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The implications of the hypothesis proposed in the science article may eventually lead us to a cure for Cystic Fibrosis. Before we examine the possibility and effectiveness of future drugs that might be used to cure this deadly disease, let us take a look at some of the conventional treatments administered to patients suffering from Cystic Fibrosis.
Although there is no cure for cystic fibrosis, doctors have developed treatments which can add years to their patients' lives. Typically, a patient with cystic fibrosis lives to his early to mid-twenties. However, treatments have advanced so that those born today with this disease are expected to live up to their forties (Purves et. al 1995).
The treatments for cystic fibrosis are used to remedy only the symptoms such as respiratory and digestive difficulties, infections due to excess mucus in the lungs, etc. Therapies involving chest thumping, exercises, nighttime mist tents and aerosols, and pills with every meal to aid digestion and prevent infections are done on a daily basis. However, more treatments are forthcoming and appear promising, such as the administration of amiloride studied in the Summary of the Article.
One of the most common treatments involves thumping the chest of a CF patient to clear lungs of mucus, also known as "bronchial drainage" (CF-FAQ Part 1 1996). The vibration of the chest wall loosens the accumulation of mucus in the lungs, thereby helping the patient to breathe more easily.
CF patients are also advised to cough and complete breathing exercises that also loosen the mucus and help to strengthen thoratic muscles. Even total-body exercises can help CF patients. By working out, these patients are able to decrease problems with shortness of breath and deterioration of muscles in addition to loosening the mucus in their lungs (CF-FAQ Part 1 1996).
Other common treatments involve the use of nighttime mist tents and aerosols made with saline, antibiotics, and/or special aerosol medications. Some of these medications can stimulate the cilia in the lungs to be more active, thereby sweeping out more mucus, or they can relax the muscles in the bronchial tube. The mist also adds water to the lungs, thus thinning the mucus to a more normal viscosity. Thinning out the mucus also makes it a little easier to remove the problematic substance.
Many CF patients are also given pills, which supplement missing digestive enzymes from the pancreas, to aid in digestion. Sometimes, it may be nessicary for these patients to have special diets in which they have smaller meals and more snacks which are low in bulk and fat content. Enzymatic therapy is also used; this treatment involves the administration of enzymes which break up the strands of DNA that are part of the mucus (CF-FAQ Part 1 1996).
Antibiotics are used as treatment as well, due to the increased likelihood of contracting an infection in the lungs because of the presence of the mucus, a prime breeding ground for bacteria. Antibiotics also serve to ward off viral infections. It is important that CF patients do not fall victim to diseases like the measles, which can destroy the cilia in the lungs, compounding problems with breathing (Warwick 1996).
The study completed in the article, "CFTR as a cAMP-Dependent Regulator of Sodium Channels" is significant because it leads to the possibility of using amiloride to block the overproduction of sodium channels, thereby decreasing the hyperabsorbtion of solute in the airway tissue (Wright 1996); the mucus in the lungs of CF patients could return to normal. Also, thinning of the viscous fluid will make it easier to remove by coughing or by the sweeping action of the cilia.
The use of amiloride as a form of treatment is significant because amiloride actually helps the body in correcting the chloride and sodium ion concentration, thereby rehydrating the cells and thinning the mucus. This process differs from current treatments which loosen the mucus or dilute it so the patients can rids their lungs of the infectious substance more easily.
