CF???

Question

Hello,
I am worried abou my 15-months-old daughter. She is coughing since the middle of January. Sometimes with fever and sometimes without. Nothing seems to help (she is inhaling cortisone…ambroxol…cough syrups…)I have been with her to the pediatrician…in hospital…general practitioner…pneumologist..they say always that it would be an infection that will go away in 2-3 weeks – this is however not the case. ..she has a lot of mucus…if she has a coughing period, she is even vomiting and gets dyspnea. This was about the cough.
Furthermore, she is from the beginning on sweating a lot and then smells like vinegar. She is also tasting salty. Since the cough started, her stools have also changed. Mostly constipation and then comes a portion of stool that smells awfully and is like diarrhea. She is 83cm tall and weighs 12 kg.
I would like to have a sweat test done – however today my general practitioner did not want to give me a prescription. I would have to go tomorrow to the pediatrician he said. Could here be CF underlying?
[Translators comment: later came the following additional information:]
Today a sweat test has been done at a CF center, this was normal with 22, the doctor said I would not have to worry....

Answer

Hello,
you report that your 15-months-old daughter suffers since January from a therapy-resistant cough that is partly accompanied by fever. Because of this, your daughter had seen a pediatrician, a general practitioner and a pneumologist and she got an inhalation with cortisone, ambroxole sirup and other substances without success. The daughter would have a lot of mucus and would partly vomit when she is coughing up the mucus. You report furthermore, that your daughter sweats a lot and that her sweat would taste very salty. With the beginning of the cough the stool had changed and she now tends to constipation and had partly very awfully smelling diarrhea.
You ask, if the mentioned signs could be a hint for CF.
First of all it has to be asked, if your daughter has a secure vaccination protection against pertussis, as the complaints are very suspicious for pertussis. If pertussis can be excluded for sure, then a sweat test should be done, in order to rule out the diagnosis or, in case of a positive result, to start immediately with the right treatment. Of course it is also possible, that your daughter had at first a viral infection and then a bacterial infection followed, that requires a consequent antibiotic therapy. In case it has not been done already, it should be considered to do a blood test with the most important inflammatory markers and to have an x-ray of the lungs done.
For sure it is right, to discuss with your pediatrician an aimed diagnostic procedure.
[ Translators comment: the information about the negative sweat test came later, and Dr. Posselt wrote then the following:]
Hello,
that are good news. If the sweat test had been done at a CF center you can rely on the result. 22 mmol/l chloride concentration is far away from a result suspicious for CF. Therefore CF can be excluded for sure and you can discuss with your pediatrician how the recurrent infections of the airways could be treated the best.
We wish you and your daughter all the best.
Yours sincerely,
Dr. H.-G. Posselt

20.06.2016