Nutritional support for adult сystic fibrosis patients receiving targeted treatment

Abstract

Cystic fibrosis (CF) is characterized by the development of a severe nutritional deficiency. A low BMI directly correlates with low lung function and requires active nutritional support. Pathogenetic (targeted) therapy aimed at restoring the chlorine channel function also leads to weight gain. The effects of CFTR modulators on extrapulmonary pathology in adult CF patients in Russia have been described very little.Aim. To evaluate the sequential impact of two targeted drugs – the potentiator ivacaftor and the triple combination of CF transmembrane regulator modulators elexacaftor/tezacaftor/ivacaftor – on the nutritional status of an adult patient with cystic fibrosis receiving nutritional support.Conclusion. Therapy with CFTR modulator in combination with sipping nutritional support promotes significant weight gain in adult CF patients. The triple combination of elexacaftor/tezacaftor/ivacaftor has a more active effect on nutritional status than ivacaftor alone. The targeted therapy requires supervision by a nutritionist.