Last modified: April 5, 2017
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Cystic Fibrosis is a hereditary disease that causes viscous and gummy mucus to accumulate in the lungs, digestive tract, and other parts of the body. It is one of the most common chronic lung diseases in children and young adults and life-threatening in its nature.
Cystic Fibrosis occurs due to the poor functioning of a protein called CTFR (or Cystic fibrosis Trans-membrane Conductance Regulator) that results in the accumulation of salt in the cells that form the lining of the glands in the respiratory tracts, sweat glands, pancreas and small intestine. The excess salt turns the usually dilute and slimy secretions of these glands into viscous and gummy as a result of which, the lubricating function of these secretions becomes ineffective and instead they block the passageways in these organs, thus causing the difficulties in the normal functioning of these organs.
The symptoms of the disease could vary from person to person, depending on the severity and age. Common symptoms include chest and respiratory infections, bowel blockages, greasy foul smelling stools, poor growth and malnutrition and a salty skin. The disease could also lead to the development of other medical problems like nasal polyps, sinusitis, cirrhosis of the liver and intestinal and renal malfunctions.
Even up to the present time, scientists have been unable to find a proper cure for Cystic Fibrosis. However, there are treatments that greatly improve the lives of the sufferers, by easing their problems and making their lives less painful. These treatments include the prevention and control of lung infections using medication like antibiotics, loosening and removing the viscous and gummy mucus from the lungs through Chest Physical Therapy that involves pounding or percussion of the chest and back using hands or devices, preventing or treating blockages in the intestines through medication or surgery, providing supplemental nutrition to prevent the effects of malnutrition and preventing dehydration, encouraging aerobic exercises that can loosen the mucus in the respiratory tracts because of the hard breathing that it causes, balancing the mineral content of the blood by replacing the salts that are excreted through mineral supplements.
Antibiotics, anti-inflammatory medicines, bronchodilators, or mucus-thinning medicines are the usual medications prescribed for Cystic Fibrosis. Depending on the severity of the infection, oral, inhaled or intravenous antibiotics are used for fighting the infections caused by the bacteria Pseudomonas that thrive in the mucus. Inhaled or oral anti-inflammatory medicines are prescribed for reducing the swelling in the airways die to the infections. Bronchodilator medicines relax the muscles in the respiratory tracts and aid in open the airways. Mucus thinners help in thinning the mucus secretions and improve lung function.
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