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My 14 year old son with CF was just told he has Bronchiectasis; is it reversible? The drs aren't doing anything about it?
Dear questioner,
as you know CF is an illness with an impaired mucociliary clearance of the airways, due to the production of a very viscous mucus, that originates from a genetic mutation of a chloride channel in the cell membrane. Due to this impaired clearance of the airways, CF patients suffer from recurrent airway infections and inflammation. This chronic inflammation damages the bronchial walls, then it comes in the long run to a destruction of the bronchial wall resulting in bronchiectasis. Many CF patients develop those bronchiectases in the course of time, unfortunately often not only localized, but diffusely distributed over the lung. Those bronchiectases can be seen in the thorax x-ray and even better in a CT scan of the lung. The therapy is mostly conservative and aims at prevention of further infections to avoid further progress of lung disease as bronchiectasis is irreversible.
Therefore, it is of utmost importance, to optimize the conservative therapy: intensify physiotherapy with drainage techniques like autogenic drainage and many other possible breathing techniques, intensify inhalation of mucolytic drugs to improve the mucus clearance, early antibiotic therapy in case of finding of pathological germs in airway samples or in case of infection (some centers even do regular i.v. antibiotic courses), in case of chronic colonization with Pseudomonas aeruginosa inhalation of antibiotic drugs. Surgical intervention comes seldomly into question in CF, as this is only a potential option in case of isolated bronchiectasis and if conservative treatment fails.
Therefore, the most important issue for your son is firstly to be regularly seen in his CF center with good surveillance of his therapy, his lung function, microbiological findings etc. in order to react early to infection or exacerbation of lung disease and secondly to follow or even intensify his inhalative and physiotherapeutical treatment regimen. With that, we cannot eliminate existing bronchiectasis but avoid the formation of new ones and slow down lung disease.
Best regards,
Dr. Daniela d’Alquen (coordinator of the central English Archive of ECORN-CF)
The answer is edited by: PhD Lisa Kent