ABPA and prednisone

Question

Dear CF-team,

My 12-year-old daughter has CF with ABPA (allergic bronchopulmonary aspergillosis). For that reason she is being treated for several years yet with prednisone. This has a consequence on her growth. She is little for her age and I presume she has to take the prednisone. What are the effects in the long runs and could she leave out the prednison for a while? Or is there another medication yet?
Thank you in advance for answering my question.
Yours sincerely, A.

Answer

Dear parent,
Thanks for this question about an important complication in some patients with CF. On this forum we can only give you some general information. It is obvious that Ecorn is not the appropriate place to discuss the specific treatment of your child; you should discuss its treatment with your doctor.

‘Allergic bronchopulmonary aspergillosis’, in short ABPA can be best described as an allergic reaction to the presence of a mould –Aspergillus- in the airways and in the secretions. Aspergillus is a mould that is really encountered everywhere; but high concentrations are present in damp and dusty places (stables, construction sites). Inhaling this mould when you’re healthy doesn’t cause any problem.
In people with diminished immunity (e.g. patients with cancer under chemotherapy) this mould can cause life threatening lung infection.
In patients with CF, this mould rarely causes infection but we know an allergic reaction ‘ABPA’ as a complication. Due to an allergic reaction to this mould, patients cough, wheeze, have shortness of breath and increased secretions. On the chest X-ray, new abnormalities can show up and lung function drops down. It is not always easy to make the distinction between ‘classical’ bacterial infection and allergic reaction to aspergillus.
For this we need blood tests: high IgE and high numbers of allergic cells (eosinophils) in the blood, together with positive allergy tests for Aspergillus in the blood or on the skin, point towards ABPA.
The treatment of this complication is not clearcut and there is little evidence for the different treatments used. Cortisone or prednisone is certainly the traditional corner stone in the treatment. This important anti-inflammatory medicine diminishes the allergic reaction. Possibly, the add-on of an antifungal drug (drug that kills moulds) is helpful but there is again little scientific evidence for this. Most frequently itraconazole or voriconazole are used. These medicines are however not always taken up in the blood very efficiently by patients with CF. Another possibility is administering an antifungal medicine via aerosol but again there is little experience with this.
Omalizumab, an antibody that combines (‘binds’) with the allergic antibodies and thereby diminishes the allergic reaction has come on the market for treatment of severe asthma. Several doctors reported beneficial effects of this new medicine for treatment of ABPA in subjects with CF.
To summarize: ABPA is a well-known complication in CF. The treatment is difficult and long-term. There is little scientific evidence to state which treatment is best.
If prednisone has to be used for a long time, side effects can be seen. By decreasing the dose of the medication, side effects can be limited.
We hope that this is an adequate answer to your question.

Sincerely yours,
Prof. K. De Boeck

13.05.2013