Cystic Fibrosis is caused by a defective gene called Cystic Fibrosis Transmembrane Conductance Regulator (CTFR), affecting many organs of the body including the gastrointestinal, respiratory tract, musculoskeletal and reproductive system. The CFTR gene affects the bodies ability to transfer sodium chloride and water to and from cells resulting in sticky, thick mucus blocking mentioned organ functions.
This sticky mucus debilitates mobile cilia to effectively move debris through the bronchi
Most with CF develop Pancreatic insufficiency. The Protease, Lipase and Amylase excreted by the pancreas are blocked
Close attention & treatment have dramatically increased expected lifespan of someone living with CF.
Cystic Fibrosis is a disease that causes the body to produce thick, sticky mucus which affects primarily the lungs & digestive system. CF also affects the sweat glands, where increased body salts are lost particularly in hot weather or physical activities. Everyone produces mucus in their lungs but someone with cystic fibrosis, the mucus is so thick & sticky it is harder to move. This tends to block up the respiratory system. This mucus being tougher than an average persons to budge, creates an environment where bacteria can grow and where there is bacteria, theres a possibility of infection.
The reason for this thick production of mucus opposed to an average person is there is a certain protein where anyone with CF is deficient of. This mucus is also lining the digestive tract, including the stomach, pancreas, liver, intestines and reproductive system. This mucus lining the pancreas makes it difficult for them to absorb the much needed nutrients and vitamins. This is the reason for the sticky stools as it still contains a lot of the good stuff.
With the treatment we want to keep the lungs clear of infection by decreasing the mucus produced & keeping the person with CF on a good diet.
You may notice that someone with CF will cough a lot. This is their way of trying to get rid of the mucus buildup from their lungs. Giving a firm clap on the back will assist to break up the mucus to be expelled. This decreases the chance of developing any serious lung infections.
A medical team will play a big part in the treatment. This will include respiratory therapists, doctors, nurses, nutritionists, and physical therapists. The patient will be taught breathing exercises which can be used to deliver moisture and antibiotics to the lungs.