Melissa Benoit

What causes cystic fibrosis...how do the genetic changes affect the body?

The CFTR protein sits in the cell membrane and acts as a channel for chloride, moving it out of the cell, which in turn causes sodium to move into the cell. By maintaining this sodium-chloride (salt) fluid layer in the airways of the lungs, the mucous is kept thin and it is easier to clear from the lungs. When CFTR protein is absent or not working properly, the mucous becomes dehydrated, thick and does not move easily out of our airways. This thick mucous layer lacks oxygen and creates the perfect environment for certain bugs to thrive in your lungs. As bacteria enter the airways, our body works to fight these bacteria with our immune system. Unfortunately, the immune system is unable to clear the bacteria that are trapped in the thick mucous layer and over time these immune cells cause inflammation in the lungs. The chronic inflammation results in damage to the lungs creating a viscous cycle of infection and inflammation. Antibiotics work to decrease the number of bacteria within the lungs which results in less inflammation. Other inhaled medications can be given to thin the mucous and make it easier to clear the secretions from the lungs.