Optimizing nutritional strategies for cystic fibrosis pediatric patients ineligible for CFTR modulators

<bold>Introduction:</bold> Cystic Fibrosis (CF) is associated with compromised nutritional status. Left untreated, CF-related undernutrition can cause severe consequences. Thus, early nutritional intervention, commencing in the formative years, is advocated. In 2021, data from the CF registry at King Fahad Medical City (KFMC) revealed that approximately 40% of children were below the 3rd percentile for BMI. In response, we devised an intensive intervention plan, addressing every aspect that could affect weight gain, and closely monitored the outcomes. <bold>Methods:</bold> We prospectively followed patients from 2021 to 2023 currently enrolled in our program, documenting their data every three months. Parameters included age, BMI, weight, caloric intake, and PERT dosing. We tracked patients prescribed appetite stimulants or overnight GT feeding. Additionally, we monitored factors influencing weight gain, such as CFRD, celiac disease, and abnormal eating behavior. <bold>Results:</bold> We monitored a cohort of 60 CF patients who were ineligible for CFTR modulators. The mean age of the group was 6.91 years, with a female-to-male ratio of 27:33. Our data revealed a significant improvement in our primary target: the percentage of CF patients with a BMI above the 3rd percentile for age and sex increased from 60% in 2021 to 73.3% in 2022 and reached 83.3% in 2023. During our intervention, we implemented overnight GT feeding in 10 patients (16.67%) and utilized appetite stimulants in 14 patients (23.3%). The latter intervention demonstrated a success rate of 71.4%. <bold>Conclusion:</bold> Continuous education for patients and their families is crucial for enhancing compliance. Effective communication within the multidisciplinary team is vital.