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Sweat test with Kalydeco®

Question
Dear physicians,
my son has the mutations G1244E and R553X and gets Kalydeco® for 2 months. He has had a sweat test result of 110 at his diagnose about 2 years ago and now after two months of Kalydeco® the value went down to 80. My question is, do you think that this is the endpoint or can one still hope, that the value goes down further? We will repeat the test after 2 or 3 further months. My son is 4.5 years old and has been diagnosed at the age of 2.5. How should the value be that he has no constraints because of his CF?
Many thanks in advance,
D.R. mother of the child
Answer
Hello,
the value has gone down markedly. More important than the observation of the changes of the chloride value in the sweat, that does at first not show any changes in the lung tissue or the gastro-instinal tract, is the observation of changes in the BMI, lung clearance index, microbiological and lab values, Elastase and Calprotectin in the stool. Furthermore probably images of the lung or ultrasound of the abdomen.
A decrease of the sweat value in the described range does however indicate, that the drug is absorbed and has an effect.
Best regards,
Dr. Olaf Eickmeier
16.04.2016