Paper Example on Cystic Fibrosis

Introduction

Cystic fibrosis is an inherited disorder that causes damage to the digestive system and lungs among other organs especially those with epithelial tissues. The disorder affects only the body cells that produce the digestive juices, mucus, and sweat. The fluids produced from these organs are thin and slippery in nature as they are used as a lubricant but a defective gene causing cystic fibrosis, cause sticky thick secretions that plug up the tubes hence not acting as a lubricant (Farrell, & White 2017).

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Epidemiology is a medical science that deals with the control and transmission of diseases in different groups of people. According to MacNeill, Cystic fibrosis is usually detected in early childhood, in most cases symptoms for a chronic lung disease can be diagnosed later in life. Statistics show that about thirty-eight percent of individuals suffering from the disease are eighteen years or older while the median survival age for people suffering from this disorder are around thirty years. Cystic fibrosis have been identified as one of the common genetic disorder in Caucasians even if it is known to affect all ethnic groups and races. However, the disorder is less common in Hispanic, Americans, Africans, and Asians and the individuals with cystic fibrosis must inherit at least one gene of the disorder from each of the parent.

Pathophysiology refers to the study of the biological and physical abnormalities occurring within the body organs as a result of a disease. The abnormality 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 (Borowitz 2015).

Abnormality of the protein blocks the movement of water and chloride ions in the lungs and other body cells as well as causes these cells to secrete abnormal thick mucus. A patient can lose excess salt hence cause abnormal heart beats or rhythms. 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.

Purulent secretions lead to bronchial wall thickening causing the obstruction. Recurrent productive cough causes; first, bronchiectasis- causes increased antero- posterior 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. Fourth, infertility in men, end up with an absence of the vas deferens' congenital bilateral with the azoospermia.

Nursing care interventions include; first, assess 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, positioning of the patient in the recovery position to facilitate easy removal of secretions. Fourth, suction of excess secretions. Fifth, teaching the patient to practice 'pursed lip breathe coughing. Sixth, provide emotional support as most acute respiratory episodes can cause fear and anxiety. Seventh, explaining medications being administered to decrease anxiety and foster treatment understanding.

Pharmacology interventions will assist 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 are nausea, vomiting, loss of appetite and stomach upset which diminishes after sometimes (Bell, De Boeck, & Amaral 2015). Mucomyst- is given in a nebulizer to decrease the viscosity of the sputum and promotes expectoration of the secretion, for example, N-acetylcysteine whose common side effects are urticarial and itchiness.

Corticosteroid drugs are the medication used to relief the inflamed body areas by lessening sells, allergic reactions, itching, and redness. Commonly, these drugs are prescribed during the last diseases of the disease or during 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. Regular vaccination against pneumococcal infections and diseases annual influenza vaccination is required to prevent an occurrence of cystic fibrosis.

Patient education requirement involves; First, to reduce exposure in crowds harboring possible infections and to people with infections. Second, teaching the patients on early signs and symptoms of respiratory infections and disease progression that indicates the needs to notify a primary health care provider. Third, encourage the patient to take adequate fluids and dietary intake to promote the removal of secretions and to ensure nutritional status. Fourth, genetic counseling on family history with cystic fibrosis especially in those couples seeking prenatal testing and planning of pregnancy.

Primary health care issues include; first, conduction of seminars for people with cystic fibrosis which is mainly to complement the care provided by specialized of cystic fibrosis services at each life stage. Second, typically taking care of patients with cystic fibrosis at specialized centers by multidisciplinary teams. Third, addressing health concerns not specific to cystic fibrosisdeterioration 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, minimizing respiratory exacerbation and deteriorating pulmonary functions through treatment in consideration of patients suffering from cystic fibrosis disorder (Boyle, & De Boeck 2013).

Conclusion

Through a vast study on cystic fibrosis, it's a chronic, progressive and fatal autosomal recessive disease that affects the respiratory and digestive systems. 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 counseling. Recognition of cystic fibrosis in the early stage followed by a comprehensive multidisciplinary therapy and improved symptom control can help increase the survival chances. Patients and their families are recommended to transfer to a regional cystic fibrosis center and foundations.

References

Bell, S. C., De Boeck, K., & Amaral, M. D. 2015. New pharmacological approaches for cystic fibrosis: promises, progress, pitfalls. Pharmacology & therapeutics, 145, 19-34.

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.

Chaudhry, S. R., Keaton, M., & Nasr, S. Z. 2013. Evaluation of a cystic fibrosis transition program from pediatric to adult care. Pediatric Pulmonology, 48(7), 658-665.

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.