Cystic Fibrosis, What is It

Cystic Fibrosis is an inherited disease that affects the tissues that produce mucus secretions. These tissues can be found in the lungs, gastrointestinal tract, pan crease, and the ducts of the liver.Cystic fibrosis can also affect the sweat glands and the male reproductive system. People with the disease have abnormally thick secretions in the lungs as well as other organs.
It was originally thought by researchers in the late eighties, that patients with cystic fibrosis had a faulty chloride channel, a gate in the cell membrane that regulates the entry of chloride. They proved through their research that the epithelial cells, which lined the body's airways, contained this malfunctioning channel. The lack of a correctly working chloride gateway results in airway surface fluid that is loaded with salt, hence, one of the symptoms of cystic fibrosis, salty skin. Researchers suspected that this faulty channel lead to the heavy mucus that blocked the airways path, eventually leading to death.
It wasn't until the nineties when researchers found that cystic fibrosis is caused by mutations in a protein called CFTR (cystic fibrosis transmembrane conductance regulator), those mutations being that proteins are absence in the cell membrane. Normally this protein, CFTR, would pump chloride ions across the cell membrane. This is not happening due to the absence of CFTR. The absence of this protein leads to the thick build up of mucus leading to frequent respiratory infections that are so characteristic of the cystic fibrosis disease.
Treatments for Cystic Fibrosis depend upon the stage of the disease the individual is at.Drug therapy has been worked on for many years. Thefirst new drug therapy developed was approved by the FDA in 1993.This gave patients the ability to take a drug called Pulmozyme. This drug reduced the number of respiratory infections and improved the functions of the lung

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