ABPA – very severe course of disease

Question

Our daughter (eight years old, CF) contracted ABPA out of the blue in September, with a very severe course of disease. Previously (over the past twelve months), there had been repeated instances of slightly increased IgE levels around 300, but that did not seem alarming). She shows the known symptoms within a few days and then, within 48 hours, has acute dyspnoea, very strongly pronounced soft tissue emphysema, among other things bilateral pneumothorax, and finally the inevitable intubation.

Upon being admitted to the hospital, her IgE levels were at 320 and rose to more than 3000 within two weeks at the hospital, whereupon ABPA was diagnosed. She has been treated with cortisone for one week. The last blood sample, just one week after beginning treatment, showed that the value increased even during cortisone therapy (50mg daily), namely to almost 4000. Is this a typical course of ABPA? Wouldn’t our daughter additionally need an antifungal drug? Being laypersons, we are very worried that the fungus actually has a walk-over with the current therapy (antibiotic and cortisone). Blood sugar is elevated, but currently still at a range where insulin should not be necessary.

Many thanks for your quick answer.

Answer

Hello,

You are saying that your eight-year-old daughter had acute complications in September, with acute dyspnoea, development of a bilateral pneumothorax with pronounced soft tissue emphysema and the necessity of intubation. The current problems were preceded by a roughly 12-month-long phase with slightly increased IgE levels around 300. In the acute phase, the IgE rose up to 3000 and then further to 4000 despite cortisone therapy. You are also saying that your daughter, in addition to the cortisone treatment, is currently taking an antibiotic. You are asking whether additional therapy with an antifungal drug would not be necessary.

What you are describing is indeed a very severe course of ABPA. Several things have to be said concerning the overall problem.

1.) The ABPA diagnosis has to be considered very likely. However, further tests should be done to see whether another allergenic cause might after all be possible as well.

2.) Your daughter’s body weight is probably somewhere around 25 kg. This means the cortisone dosage is calculated to a maximum of 2 mg/kg. Based on the severe course of disease as well as on the still slightly increasing IgE values, the dosage should be increased considerably.

3.) You are right in asking about additional therapy with an antifungal drug. Such a supporting therapy should absolutely be started.

4.) Furthermore, it should be discussed in consultation with an experienced allergist whether therapy with a monoclonal antibody against IgE (omalizumab) should also complement the therapeutic regime.

5.) Based on your manner of speech, I assume you live in Switzerland. You should urgently consult a designated cystic fibrosis specialist who is also considered an expert in ABPA treatment. Maybe the Swiss Society for Cystic Fibrosis can provide an appropriate contact address.

We wish your daughter a speedy recovery.

Kind regards,
Dr. H.-G. Posselt

04.11.2013