Pathology of Cystic Fibrosis

Pathology of Cystic Fibrosis Cystic fibrosis is an inherited condition that affects the exocrine glands leading to severe damage to the respiratory system, the digestive system and various organs of the body. This disorder has a detrimental impact on the cells that secrete mucus, digestive juices, and sweat.

Under normal circumstances, these secretions are thin and slippery. However, in cystic fibrosis, the secretions become thick and sticky. Rather than these secretions acting as a lubricant, they plug up tubes, passageways, and ducts, particularly in the pancreas and lungs.

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An understanding of the pathophysiology of cystic fibrosis is important since it helps determine the most effective treatment intervention to initiate for a patient failure (Haack, Aragão, & Novaes, 2013). This paper will highlight the basic pathophysiology of cystic fibrosis and a potential treatment plan for a person with the disease.

Pathophysiology Cystic fibrosis is a genetic disorder associated with a gene mutation. The gene associated with this disorder is referred to as the cystic fibrosis transmembrane conductance regulator (CFTR) (Mayo Clinic, 2018). This mutation results in extreme changes in phenylalanine the protein involved in the regulation of salt movement in and out of cells (Horsley et al., 2015). The severity of this disorder is related to the type of gene mutation that occurs in an individual. To develop the disease children must inherit a copy of the gene from both parents. Pathological changes associated with cystic fibrosis occur in the respiratory system, hematopoietic system, endocrine system, gastrointestinal system and the reproductive system (Mayo Clinic, 2018).

Cystic fibrosis leads to the secretion of thick, sticky mucus that clogs airways thus preventing the free flow of air into and out of the lungs (Haack et al., 2013). The alveoli may become expanded due to air trapping which over time results in a barrel-shaped chest. The increased parenchymal arterial pressure emanating from the destruction of pulmonary parenchyma may eventually lead to right-sided heart failure (Haack et al., 2013).

Changes in the protein phenylalanine are also believed to negatively affect the capacity of the pancreas to secret and supply digestive enzymes to the intestine. The thick mucus associated with this condition obstructs pancreatic ducts further limiting the volume of secreted enzymes (Haack et al., 2013).

Treatment Plan

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Since there is no cure for cystic fibrosis, treatment for this disorder is aimed at alleviating symptoms and reducing complications. Early diagnosis and treatment are recommended to improve the quality of life of the patient. The treatment for this disorder should employ a multidisciplinary approach due to its nature of affecting more than one body systems (Mayo Clinic, 2018). The primary goals of cystic fibrosis treatment are to prevent and control lungrelated infections, remove and loosen mucus in the lungs, open up blocked ducts and provide adequate nutrition (Mayo Clinic, 2018). The patient may be administered with a combination of medications including antibiotics for treating infections, anti-inflammatory drugs, and bronchodilators. Thick Mucus in the respiratory system can be loosened through chest physical therapy (Mayo Clinic, 2018).

Conclusion Cystic fibrosis is an inherited condition that affects the exocrine glands leading to severe damage to the respiratory system, the digestive system and various organs of the body. This condition is typified by the secretion of thick, sticky mucus that clogs and blocks passages and ducts in the body. Since there is no cure for cystic fibrosis, treatment for this disorder is aimed at alleviating symptoms and reducing complications.

PATHOLOGY OF CYSTIC FIBROSIS

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Haack, A., Aragão, G. G., & Novaes, M. (2013). Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases. World Journal of Gastroenterology, 19(46), 8552–8561. doi: 10.3748/wjg.v19.i46.8552 Horsley, A., Cunningham, S., & Innes, J. A. (2015). Cystic fibrosis. Oxford, UK: Oxford University Press.

Mayo Clinic. (2018). Cystic fibrosis. Retrieved from https://www.mayoclinic.org/diseasesconditions/cystic-fibrosis/symptoms-causes/syc-20353700