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Airway Surface Liquid Homeostasis in Cystic Fibrosis

   

Added on  2021-06-17

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CYSTIC FIBROSIS (CF)1Cystic Fibrosis (CF)NameDate

CYSTIC FIBROSIS (CF)2AbstractIntroduction Homeostasis is the maintenance of a constant internal environment of an organism; when this is achieved, the organs and organ systems of the organisms function optimally, ensuring health. However, homeostasis may be interrupted, leading to various conditions. In this brief abstract, the cystic fibrosis (CF) condition is discussedThis is an inherited genetic condition that mostly affects the lungs, as well as the liver, pancreas, intestines, and kidneys (Orenstein, Spahr, & Weiner, 2015, p. 14; Powell, 2013, p. 32). The long term effects include breathing difficulties and coughing mucus due to frequent infections of the lungs (Goss, Edwards, Ramsey, Aitken, & Patrick, 2009, p. 258; 'Milton S Hershey Medical Center', 2011). Other symptoms are sinus infections, fatty stool ('Healthline', 2018; McIntosh, 2018), poor growth and lack of weight gain despite normal nutrition, finger and toe clubbing, frequent infections of the chest, and shortness of breath or coughing (Langwith, 2009, p. 24; Bjorklund, 2009, p 12), and infertility in males (Lipshultz, Howards, & Niederberger, 2009, p. 274).Lung infections occur as a result of airways being clogged due to mucus build up and decreased mucociliary clearance that cause inflammation, further causing lung disease (Goss, Edwards, Ramsey, Aitken, & Patrick, 2009, p. 258). Because the pancreas can be damaged and yet it is responsible for insulin generation in the islets of Langerhans, the condition can cause elevated blood glucose levels (Atlas & Rosh, 2011). CF is inherited in an autosomal recessive manner due to mutations in both the gene copies for CFTR (cystic fibrosis trans membrane conductance regulator) protein (Orenstein, Spahr, & Weiner, 2015, p. 14). This happens because of the deletion of Δ in the CFTR ΔF508 in which three nucleotides are deleted that leads to the amino acid phenylalanine (F) being lost at the protein’s 508th position (Liu & Dawson, 2014, p. 5617). CFTR is involved in the production of digestive fluids, sweat, and mucus and when it is non functional as in cystic fibrosis, the secretions become thick. People with a single copy of this protein that is working are just carriers and remain normal. The condition is diagnosed using a sweat test and also genetic testing. There is no known cure for CF and the only method is management through treatment (Bjorklund, 2009, p 12). The main approaches to management include proactive airways infection treatment, proper nutrition, and a lifestyle that is active (Lukacs & Verkman, 2012). Throughout the life of the victim, pulmonary rehabilitation must be done with the aim of maximizing the function of organs and enhance the quality of life and antibiotics also help (Griese, 2010). Conclusion

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