The Devastating Disease called Cystic Fibrosis (CF)



This is a picture represting organs that are affected by cystic fibrosis.







Cystic fibrosis is a life time threatening genetic disease that causes mucus to build up in your lungs, and clog organs in your body. By having mucus clog your lungs it causes you to have problems breathing well. Bacteria then occurs and it leads to horrible infections. People inherit this disease from their parents through genes. Genes are found in the nucleus of all the body's cells, and it controls cell function by serving the role for the production of proteins.

 In order to have cystic fibrosis, the person must inherit two copies of the defective CF gene, which is one copy from each parent. There are 30,000 people in the United States who have this disease. One in every 31 Americans are carriers of the defective CF gene, but they actually don't have the disease. Some of the symptoms of cystic fibrosis is if you have salt-tasting skin, if you have a lot of lung infections, the consistency of coughing a lot, and shortness of breath. The majority of people are diagnosed at birth through a process called newborn screening. If the doctor sees symptoms of cystic fibrosis then they will order a sweat test to confirm the diagnosis. A sweat test is a very common method that is used in which a small electrode is placed usually on your arm to stimulate the sweat glands. If the level of chloride is high then that means the person has cystic fibrosis.

The healthy CF gene makes a protein which is Cystic Fibrosis conductance Transmembrane Regulator which controls the movement of electrically charged particles in and out of the cells. People who have cystic fibrosis, this protein is defective and the salt balanced is disturbed. Since there is a small amount of salt and water on the outside of the cells, the layer of mucus becomes very thick. There is no cure for cystic fibrosis, but medical care, and drug treatments can lenghten the lives of people with this severe disease. Most of the people living with CF take multi-vitamins, and take pancreatic enzyme supplement capsules with every meal. There is not a life expectancy, because different factors affect a person's health. Mucus-Thinners and Anti- inflammatories are some medicines that treat lung infections. 

Lining cells have channels on their outside surface. There are two channels and one of the channels allow sodium ions to flow into the cell and the other channel controls the path of chloride ions out of the cell into the mucus on to the airway surface. Water is drawn out through the gaps between the cells, which keeps the mucus moist. The vital chloride channel is blocked in people with cystic fibrosis . There is no movement that occurs of chloride ions into the mucus. The chloride channel is made up of a protein called CF transmembrane regulator. Its main function is to maintain the flow of chloride ions from the cell. Nothin is confusing to me about the role of CF. I have a good concept of what cystic fibrosis is, and how the channels function by having this disease.