When the tiniest cause the biggest issues
Due to the fact that there is a lot of viscous secretion in the lungs, this is the perfect environment for bacteria like pseudomonas or staphylococcus. These bacteria settle down in the lungs and bronchia and cause viscous mucus which is plugging the tracheae. In order to free the pipes, the bacteria are fought with antibiotics on the one hand (pills, inhalation or IV). On the other hand NaCl inhalation tries to fluidify the mucus and with a following physio therapy it is easier to cough and get rid of the mucus.
In most cases a CF patient will be infected by pseudomonas at some point in his life for example by drinking polluted water or swimming in lakes. Once the bacteria is in the lungs there's hardly any chace to get rid of it again. With antibiotic pills and daily inhalation sessions it can be dammed up but sooner or later, these medicine is no longer having a big enough effect and therefore a two week IV therapy is needed.
Because of the mucus in the lungs and bronchia CF patients do always have a reduced lung function. The air exchange is limited due to the blocked air pipes and this causes a volume trauma and shallow breathing. A common value within the lung function testing is the so called FEV1. This is the maximum volume of air which can be exhaled in the first second. If a healthy person has values around 4 litres, a CF patient would only score 1-2 litres. That's why these patients suffer from breathlessness very quickly and their performance ability is very limited. Climbing a few stairs can already be a real challenge.
The "missing" air in the lungs is also causing a reduced oxygene saturation in the blood. Personally, I have values of 90-94% whereas a healthy person would have 99 or 100%. Only the slightest activity makes the saturation drop below 90% which is called anaerobic and causes quick fatique and long recover phases. In serious cases there's need for an oxygene mask. I have such a machine at home but luckily I only need it while sleeping at night in order to recover better and quicker.
The neverending therapy with a variety of antibiotics can cause resistant bacteria. This means that the antibiotics are no longer killing the bacteria and there is no other help. The last possibility in these cases is having a lung transplantation. Here in Switzerland, every year there's only very few lungs available for transplantation. And due to the fact that people only get on the list once the disease is very progressed many people are still dying every year.
The following might be only suitable for Swiss people but I am sure that it will be similar in other countries.
No one is keen to think about his own death. Nevertheless it is important that you have an opinion about what is going to happen with your organs if you are dead. It doesn't matter whether you are willing to donate them or whether you don't. The important thing is that you have made up your mind and also wear a donation pass with you. In Switzerland, such a donation agreement can be downloaded on the Swiss Transplant page. There might be similar solutions in other countries.
I sincerly thank you in the name of all patients on a waiting list!