 < 50). Weight-for-length or BMI at 12-months-after-ILC did not significantly differ when compared to before ILC but did trend towards a slight decrease. Conclusion: The use of ILC with enteral tube feeds significantly improved height in pediatric patients with CF over usual management. The trend towards a decrease in weight-for-length or BMI may be due to an increase in height preceding an increase in weight. ILC promises to improve caloric intake hopefully leading to associated benefits (see Table 1). Table 1. - Descriptive characteristics of study participants Variable (Subject-Specific) All (n=29) Age at start of ILC, mean (SD) 8.41 (5.00) Male sex, n (%) 15 (51.72%) PERT dose with GT feeds prior to ILC (kg/feed), mean (SD) 1934.34 (426.76) Continue oral PERT with GT feeds after starting ILC, n (%) 13 (44.83%) CF Mutations, n (%) dF508 homozygous 12 (41.38%) dF508 heterozygous 15 (51.72%) Other 2 (6.90%)