Cystic Fibrosis Research Paper

Cystic fibrosis is a chronic lung disorder caused by an autosomal recessive multiorgan disorder. The disorder is due to an exocrine gland dysfunction. The deficiency in chloride flow and increase sodium absorption results in thick sticky mucous that clogs the lungs and obstruct glands The pancreas obstruction leads to the failure of secretion of enzymes. The chronic lung disorder is prevalent amongst Caucasians. “Cystic fibrosis occurs in one of 2,5000Caucasians,approximately 30,000 individuals most are diagnosed by six months of age a are living in the United States”(DNA Testing In Prenatal Case, Cystic FIbrosishttp://www.ncbi.nlm.nih.gov/ pmc/articles/PMC1200714/). “12 million more Americans are carriers but not affected by it”(Plain patient
The gene is found on chromosome 7, and controls the secretion of chloride, sodium, and bicarbonate ions across the epithelium tissues. The gene is required to regulate the components of sweat, digestive juices, and mucous. The product of the gene is a halide anion channel that contains two ATP-hydrolyzing domains, which allows protein to be used as energy in the form of ATP. Only one normally functioning gene is required to prevent cystic fibrosis. The disorder develops when neither of the two genes works appropriately. This malfunction of the gene is why cystic fibrosis is considered an autosomal recessive disease. If both parents are carriers, each parent can pass on the dysfunction copy or the normal copy to their children. Children who inherit two dysfunctional copies of the CFTR gene are affected and have the disease cystic fibrosis. There are multiple theories of why the mutation occurs. For an example F508 creates a protein that doesn’t fold normally and is disregarded by the cell. If the proteins are not the correct size the production is ended abruptly. Mutations also lead to fewer copies of the CTFR gene production. When the gene doesn’t work, chloride and thiocynate are trapped inside the cells in the airway and outside the skin. Chloride is negatively charged, positive charged cations cross into the cell. Sodium is the extracellular space cation and the
There is no prescribed treatment to eradicate the condition but prevention of respiratory infections, malabsorption problems and maintaining good nutrition and an active lifestyle can aid individuals in living a sustainable life. The goal for all patients should be maintaining their quality of life. Since the condition is diagnosed before birth parent teaching is vital in managing cystic fibrosis. Education on medication regimen of bronchodilators, nebulizer treatments and all respiratory treatments. Administer pancreatic enzymes before all meals and snacks. Fat-soluble vitamins replacement. Identifying the signs and symptoms of infection is important; monitoring their vital signs, temperature, listening to their chest, nasal flairing, chest retraction, cyanosis color, persistent cough and wheezing. All abnormal symptoms are to be notified to the physician and child should be brought in for further evaluation. As adolescents the cystic fibrosis patient should engage in sports, activities, avoidance of drugs, smoking and alcohol. Encourage all women with the disorder to participate in genetic testing. The prognosis of Cystic fibrosis has improved. For an example the U.S Cystic Fibrosis Foundation Complies that in 2008 the life expectancy has improved to 37.4 years old and 240 American with cystic fibrosis were able to become pregnant. The Priority Nursing Diagnosis’s are: Ineffective airway clearance related to