ESPE Abstracts

Introduction: Triple CFTR modulator therapy has been shown to improve lung function and quality of life in patients with at least one F508del mutation in the CFTR gen. However, effects on glucose metabolism are not yet well defined. This paper aims to describe the effects of this treatment on glucose metabolism in CF patients.

Methodology: Ambispective study.

Inclusion criteria: patients ≥8years old with CF genetic diagnosis, undergoing triple modulator therapy with ivacaftor-tezacaftor-elexacaftor, who have OGTT and/or continuous-glucose-monitoring (CGM) performed before (±4months) and after therapy onset (tests performed under baseline conditions, without exacerbation of background disease or corticotherapy at the time of testing or 4 weeks prior). OGTT: normal (NGT), indeterminate (INDET), impaired (IGT) or diabetes (CFRD). CGM: FreeStyleLibre2-Abbott performed for 14 days with exercise and regular diet; patients were classified according to previous published data, NGT: <4,5% of glucose monitoring time >140mg/dl, AGT ≥4,5% of monitoring time >140mg/dl or a glucose peak ≥200mg/dl and CFDR ≥2 glucose peak ≥200mg/dl on different days.

Results:

Nine patients (3 females), mean age 14,75years. All patients have pancreatic insufficiency and HbA1c ≤5,9% before and at the therapy, except for patient 9 who started with HbA1c of 7,9%. Patient 9 required nasogastric tube feeding, insulin (CFRD) and was a lung transplant candidate before beginning triple therapy, nowadays he doesn’t need nasogastric tube feeding, stopped insulin after 16 months on therapy and he is no longer a transplant candidate. We observed CGM glucose peaks >200mg/dl while having at the same time a normal OGTT (patients 1, 8 and 9).

Conclusions: • The effects of triple CFTR modulator therapy on glucose metabolism is still uncertain. Larger prospective studies are needed. • CGM is more sensitive than OGTT in detecting changes of glucose metabolism in CF patients.