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.
Treatments for Cystic Fibrosis depend upon the stage of the disease the individual is at. Drug therapy has been worked on for many years. The first 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 lungs. In 1995, it was shown that ibuprofen reduced the rate of lung inflammation in children if given at high doses under a controlled condition. Then in 1997, TOBI (tobramycin solution for inhalation) was developed. One of the best benefits of TOBI is that it is delivered straight to the site of the problem. As stated earlier, salt doesn't move properly out of the cells, so scientist are looking for a way to get rid of this. IN365 is being evaluated for its ability to stimulate the cells to secrete chloride (salt). This is turn should lessen the thickness of the mucus making it easier for a CF patient to breath and ward off infection.
Researchers through the years have tried to gain a
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