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29 years old CF patient

I am from Romania and I have a 29 years old son with CF, which was diagnosed late... when he was 24 (genetic mutation-milder form= G85E; R347P and test sweat = 110 )

In childhood he was diagnosed with a syndrome malabsorption . At age 12 he already hadsinusitis and nutrition problems, dry cough.. In Bucharest I consulted over the years, medical specialists: Otorhinolaryngology, nutrition , pulmonary, who have treated each disease on his specialty...

In this moment he is under observation and treatment of a doctor specialist in CF from Bucharest with medical tests regularly at 3 months, and he takes proper medications ( Kreon, Pulmozyme,Seretide, gentamicin/ colistin in aerosols, vitamins, omeprazol)

I wish to find another medical opinion (I know at this momment, no cure found in this disease, yet!) but I would like to find what treatment options currently exist in THERAPHY OF CF IN ADULTS

Can you please advice me if there is any clinic or hospital specialiezed in the treatment of CF for adults especially, with good experts where my son can be checked .. in Europe or other places?

I am looking forword for your answer,
Dear questioner
Thank you for your question. We have contacted a colleague in the European Cystic Fibrosis Society to help us with your question. Please see the information below from the Romanian CF association website page dedicated to CF centres

Regional Centre Bucharest, Dr.Popa Cristian, Institutul de Pneumologie Marius Nasta, 0213356910 / int43

Regional Centre, Timişoara, Prof dr Tudorache Voicu, Clinica de Pneumologie, Spitalul Victor Babeş, 0256207670 / 0256207671

I hope you find this useful.
Judy Bradley

The answer is edited by: Prof Judy Bradley
To adress the part of the question about current treatment options in the therapy of CF in adults, a few remarks can be made:
In general, therapy of CF adults does not differ in the main from the therapy of CF children, apart form a few techniques or medication formulations that can not be used by little children.
The CF therapy is mainly based on the therapy of maldigestion and lung disease. Of course, if other problems like diabetes or nasal polyps/sinusitus i.a. occur, other treatment regimens join in.
For treatment of maldigestion and nutrional health the correct replacement of pancreatic enzymes at any meal (in case of pancreatic insufficiency) dependent on the amount of fat in the food is mandatory. Control and supplementation of fat soluble vitamins is also important.
To control symptoms of lung disease, regular physiotherapy, inhalative therapy and antibiotic therapy in case of exacerbations are the cornerstones.
It is important to start physiotherapy early and to have the supervision and teaching of an experienced physiotherapist in CF. In early childhood the physiotherapy techniques differ of course from those used in older children or adults. Older children and adults can use more active techniques that makes them more independent as they can perform their physiotherapy with the help of e.g. autogenic drainage themselves.
In combination with physiotherapy, inhalation of mucolytics (Pulmozyme® (rh-DNAse), hypertonic saline or Bronchitol®(Mannitol)) is very important to support clearance of the airways from the sticky secretions. Some patients
profit also from inhalation of drugs that wide the bronchi (so-called beta-2-mimetics). In case P. aeruginosa is chronically present in the bronchi and can not be eradicated after acute infection, inhalation of antibiotics like tobramycin or colistin are an important part of the long-term therapy. Recently, many new formulations for inhalative drugs, such as dry powder inhalers, have gained market- authorization. In contrast to little children, that can in early years only inhale via a mask and nebulized compressor inhalation, adults and older children can choose which kind of device they want to use for application of drugs and like this miminize time for inhalative therapy (e.g. fast nebulizing mesh devices like pari-e-flow or dry powder inhalers for some drugs).
For all patients, regular controls at CF-center (at least every 3 months) are important, to assess lung function and to screen for pathogens that colonize the airways and like this to be able to react promptly to those findings. A prompt antibiotic therapy of any worsening of the clinical status is also important for maintenance of lung health.

If you want to read more about standards of care for CF patients, please use the following link on the ECFS website:

There you can download the PDF file of the article:

Kerem et al. in the Journal of Cystic Fibrosis 2005, 4:7-26:”Standards of care for patients with cystic fibrosis: A European consensus.”

D. d'Alquen