Lung produces since recently extremely much mucus, why?

Question

Dear expert team,
my brother, my sister and me, we suffer from CF.
Unfortunately the condition of my brother worsened extremely in the last 4 weeks. The reason is until now not really clear. He got 5 weeks ago ORKAMBI as a new drug. Unfortunately it seems that at the same time a pneumonia came up.
This had seemingly the effect, that his lung produces extremely much mucus. This had been mixed with blood in the beginning, now it is brownish. He expectorates the mucus with inhalation and physiotherapy.
Unfortunately the lung goes on in producing much mucus and our physicians of the center cannot explain this.
Is there a reason known to you why such immense amounts of mucus are produced?
Actually my brother is transferred to a center in order to get prepared for lung transplantation.
Many thanks for your help!

Answer

Dear questioner,
I believe, that the phenomenon of the increased mucus production can also be explained by the effect of the drug "Orkambi". This combination of a correcor/activator leads in the end to a partly restoration or the CFTR protein, that is not functioning in CF; this protein has the function of an ion channel for chloride and bicarbonate in the cells of the epithelium of exocrine glands. This means, that e.g. in the lung the necessary fluid flow on the bronchial epithelium, that is necessary for the movement of the ciliae and for the transport of the mucus, is restored. With this, the so-called "muco-ciliary clearance", that means the transport of the mucus out of the bronchi, is restored. It is well imaginable, that in case of your brother, the drug is working and also "old" mucus, which accumulated there for years, is again mobilized and finds its way out of the lung. This has not necessarily a better clinical condition as a consequence in the beginning. Much mucus can obturate the airways and can lead to a worse clinical condition of the patient. This effect could also be an explanation for the only slight clinical improvement of the FEV1 in the studies. Orkambi would even not be a wonder drug if it had 100% efficacy, because it would then on the one hand correct the defect of CF for 100%, however on the other hand it could not change the already existing secondary changes and destructions of the lung and the bronchi.
In so far time will show, if your brother could profite subjectively after some time from Orkambi. I keep my fingers crossed.
Best regards,
Olaf Sommerburg

12.06.2017