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Orkambi and diabetes

my son takes Orkambi for a short time period. He has CF and diabetes, that is treated with Repaglinide (3 x 0.5mg). Among the drug interactions of Orkambi is is written, that is decreases the effect of Repaglinide. Is it therefore sensible to go on taking Repaglinide or are there any alternatives?
Many thanks and best regards,
Dear questioner,
thank you for your request.
Orkambi is licensed for patients over 12 years of age with the mutation delta F508 homozygously in the CF gene (=cystic fibrosis transmembrane regulator protein (CFTR). Insofar I assume, that your son is homozygous for deltaF508 (both genes carry the mutation deltaF508) and is over 12 years of age.
As you already mentioned, the effect of Repaglinide can be decreased by Orkambi (in the sense of an interaction).
I assume, that you son controls his blood sugar respectively the treating physician is measuring the HbA1c. In case the diabetes should worsen resp. via the interaction less Repaglinide is available, you should discuss with your treating physician alternatives (insulin or other oral antidiabetics). As you know, Orkambi is a combination of Lumacaftor and Ivacaftor. One knows from patients, who took Ivacaftor in case of the muation combination deltaF508 and G551D the following: there are reports, that the diabetes (in one case) disappeared completely or the need of insulin decreased. Therefore it cannot be excluded, that your son has less need for Repaglinide. Possibly the decreased amount is sufficient, in order to control the diabetes well. A monitoring of the blood sugar is important (short term effect) and of the the HbA1c (long term effect).
I hope to have helped you furhter.
Best regards,
PD Dr. Sabine Schmitt-Grohé