Malnutrition, fat malabsorption/vitamin D deficiency, and pulmonary decline traditionally characterize cystic fibrosis (CF). Fortunately, >90% of patients with CF ≥ 6 yrs old are now eligible for exacaftor/tezacaftor/ivacaftor (ETI) therapy which vastly improves lung function and nutrition by partially correcting the CF mutation. However, overweight/obesity is a rising concern. Although previously rare conditions of high blood pressure (BP) and dyslipidemia are also emerging in adults, pediatric CF data is limited. Moreover, data is lacking in minoritized groups such as Hispanics, who in the non-CF population are more prone to obesity when compared to non-Hispanic whites (NHW). To evaluate the impact of ethnicity on changes in body mass index (BMI) and obesity-related conditions, we performed a retrospective chart review of patients ≤18yrs old treated with ETI for at least 12 mo at our CF Center. Demographics including self-reported ethnicity (Hispanic vs NHW) and BMI, BP, A1c, liver profile, Vitamin D, lipids, and lung function (FEV1) at baseline and 12 mo post-ETI initiation were extracted from the medical record. Thirty-five children with CF [54% Hispanic, 57% male, age 13±3 yrs] were assessed. At baseline, Hispanics were more likely to have Medicaid, higher FEV1, and lower Vitamin D (all p<0.05). Nine (26%) children [7 Hispanic, 2 NHW] were overweight/obese. Post-ETI, BMI and FEV1 (both p<0.001) increased similarly in Hispanics and NHW, and Vitamin D improved in Hispanics (interaction, p<0.05). Lipid data was limited to 3 patients, therefore excluded from analyses. Post-ETI, 13 (37%) children were overweight/obese [8 Hispanic, 5 NHW]. Overweight/obesity is a rising complication in our ethnically diverse CF center. Both Hispanic and NHW children experienced increases in BMI after 12 mo of ETI therapy. Obesity research is imperative to inform CF nutrition guidelines and obesity-related screening (i.e., lipids) for children receiving ETI therapy.
K.Vavrina: Speaker's Bureau; AbbVie Inc., Chiesi USA, Inc., Alcresta Therapeutics. R.Pillai: None. M.S.Rayas: None.
National Institutes of Health (KL2TR002646)