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CYSTIC FIBROSIS By (Student’s Name:)

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Pathophysiology Pathophysiology refers to the study of the biological and physical abnormalities occurring within the body organs as a result of a disease. The anomaly in cystic fibrosis results from a mutation in the protein-excreting gene that is found in the cells lining of the colon, pancreas, lungs, and the genitourinary tract and is responsible for transporting the sodium and chloride ions through the cell membranes (MacNeill, 2015,20). Pathophysiology further describes the functional changes that tend to occur within an individual as result of pathologic conditions and also diseases, it’s a crucial area of study applied in most of the healthcare professional learning institution programs, an example; medical, paramedic programs, radiologic science, nursing, chiropractic programs, pharmacy, dental and radiation therapists (Borowitz, 2015, 50). Impact on a patient’s lifestyle and activities of daily living Abnormality of the protein blocks the movement of water and chloride ions in the lungs and other body organs as well as causing abnormal secretion of thick mucus by these cells. A patient can lose excess salt a condition which results in irregular heartbeat or rhythm. The produced thick mucus then accumulates in the pancreas, lungs, intestines, liver, and the reproductive system, causing frequent infections in the respiratory system, lung diseases, poor growth, infertility, diabetes, and malnutrition among other health disorders. (Quinton, 2010, 1222) Purulent secretions lead to bronchial wall thickening causing the obstruction. Recurrent productive cough causes; first, bronchiectasis- causes increased anteroposterior chest circumferences, hyper-resonance with percussion and apical crackles on auscultation due to permanent abnormal dilation and destruction of the bronchial wall. Second, impaired gas exchange resulting from hypoxemia, hypercapnia due to an infestation of the airways with pathogenic bacteria. Third, decreased or reduced exercise tolerance, muscles weakness, recurrent infection facial sinus, tenderness and purulent nasal discharge (Boyle, & Boeck, 2013, 158). Treatment programs of patients with this disease mostly focus on several areas, such as the psychological support, clearance and reduction of lower airway secretions, pulmonary rehabilitation program, adequate fluid and dietary intake, surgery, genetic and occupational counselling. Fourth, infertility in men, end up with an absence of the vas deferens’ congenital bilateral with the azoospermia (Quinton, 2010, 1222). Nursing care interventions Interventions on nursing care to the patients include; first, assess to the history of symptoms or circumstances to provide a possible trigger to the respiratory response. Second, Administration of supplemental oxygen to treat the progressive hypoxemia. Third, the positioning of the patient in the recovery poses to facilitate easy removal of secretions (MacNeill, 2015, 20). Fourth, suction of excess secretions. Fifth, teaching the patient to practice pursed lip breathes coughing. Sixth, provide emotional support as most acute respiratory episodes can cause fear and anxiety. Seventh, explaining medications administered to decrease anxiety and foster treatment understanding (Borowitz, 2015, 50). Pharmacology interventions Pharmacological interventions perform fundamental roles in the determination of drugs to be used, the mode of action and the side effects: Antibiotics- to prevent and treat chest infections, for example, gentamicin treat severe bacterial infections found in various parts of the body, and its common side effects include; nausea, vomiting, loss of appetite, chills, cramps, and also abdominal pains which diminish after some time (Boeck, &Amaral, 2015, 158). Mucomyst- is given in a nebuliser to decrease the viscosity of the sputum and promotes expectoration of the secretion, for example, N-acetylcysteine where its common side effects are urticarial, itchiness, nausea, vomiting and there are signs of mouths sore as well as running nose, a condition which occurs when the patient is inhaling this medication by mouth. Patients are advised in a situation where the disease persists should inform the doctor or pharmacist promptly (Borowitz, 2015, 50). Corticosteroid drugs are the medication used to relieve the inflamed body areas by lessening sells, allergic reactions, itching, and redness. Commonly, these drugs are prescribed during the last stages of the disease or severe respiratory exacerbations as they help reduce the edema of the inflammatory airway. Its common side effects are hypertension, osteoporosis in fragile bones, diabetes and thinning of the skin. Ibuprofen drug-reduces the rate of deterioration in pediatric patients with milled disease of cystic fibrosis (MacNeill, 2015, 20). Regular vaccination against pneumococcal infections and diseases annual influenza vaccination is required to prevent an occurrence of cystic fibrosis. Recognition of cystic fibrosis in the early stage followed by a comprehensive multidisciplinary therapy and improved symptom control can help increase the survival chances (Boyle, & Boeck, 2013, 159). Patient education requirements Essential requirements for educating patients involves; First, to reduce exposure in crowds harbouring possible infected and to people with infections. Second, teaching the patients on early signs and symptoms of respiratory infections and disease progression that indicates needs to notify a primary health care provider. Third, encourage the patient to take adequate fluids and dietary intake to promote the smooth of secretion and to ensure nutritional status. Fourth, genetic counselling on family history with cystic fibrosis especially in those couples seeking prenatal testing and planning of pregnancy (MacNeill, 2015, 20). Primary health care issues Fundamental healthcare issues include; first, conduction of seminars for people with cystic fibrosis, which is mainly to complement the care provided by specialised of cystic fibrosis services at each life stage. Second, typically taking care of patients with cystic fibrosis at specialised centres by multidisciplinary teams (MacNeill, 2015, 20). Third, addressing health concerns not specific to cystic fibrosis deterioration, mental and sexual health contraceptive advice in an area that has lacked attention. Fourth, providing survival means to patients by regarding cystic fibrosis as the chronic multisystem disease for adolescence and young adulthood. Fifth, minimising respiratory exacerbation and deteriorating pulmonary functions through treatment in consideration of patients suffering from cystic fibrosis disorder (Boyle, & Boeck, 2013, 160).

References

Borowitz, D. 2015. CFTR, bicarbonate, and the pathophysiology of cystic fibrosis. Pediatric pulmonology, 50(S40), 2S4-S30. Boyle, M. P., & De Boeck, K. 2013. A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect. The Lancet Respiratory Medicine, 1(2), 158-163. Farrell, P. M., & White, T. B. 2017. Introduction to “Cystic Fibrosis Foundation Consensus Guidelines for Diagnosis of Cystic Fibrosis”. The Journal of pediatrics, 181, S1-S3. Knudsen, K. B., Pressler, T., Mortensen, L. H., Jarden, M., Skov, M., Quittner, A. L.,&Boisen, K. A. 2016. Associations between adherence, depressive symptoms and health-related quality of life in young adults with cystic fibrosis. SpringerPlus, 5(1), 1216. MacNeill, S. J. 2015. Epidemiology of cystic fibrosis. Hodson and Geddes’ cystic fibrosis, 4, 19-20. Mogayzel Jr, P. J., Naureckas, E. T., Robinson, K. A., Mueller, G., Hadjiliadis, D., & Hoag, J. B. 2013. Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. American journal of respiratory and critical care medicine, 187(7), 680-689. Quinton, P.M., 2010. Role of epithelial HCO3− transport in mucin secretion: lessons from cystic fibrosis. American Journal of Physiology-Cell Physiology, 299(6), pp.C1222-C1233.