Pancreas 2

Question

Dear Dr. d'Alquen,

my daughter has the mutation R1048G and R1162G; in our CF center, as well as in your forum we have been told, that that kind of mutation probably speaks for a mild course, whereby one has never a guarantee.
In the last winter my daughter had finally 7 infections of the lung, better said bronchitis, but she never had a colonization with Pseudomonas. In most cases the influenza virus had been detected, as well as other bacteria in the upper airways.
At the routine check in may we have been told by the CF-center, that one has to find out, why she has had so many infections.
Therefore we have had a stay in hospital in june for some days, the aerosol-therapy had been shown to us (two times a day beclomethasone and salbutamole with saline) and she takes since may every second day 250mg Azithromycin.
In September we had another check and we have been advised to go on with the therapy that my girl will be well-off. To our question, if the dosage is not a bit too high, they said that this would be better than again having infections.
Unfortunately the doctors do not know how it will go on, and my concern, that now also the pancreas could be involved, is present.
In any case we will tell them about our observations in january - we can wait until then, or not?
My daughter is doing really well at the moment, nearly no mucus at inhalation and her apetite is according to her age. The only striking thing is a rather soft stool, not to say mild diarrhoe from time to time.
Best regards and many thanks

Answer

Dear questioner,

it is good to hear that your daughter has not a colonization with Pseudomonas and that the Azithromycin is taken as a prophylaxis against new infections. Beside the usage of this antibiotic at chronic colonization with Pseudomonas, also in case of being free of Pseudomonas a positive clinical influence has been described and is therefore possible in case of signs of chronic inflammation or other germs.

The mentioned dosage of 250mg every 2 days is according to the recommended dosage for children under 18 years and under 40 kg bodyweight. Of course one has to make an individual decision about the duration of the intake and the dosage and your doctors in charge of the CF center know your daughter the best and only they can make an individual decision, when e.g. the therapy could be terminated.

From your remarks I conclude furthermore, that your daughter does not take any enzymes, therefore has had a sufficient functioning of the pancreas until now. You observe now from time to time a soft stool to a mild diarrhoe, but you describe that the apetite is normal.
In any case you should mention this at your next visit at the CF-center, even if it has been said that the mutation of your daughter does speak rather against a dysfuntion of the pancreas. In case there is any clinical suspicion for that, the function should be investigated (e.g. fat in the stool) in any case, in order to make sure that the diarrhoe is not caused by a dysfuntioning of the pancreas, even it is seems theoretically improbable.

In case your daughter shows acute complaints, you should of course present her immediately again; in case she is doing well, gains weight and has only mild changes of the stool without pain, you tell about your worries at the next regular check.

I hope to have helped you with the answer,
Yours sincerely,
Dr. D. d'Alquen

17.01.2010