Amiloride therapy

Question

Hello,
my 14-year-old son has Burkholderia Cepacia for about 2 years.
After the iv-therapy in March 2010 as well as in June 2010 were not successful, one has the hope now to get rid of the germ with an amiloride inhalation quasi as an "intensifier" in parallel to the TOBI inhalation and the intake of Ciprofloxacin 750 mg 2 times a day and cephalexin 1000 two pieces, two times a day.
In your question Archive there is up to now nothing about amiloride. I would like to be informed about potential side effects and to be encouraged with success, as I do not feel comfortable with such amounts of drugs. (DNAse is part of his daily life anyway). The therapy should last at least half a year and run with the cycle of 20 days-1 week pause - 20days - 1 week pause...
Many thanks for your answer.

Answer

Dear questioner,
Your question has 2 parts.
1) Is the combination of TOBI and amiloride an effective therapy against B. Cepacia and 2) which kinds of side effects do occur?
Ad 1): After the first results from laboratory investigations in the 80ties of the last millenium, there were some single case reports about a successful eradication of B. Cepacia with the above mentioned combination therapy. A short report in January 2010 in the Journal of Cystic Fibrosis relativizes this success unfortunately. There patients with a long-term B. Cepacia colonization of the lung (more than 7 months) have been investigated. Here the wanted eradication of B. Cepacia was not successful. The successful eradications (reported in the European Respiratory Journal 2005) concerned the early therapy of B. Cepacia, not the therapy of the chronic infection. In addition, the dosages have been higher there.
In your son, an idvidual therapeutic trial is made. The expectation is unfortunately more subdued (see above), as the B. Cepacia is likely to be chronic after 2 years.
Ad 2): Side effects of the amiloride inhalation in the common inhalative dosage should be manageable, however it has to be said, that there is hardly any data for the inhalative usage.
In sum, an individual therapeutic trial, as in your son, is in respect of the B. Cepacia problematic justifiable. The rates of success should be judged realistically.
Yours sincerely,
Prof. M. Ballmann

29.11.2010