Cystic fibrosis damages lung tissue, increases bacterial infection risk, and causes mucus buildup, inflammation, and blocked airways. It is caused by two mutated forms of the CFTR protein, leading to a thick layer of mucus that impairs cilia function and causes inflammation and hypoxia. Lung transplants may be necessary.
There are several main effects of cystic fibrosis on the lungs. In particular, the disease creates damage to lung tissue and increases the risk of bacterial infection. Cystic fibrosis is autosomal recessive, meaning it is found on a non-sex chromosome and an individual must have two mutated forms of the gene to develop the disease. Other common harmful effects of cystic fibrosis on the lungs include mucus buildup, inflammation, increased blood pressure in the lungs, and blocked or narrowed airways.
Cystic fibrosis is caused by two mutated forms of a protein known as cystic fibrosis transmembrane regulator (CFTR), which regulates salt levels in sweat, mucus, and pancreatic secretions. Instead of maintaining 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 layer of mucus that doesn’t contain a proper balance of fluids or electrolytes.
The thick mucus that is characteristic of the disease leads to many lung problems. Cilia, or tiny hair-like structures that line the inside of the lung, have a hard time clearing this thick mucus from the body. Lung health is optimized by these small structures, which clear trapped debris and bacteria, but the cilia in patients with cystic fibrosis often fail to rid the lungs of potential pathogens. This not only leads to an increase in bacterial infection, as the bacteria get trapped in the lungs, but also causes the buildup of excess mucus that is harmful to the airways.
Another harmful result of cystic fibrosis on the lungs is inflammation. Along with damage from a bacterial infection, inflammation can often exacerbate existing breathing problems by narrowing the airways and making it more difficult for the body to clear the mucus developing in the lungs. Inflammation can also lead to increased blood pressure in the lungs, which is risky for already fragile tissue.
Some of the more serious effects of cystic fibrosis on the lungs include pneumonia from frequent bacterial infections, violent coughing that sometimes produces blood, cardiorespiratory problems, and hypoxia, a condition in which the body does not get enough oxygen. You may also experience serious bacterial infections, some of which are resistant to antibiotics. The damaging effects of cystic fibrosis often mean that patients with the disease will need lung transplants to regain basic lung health and function.
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