Information on Cystic Fibrosis

What is cystic fibrosis?

Cystic fibrosis (CF) is a disease in which a genetic mutation affects the protein that regulates transportation of chloride ions through cells in various parts of the body. The relations of sodium and chloride mean that this causes problems with salt concentrations, in turn causing osmotic irregularities that cause mucus throughout the body to be very thick and sticky.

The main organs affected are the lungs. The lungs have a mucus lining that the body uses as a defense against irritants and germs that are breathed in from the outside environment. The invaders are trapped in the mucus, and the cough reflex is used to get them out. For people with cystic fibrosis, the mucus is much stickier than the mucus of healthy people. This causes problems because it is much harder to cough out the foreign particles, and bacteria can get stuck in the lungs and proliferate there. Then the body starts fighting off the infections with its normal immune response; however, due to the excessive amounts of infection, these immune ‘fights’ end up with fragmented blood cells getting into the mix of the mucus and can cause further clogging. This cycle of clogging leads to damage in the lungs.

Other parts of the body are affected as well. Mucus in the pancreas is also sticky and blocks the ducts that secrete digestive enzymes. As a result, most CF patients have to take enzyme pills every time they eat so that their food can be properly digested. Irregularities of salt transport lead to excessive salt in sweat (this is how most CF cases are diagnosed - with sweat tests as infants). Additionally, complications in the reproductive system cause most men with CF to be infertile.

CF is a pretty serious disease that causes problems throughout the body, and there is no cure. However, there is good news: many advancements in therapies and medications for CF patients have greatly extended and improved their lives. You can read more about these advancements on this site.

Learn more about CF and how you can help at the Cystic Fibrosis Foundation.