Routine inpatient admission

Frage

Good day,

Our 6-year-old son, who has the G542x and E92x mutations, is being considered for inpatient admission every three months. He has chronic Staphylococcus aureus (Pseudomonas has never been detected, but it was treated with IV antibiotics during his last two inpatient stays, based on suspicion). A port catheter is also being considered.

The treating physicians are considering the Danish model to slow further progression. Are you familiar with this model? What are the advantages and disadvantages of this proposed treatment approach?

Thank you very much for your assessment.

Antwort

Dear questioner,

I, Dr. d'Alquen, will first provide information on the Danish model before Dr. Heuer discusses your son's clinical situation.

First, let's look at the Danish model, or Copenhagen model, which was established in Denmark in the late 1970s. This model addresses infection with Pseudomonas aeruginosa. Among other things, it involves early and consistent treatment of the initial Pseudomonas diagnosis to prevent chronic infection. Therefore, according to the model, inhaled colistin should be started immediately upon initial diagnosis for four weeks, along with oral therapy using the antibiotic ciprofloxacin. If chronic infection cannot be prevented, the model recommends a planned 14-day course of intravenous [medication] every three months. Therapy (with two antibiotics effective against Pseudomonas) was administered even if the patient with chronic Pseudomonas was clinically stable and had no exacerbation, in addition to inhalation with colistin. Other models in other countries only call for IV therapy if there are exacerbations. Since later systematic reviews did not provide clear evidence that the Danish model is clearly superior, it has not become standard practice in all countries.

Your son has a chronic infection/colonization with Staphylococcus aureus; Pseudomonas was never detected, but was sometimes treated concurrently. Were antibodies against Pseudomonas ever tested? If these are negative and the bacterium was never detected, this argues against an undetected Pseudomonas infection. Often, the young patient cannot produce sputum, and throat swabs are not reliable—especially when making far-reaching decisions such as those concerning regular IV therapy. If necessary, bronchoscopy should be performed to obtain secretions from the lower airways to definitively rule out Pseudomonas.

Let's assume, therefore, that only Staphylococcus aureus is involved. Regarding this bacterium, the Danes tend to pursue a more aggressive strategy with early and consistent treatment—for example, treatment only if the bacterium is detected, even in the absence of symptoms. Treatment for this bacterium is primarily oral and can be administered for a longer period. There are also countries where children with cystic fibrosis receive long-term oral antibiotic prophylaxis against Staphylococcus aureus from birth to prevent colonization. Others treat only if the bacterium is detected, administering oral medication every three months and always in cases of clinical deterioration caused by the bacterium. In cases of clinical deterioration, intravenous therapy against Staphylococcus aureus would also be conceivable, but I could not find any literature on the "Danish model" that describes regular intravenous therapy every three months against this bacterium as standard practice, as is the case with Pseudomonas.

Now let's examine your son's clinical situation:

Due to the mutations, modulator therapy is apparently not an option.

The staphylococcal infection obviously necessitates intensive, regular IV antibiotic therapy.

The regular IV antibiotic therapy will also prevent a potential Pseudomonas infection.

If the venous access is not optimal, port implantation could provide significant relief for the boy.

Since modulator therapy is apparently not possible, and thus the underlying problem of cystic fibrosis cannot be addressed, and the chronic infection in the lungs is apparently progressing, I can understand the approach taken by the doctors at your CF clinic.

Due to the progression of the lung infection, I have always used a regular on-off IV antibiotic regimen in my CF center. This approach may also be reflected in the so-called Danish model.
... You will understand that, based on the information you provided, I can only offer a cautious assessment of the advantages and disadvantages of the proposed treatment.

My suggestion is to discuss the procedure in detail with your CF doctors and, if necessary, request a referral to a larger CF treatment center.

Sometimes a second opinion is helpful.

For example, the CF Center at Hannover Medical School (MHH) or Prof. Jochen Mainz
Prof. Dr. med. habil. Jochen G. Mainz
Senior Physician, Head of Pediatric Pulmonology, Cystic Fibrosis
Brandenburg Medical School (MHB)
West Brandenburg Hospital, Department of Pediatrics and Adolescent Medicine,
Hochstraße 29,
14770 Brandenburg an der Havel
Tel: +49 3381-411803
Fax: +49 3381-411809
j.mainz@klinikum-brandenburg.de

It has become clear to me that your son, with his chronic Staphylococcus aureus infection, requires very competent and comprehensive care.

And I have the impression that your doctors are giving serious and very conscientious thought to a suitable CF therapy for your son.

Please feel free to contact me again.

Sincerely, Dr. H.-E. Heuer and Dr. D. d'Alquen (Part I)

12.10.2026