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Kalydeco and enzymes

my daughter gets Kalydeco for 16 months now. Now it came out more and more, that she gets constipation and stomach ache with the normal dosage of enzymes. We have markedly reduced the enzymes then. Unfortunately, this was not successful either. Then we did not give any enzymes at all anymore. Now the digestion is perfect. Now it is however like this, that she coughs for instance from time to time. Not much, but before, she did not cough at all. Now I am worrying a lot if Kalydeco cannot act without enzymes.
Is this possible in your opinion?
Best regards!
you report, that your daughter gets Kalydeco for 16 months. Under this therapy, a strong constipation occurred, that did not improve when reducing the dosage of the enzymes. Then no pnacreatic enzymes were given at all anymore. Now the stool was o.k.Your daughter coughs from time to time, which was not the case before. You are worrying, that Kalydeco could probably not be effective without pancreatic enzymes.
Before it has in general to be said, that it came out in the meantime, that some CF patients regained a normal function of the pancreas under the therapy with Kalydeco. This could be the case in your daughter. This has however to be proven by an investigation of the pancreatic elastase I in the stool (best would be two investigations). If it should come out by this investigation, that the pancreatic function is normal again, the stop of the enzyme therapy is justified. As Kalydeco is also influencing the electrolyte and biocarbonate metabolism in the gut positively, one can in general count on an improvement of resorption. Therefore it is unilkely that with stopping the pancreatic enzymes the therapeutic effect of Kalydeco is reduced. However it should be pointed out again, that the enzymes can only be stopped, if a normalization of the pancreatic function can be proven.
Finally a hint for the control of the therapeutic success of Kalydeco: in your child, the sweat test values had for sure be controlled under the therapy with Kalydeco and for sure a positive development of the values in the direction of normal sweat test values could be found. If however an actual control should show a worsening of the sweat test values, this could be a hint for a reduction of the therapeutic effect of Kalydeco.
Best regards,
Dr. H.-G. Posselt