Nasal polyps, complete occlusion - 12-year-old son

Question

My newly diagnosed 12-year-old son, 2 positive sweat tests, complete occlusion of the right and left nose, should undergo an operation. Who has experience in this field, how are the chances for his cure (risk of relapse?) respecitvely which kind of therapies, food supplements etc. are there for this rare symptoms? Nasonex® spray (cortisone) did not have an effect after 3 weeks. I am also thankful for a respecitve link, as in the web almost only bronchial and lung therapies etc. are covered.
Many thanks for the answer!

Answer

Hello,
an involvement of the nose and sinuses is present in nearly 100% of CF patients, even if it deals sometimes only with a diminishment or a lack of development of frontal sinuses. Up to 50% of all poeple with CF have nasal polyps in the rhinoscopy or in the imaging (CT/MRI) in the course of time. These are benign proliferations of the mucosa caused by the chronic inflammation due to the underlying CF and the colonization of the sinuses with problem germs, to which also S. aureus and P. aeruginosa may belong. Polyps can be as small as a few millimeters and never be a problem, however they can also grow out of the sinuses and occlude the entire nasal meatus. Then an impairment of the nasal breathing is the consequence, warming, humidifiying and cleaning (filter function) of the nose is lacking, dry and cold air is breathed in directly via the mouth and the lower airwaysin into the lung. Snoring, headache, retention of secretions with purulent discharge can be a consequence.
Nasal polyps react very well (in case of allergic polyposis better than in case of CF) to low dose cortisone sprays such as Nasonex®, Avamys®, Budenasal® or the like. Due to their low dosage, they do not have the feared systemic side effects of cortisone. Therefore, the feared long term usage over months and years is relatively uncritical.

Unfortunately, one does not reach the sinuses with nasal spray, from which polyps grow out in general and also in case of a very marked finding it is difficult to get a great effect with the spray. Therefore an operation can be necessary.
In case of ENT operations in CF patients, we in Jena [university town in Germany] recommend special attention and aftercare, in order to avoid a recolonization of the sinuses with problem germs such as P. aeruginosa, that we experienced in single patients.
A monthly microbiological investigation of nasal washing secretions before and after operation is for us standard as well as in the meantime also an antibiotic prophylaxis with ciprofloxacin or a 2-week i.v. antibiotic course in case there is no P. aeruginosa colonization of the lung.
In order to avoid the very frequent relapses in CF, the usage of a cortinsone nasal spray for 3-6 months after the ENT operation is sensible as well as the regular removal of secretions and crusts with the nasal shower with 250ml NaCl 0.9%. As well as with inhalation devices special attention has to be paid here on the cleaning of the nasal cans.
In Denmark, antibiotics are added to the nasal washing solution after ENT-operation (Aanaes 2012/2013).

With best regards,

PD Dr. Jochen Mainz

03.12.2013