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Continued fever with CF

Dear expert team,

our 14-year-old son has been running a fever of more than 39 degrees since April. He has P. aeruginosa, mycobacterium abscessus and ABPA. He has a port that was already contaminated with staph epidermids and changed in 2010. Currently, he is taking Tobi® (tobramyin), tazobactam [translator’s note: the original question says “taxobact”], vancomycin i.v., clarithromycin and an antimycotic (not Sempera®) orally. In addition, he is inhaling MucoClear® (NaCl) 6%, Biklin® (amicacin), colistin and Pulmozyme® (DNAse), plus salbutamol and Atrovent® (ipratropium bromide) as dosage aerosols.

Do you have any advice as to what else we could do?

Kind regards and many thanks.

An infection with the germs you mention requires consistent multi-drug antibiotics therapy with the aim of at least reducing the germ count. The intensity of such treatment is based on the degree of loss in lung function that has already occurred. A possible recurrent colonization of the port catheter (with or without functional limitation) must also at least be considered as a cause of the fever.

Successful therapy against M. abscessus is difficult. I.v. and subsequent oral therapy with several antibiotics (rifampicin and ethambutol), even when carried out over several months, is often successful in ca. 50% of patients only.

In case of existing ABPA, and depending on the substance used, therapy should be carried out after checking the serum level. Elimination and eradication of the germs you mention is usually hard to achieve after a long-term infection. In order to suppress the infection, sufficiently long treatment with the antibiotics mentioned is particularly necessary, as are inhalation therapy as well as physiotherapy and the breathing technique used in it in order to remove the infected secretion.

The only advice I can give you is that your son should do the daily physiotherapy routine as consistently as possible and that antibiotics therapy (i.v., orally and inhalative) is carried out long enough. Which particular antibiotics should be used needs to be determined in consultation with your CF centre and a microbiologist.

Kind regards
Dr. H. Ellemunter.