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How to react to an inexplicable decline in lung function?

Dear expert team,

I am 24 years old and have CF. As a basic therapy, I have been inhaling colistin, salbutamol, Atrovent® (ipratropium bromide), saline solution 5.85% and Viani mite® (fluticasone/salmeterol) long-term. In addition, I take azithomycin orally, 3x500mg per week. My diabetes is under control, and there are no problems with the pancreas either.

Since September, my FEV1 has declined continuously from 66% to currently 50%; the MEF25 is now at 8-10%. I have since had significantly more sputum, though mostly rather light-coloured. As soon as I do not inhale for 6-8 hours, breathing becomes harder and you can hear distinct breathing sounds in all variations even without a stethoscope. Neither an i.v. therapy with cefuroxime or tobramycin in October, nor a switch to cotrimoxazole orally in December could stop this development; actually, the latter made it even worse.

I have not had a noticeable infection since September, however; the last cold dates back about a year. The sputum currently only shows increased staphylococcus counts, of which I have had large quantities almost always since I was a baby, though. Pseudomonas is not traceable. My CRP usually, and currently as well, does not yield pathological findings. Other blood values such as IgE, etc. are okay.

At the moment, we are thinking about another i.v. therapy with ceftadizime and tobramycin or ciprofloxacin orally. However, I wonder whether this makes sense at all if the CRP is negative and there are not really any relevant germs.

Do you consider an i.v. therapy or an oral antibiotic sensible in this situation? Do you happen to have any other ideas as to where this decline could come from and what one could do about it?

Many thanks in advance!

Kind regards.

it is not easy to find a good solution here. Unfortunately, these types of progression happen for some CF patients, where nothing really serious is found in the sputum or the lab and lung function nevertheless declines, and it often is difficult to do anything about it. I am also not sure which other tests your doctor has done, but you should consider the following:

On the one hand, the doctor at the CF clinic should check again for asthma – after all, one can have CF and asthma at the same time – and also for ABPA (allergic bronchopulmonary aspergillosis), even if the IgE is normal, as you say.

On the other hand, one also has to look for germs that many labs often miss or do not test for if they are not requested to. These include so-called anaerobes (germs that live in environments that are low in oxygen) and also atypical mycobacteria. Your doctor should request this when he/she sends in your next sputum sample. Perhaps he/she has already done it – just ask him/her. If these germs are traceable, they could be the cause for your decline in lung function, and then the usual antibiotics therapy does not help, or at least not much.

Kind regards,
Prof. Dr. Joachim Bargon