First-author, Max C. Petersen, MD, and collaborators (Lauren Begnel, RDN, LD; Michael Wallendorf, PhD; and Marina Litivin, MD) had their article, “Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis,” published in the Journal of Cystic Fibrosis.
The combination cystic fibrosis transmembrane regulator (CFTR) modulator therapy elexacaftor-tezacaftor-ivacaftor was approved by the FDA in 2019.
The authors conducted an observational analysis of the effect of elexacaftor-tezacaftor-ivacaftor on body weight and cardiometabolic parameters in 134 adult CF patients of the Washington University Adult Cystic Fibrosis Center.
In this observational study of 134 adults with CF, initiation of elexacaftor-tezacaftor-ivacaftor was associated with increases in BMI at a mean follow up of 12.2 months. Changes in other cardiometabolic risk factors were also observed. Widespread use of elexacaftor-tezacaftor-ivacaftor may be expected to increase the incidence of overnutrition in the CF population.
The authors stated, “Overall, our data suggest that the approval and widespread use of elexacaftor-tezacaftor-ivacaftor should be expected to accelerate the trend toward increased weight and BMI in adults with CF that has emerged and progressed over the last 20 years. As we demonstrated in our patient cohort, patients with CF on elexacaftor-tezacaftor-ivacaftor are at risk of developing hyperlipidemia and hypertension. Given the increasing median age of patients living with CF, and the increased likelihood of developing CFRD with age, the rising prevalence of overnutrition, hypertension, and hyperlipidemia in the CF population is likely to increase the incidence of cardiovascular and cerebrovascular disease, conditions for which patients with CF have been at low risk previously. Acknowledging this changing paradigm, recent guidelines from the Academy of Nutrition and Dietetics highlight the increased prevalence of overweight and obesity in the CF population and recommend use of dietary patterns associated with cardiovascular health in the general population, rather than traditional high-fat, high-energy diets . Importantly, as elexacaftor-tezacaftor-ivacaftor use becomes the standard of care in pediatric patients with CF, obesity and its metabolic consequences will be of paramount importance.
In conclusion, elexacaftor-tezacaftor-ivacaftor use is contributing to the changing landscape of nutrition in CF. In addition to providing nutritional support services to identify and treat patients who are underweight, clinicians should monitor patients with CF for evidence of overnutrition and related complications. Caloric, nutrition and fitness goals should be reassessed and individualized at every visit, and blood pressure and lipids should be closely monitored.”