128 The metabolic consequences of CFTR modulation with ivacaftor in a single adult cystic fibrosis centre cohort

• Published in: Journal of cystic fibrosis 2015-06, Vol.14, p.S90-S90
• Main Authors: Ronan, G, Ronan, N.J, Shortt, C, Fleming, C, Cronin, K, McCarthy, M, Hickey, C, Murphy, D.M, Eustace, J.A, O'Halloran, D.J, Plant, B.J
• Format: Article
• Language: English
• Subjects: Pulmonary/Respiratory
• ISSN: 1569-1993; 1873-5010
• DOI: 10.1016/S1569-1993(15)30305-2

Objectives Ivacaftor produces significant benefit in patients with cystic fibrosis (CF) with the G551D mutation. Recent data have supported improved insulin secretion profile and reversal of CF related diabetes (CFRD) in patients treated with ivacaftor. Significant weight gain has been demonstrated with ivacaftor, thus raising the potential for development of dyslipidaemia in a cohort traditionally treated with a high fat diet. Methods 24 Adult patients with the G551D mutation had Oral Glucose Tolerance Test (OGTT), Fasting lipid profile and HbA1C measured before and after commencing ivacaftor therapy. 6 patients had pre-existing CFRD and Insulin requirements were analysed in this cohort before and after ivacaftor. Data was analysed using Wilcoxon signed-rank test. Results No significant change was observed in fasting glucose, 2 hour post prandial glucose, or HbA1C after commencement of ivacaftor. Significant median increase in total fasting cholesterol (0.2 mmol/L, P = 0.025) and HDL (0.16 mmol/L, P = 0.03) were observed after commencement of treatment. There was no significant change in LDL or Triglycerides. In the 6 patients with CFRD no significant change in either total insulin requirements or units of insulin per kilogram of weight were observed. Conclusion In a large single centre cohort with the G551D mutation we report no significant change in OGTT, HbA1c or insulin requirements after ivacaftor. While a significant increase in Fasting cholesterol was observed, median values were within normal limits. Changes in lipid profile may reflect the impact of adhering to a high fat diet as a consequence of therapy and will required long-term follow up.