Pancreas

Question

My daughter’s CF was clearly diagnosed by genetic testing, however her case seems to be a borderline case. Should I point out these new circumstances when we go to the next regular check-up in the CF center? In the meantime, her appetite is better again and she complains less frequently about having abdominal pain. Is it possible that the sypmtoms are caused by long-term antibiotic treatment with Zithromax® (azithromycin)? Many thanks.

Answer

Dear questioner,

before I answer your questions let me consider your/your daughter’s situation first since your question does raise further questions on my part.
You say that your daughter’s CF was clearly diagnosed by genetic testing and that her case seems to be a borderline case and you want to know if you should point out these new circumstances when you go to the next regular check-up in the CF center. From this I take it that these so called “new circumstances” (that it seems to be a borderline case) have not been diagnosed by your CF center. This raises the question what your assumption of your daughter being a borderline case is based on, e.g. did you perhaps go to a genetic consultation independently of your CF center and there (at the genetic consultation) they told you that it is a borderline case based on the kind of mutation? Whereas generally speaking it has to be noted that the same mutation in different patients can have a different clinical course as well.

Whereever you got the information from you should discuss such additional information with your CF center in any case. This helps to find the optimal therapy for your daughter, to keep a mutual trust and even to avoid misinterpretations by going through all findings and the consequences again.

Therefore the answer to your first question is clear: please inform your CF center about all new circumstances when you go to the next check-up. Furthermore, it also has to be assessed clinically if it is really a borderline case; a borderline case would imply a rather mild clinical course.

In this context I wonder about the long-term antibiotic treatment with azithromycin (Zithromax®) that you mention. Why does your daughter get a long-term antibiotic treatment? Typically, a long-term antibiotic treatment with azithromycin is used in case of a chronic colonisation with Pseudomonas aeruginosa since it has been shown that this medicament does not only have antimicrobial effects but anti-inflammatory effects as well what can improve the lung function. A chronic colonisation with Pseudomonas aeruginosa does not fit exactly in the typical frame of a borderline case; however, there are always exceptions to the rule. With the given information I cannot make a statement about this.

Regarding your last question: Indeed, absence of appetite and abdominal pain are typical side effects of a therapy with azithromycin as well, but they can also be an expression of a pancreas disfunction in CF. Therefore, it is important to have checked the pancreas function and administration of enzymes by your CF center in order to exclude that this is the reason for the discomfort.

I hope to have been helpful with these considerations.
Best regards,
Dr. D. d’Alquen

14.01.2010