People without CF have proteins that regulate the way chloride ions move into and out of particular cells; which in turn regulates the movement of water into and out of those same cells (remember learning about osmosis in high school biology?). These proteins act a little bit like bouncers regulating the flow of people into and out of a bar.
PWCF have little or no functioning of these proteins – the chloride ions and water don’t go where they should and the secretions produced by the cells are way too thick. To extend the above analogy, this is like ending up with an exceptionally crowded bar because the bouncers didn’t turn up for work.
In the lungs, the thick mucus can’t be easily shifted up and out which creates a lovely environment for bacteria to flourish. In the pancreas, mucus blocks the tubes that are meant to transport the digestive enzymes to the food. Other parts of the body can be seriously affected too but the lungs and pancreas are consistently affected in PWCF.
Without modern medicine, this situation is a disaster and means a pretty quick death. With modern medicine, there are things we can do and medications we can take to circumvent these problems to a certain extent. We’re pretty lucky that the way CF is treated is still evolving and improving and new treatment options are becoming available.
In addition to prescribed medications, Dave and I add extra salt to our food, take salt tablets on really hot days, regularly inhale salt solutions or squirt them up our sinuses and holiday by the beach to try and get the salt where it should be – in our bodies.
Funnily enough, when we sweat our sweat is very salty! Our Kelpie dogs Flame and Scout love nothing more than to lick the salt from my arms and legs when I come back from a run. Prior to the 80’s when diagnosing CF was a bit hit and miss it was often suspected when a sickly child’s skin tasted salty. Measuring the level of salt in sweat is still the gold standard of CF diagnosis.