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Can NaCL infusion help CF patients

Question
Dear Sir/Madam,
I am a mother of a child with CF. Maybe my question is stupid as I do not have medical background, but I am wondering can usage of Na Cl infusion through the blood once a week help CF patients in term of bringing salt through the blood into lungs, pancreas, stomack, etc.? This might help nutrition and braking up the mucus or not?
Answer
Dear questioner,
CF is an illness that is caused by a genetic mutation in the so-called CFTR gene. This gene codes for a chloride channel in the cell membrane. If the CFTR gene carries a mutation, not enough or dysfunctioning chloride channel reaches the cell membrane. As a consequence, chloride, sodium and water cannot be transported properly from the inside of the cell to the outside surface of the cell and in the airways, for example, the mucus layer on top of the epithelium cells is too viscous as water and salt is lacking. The same happens in other organs like the pancreas for example —secretions and mucus are too thick mucus resulting in organ damage. So even if you would infuse NaCl directly in the blood, the cell could not transport it from the blood into the mucus, as the chloride channel is the problem and therefore, water and salt does still not reach the mucus.
However, patients with CF have a very salty sweat, as in the sweat glands there is a different secretory mechanism, and here, water and salt is secreted in higher amounts compared to healthy people. Therefore, CF patients tend to dehydrate and to lose too much salt when sweating in warm climate or during sports. Additionally, CF patients are less thirsty then healthy people and this aggravates the dehydration. Therefore, it is recommended for CF patients to drink a lot and to take extra salt (tablets, for example) when being exposed to the mentioned conditions. Therefore the idea of replacing salt and water is good, in situations where CF patients have an increased loss; however, you cannot improve the general problem of sticky secretions with regular NaCl infusions.
Best regards,
Dr. Daniela d’Alquen (Coordinator of the Central English Archive of ECORN-CF)
31.03.2014
The answer is edited by: PhD Lisa Kent