There are several main effects of cystic fibrosis on the lungs. Most notably, the disease creates lung tissue damage and increases the risk of bacterial infection. Cystic fibrosis is autosomal recessive, which means that it’s on a non-sex chromosome and that an individual must possess two mutated forms of the gene in order to develop the disease. Other harmful common effects of cystic fibrosis on the lungs include mucus build-up, inflammation, increased blood pressure in the lungs, and blocked or tightened airways.
Cystic fibrosis is caused by two mutated forms of a protein known as the cystic fibrosis transmembrane regulator (CFTR), which regulates the salt levels in sweat, mucus, and pancreatic secretions. Instead of keeping up normal functioning of these fluids, the mutated CFTR protein is unable to maintain healthy levels of salt and water on the surfaces of the lungs. The result of cystic fibrosis on the lungs is a thick mucus layer that does not contain a proper fluid or electrolyte balance.
The thick mucus that is characteristic of the disease leads to many problems for the lungs. Cilia, or tiny, hair-like structures lining the inside of the lung, have difficulty sweeping this thick mucus out of the body. Lung health is optimized by these small structures, which clear away trapped debris and bacteria, but the cilia in cystic fibrosis patients often cannot rid the lungs of potential pathogens. Not only does this lead to an increase in bacterial infection, as the bacteria remain trapped in the lungs, but it also causes the buildup of excess mucus harmful to the airways.
Another harmful result of cystic fibrosis on the lungs is inflammation. Coupled with damage due to bacterial infection, inflammation can often exacerbate existing breathing problems by tightening the airways and making it more difficult for the body to clear away the developing mucus in the lungs. Inflammation can also lead to an increased blood pressure in the lungs, which is risky for already fragile tissue.
Some of the more severe effects of cystic fibrosis on the lungs include pneumonia from frequent bacterial infections, violent coughing that sometimes produces blood, cardio-respiratory problems, and hypoxia, a condition in which the body does not get enough oxygen. Severe bacterial infections might also occur, some of which are antibiotic-resistant. The damaging effects of cystic fibrosis often mean that patients with the disease will need lung transplants to regain basic lung health and functioning.