4th operation of the sinuses?

Question

I face the question to have our son be operated at the sinuses for the 4th time. [Remark of the translator: I left out the name of a German hospital that was considered for the operation]

Answer

Dear family with the 16-year-old son, who should get for the 4th time an operation of the sinuses.
In case of CF, the involvement of the nose and sinuses as well as the involvement of the lower airways and lungs, is a typical finding. In nearly 100% of patients, pathogenetic changes of the sinuses can be found in the CT or MRI scan. The cause is the defect of the chloride and sodium channels (CFTR defect) that is also present in the mucosa of the upper airways. As a consequence, very many CF patients suffer from a chronic inflammation of the nose and its sinuses (rhinosinusitis) and up to 50% of CF patients have nasal polyps in their lives.
Even if the conservative possibilities of an ENT therapy should be fully exploited before an ENT operation is considered, this is however very often not the case in CF, either. As a consequence, many CF patients are operated again and again - whereby the underlying CFTR defect persists and neither the cause nor the resulting colonization with germs can be removed by the operation. Therefore I know CF patients, who have been operated for 13 or even over 18 times at the sinuses.
Conservative therapeutic measures are the removal of secretions and crusts via nasal irrigations with 250ml isotonic sodium chloride solutions in general. Partly this can also be achieved with lower amounts. The long term usage of local steroids (Nasonex®, Avamys®, Flutide® nasally, Budenasal® etc.) inhibits the inflammation and is relatively effective on the swelling of the mucosa and the size of the polyps; those steroids can be used as nasal spray at night for months and years. The cortisone dosage is hereby so low, that is does not become effective in the blood and therefore no typical cortisone side effects occur. Decongestive nasal sprays can be used short term for maximally 5-7 days, also directly bevor the usage of nasal irrigations, of nasal sprays and nasal inhalations. However, if they are used for a longer period of time, they damage the mucosa!
In case of respective colonization with germs (e.g. Pseudomonas aeruginosa) also an inhalation with e.g antibiotics as vibrating aerosols into the nose and its sinuses could be recommendable.
Aim of the conservative measures is to lower the necessity of recurrent operations and the frequency of relapses.
Best regards,
PD Dr. J. Mainz

30.04.2014