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Kalydeco® and salt supplementation


In case of scorching heat salt supplementation is recommended for all CF patients.
But what about for patients on Kalydeco®? Because their sweat normalizes, so we can assume that salt losses are less ...

Precautions in case of hot weather, including hydration and salt supplementation, apply to all CF patients including patients treated with ivacaftor.

Certainly, treatment with ivacaftor in patients with mutation class 3 called "gating mutation" whose leader is the G551D, causes a reduction of salt in the sweat below the positivity threshold (60 mmol per liter of chlorine sweat), but this decrease varies from patient to patient. If the rate of salt falls below the negative threshold (30 mmol / l), the precautions are the same as those that apply to the general population. If the salt level remains high in an intermediate zone (between 30 and 60 mmol / L), the salt losses remain higher than in the general population: it is therefore necessary to remain vigilant and adapt extra salt intake and water.
On the other hand, the effect on the sweat test of ivacaftor and lumacaftor treatment in homozygous F508del patients is much lower. Precautions in case of high heat then apply fully.

Wishing you to enjoy safely the summer.
Hope this answer can help.
Best wishes
Gilles RAULT, MD, Roscoff CF Center