Introduction/Overview of the Respiratory System
The human respiratory system is a crucial, yet extremely exposed system within the body. The exposure is based on the fact that the respiratory system is in constant use, which exponentially limits the ability to regulate what goes into it. The respiratory system is the means through which the human body performs gaseous exchange between the atmosphere and the blood (Blahd, 2017). The respiratory system begins in the nose and ends inside the two lungs. More specifically, it involves the nasal cavity and sinuses which are inside the nose (Zimmermann, 2018). As it goes down it involves the pharynx which is behind the cavity of the mouth, then the larynx which is in the cavity of the throat. The trachea, which is inside the chest cavity follows the larynx. Lower in the chest cavity, the trachea will branch into two bronchi, each of which enters one of the two lungs. Inside the lungs, the bronchi will break into a collection of bronchioles each of which connects into an alveolus (Zimmermann, 2018). The alveoli are blood rich air sacks where the actual gaseous exchange takes place with Carbon dioxide leaving the blood and being replaced with Oxygen. To get to the Alveoli, oxygenated atmospheric air moves down the organs of the respiratory system in the order described above while carbon dioxide moves out of the Alveoli and up the same path to be released through the nose. The entire respiratory system from the inner nose to the lungs is covered by a very thin layer of mucus known as the airway surface liquid (ASL) (Tang et al., 2016). The obligation of the ASL is to keep the respiratory pathway moist, clean and devoid of injury from the chemicals and debris constantly passing through it.
Cystic Fibrosis
Cystic Fibrosis is a genetic disorder and disease that affects the ASL alongside other mucous membranes in the body. Cystic Fibrosis is caused by a genetic mutation that causes a defect in the CFTR gene (NIH, 2018). The defect affects the movement of salt in and out of cells. Being a genetic mutation, the causation of cystic fibrosis is most prevalent among individuals whose close family members also have the disease. Further, the disease has a racial bias with Northern European Caucasians having a higher propensity for getting the disease than other races (Mayo Clinic, 2016). The genetic causation is also a major bearing factor in the prevention of the disease as it is hard to resolve genetic disposition (Cutting, 2015). A common preventative means involves parents whose children may be susceptible to cystic fibrosis abstaining from having children upon confirmation of the probability through genetic tests (Mayo Clinic, 2016).
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The salt imbalance affects the general homeostasis of the ASL such as interfering with the PH of the ASL. The homeostasis of the ASL is crucial in ensuring the functionality of the membrane (Tang et al., 2016). ASL must be capable of keeping the respiratory system clean and free of inordinate infection or injury while contemporaneously not interfering with the continuous process of breathing, respiration, and gaseous exchange (Muhlebach et al., 2018). The impact of cystic fibrosis will either cause the thinning or overcompensation of the ASL thus limiting its capability to protect the lungs or causing it to interfere with the process of breathing both of which will have adverse ramifications on the patient (NIH, 2018). Possible complications include bronchiectasis with physical damage to the respiratory system, acute infections on respiratory organs, and pneumothorax where air collects inside the chest cavity outside the lungs (Muhlebach et al., 2018). In extreme cases, cystic fibrosis can cause respiratory failure and eventual death. Diagnosis depends on the age of the patient. Susceptible children are tested through genetic testing or testing the sweat or blood of the child (Tang et al., 2016). Older people who have symptoms such as chronic chest infection and nasal polyps are also encouraged to undergo testing. Diagnosis for adults is through genetic and sweat tests. As there is no cure for cystic fibrosis, treatment and management are symptomatic (Mayo Clinic, 2016).
Effects on Other Body Organs
Cystic Fibrosis is not just a disease of the respiratory system as the condition affects all mucous membranes. Two other body systems that rely on the homeostasis of mucous membranes are also affected by cystic fibrosis. These are the reproductive system and the digestive system. In the digestive system, the illness causes the mucous membranes to thicken thus inhibiting access to different digestive juices leading to poor digestion (Li & Somerset, 2014). Constipation, weight loss, and smelly stools are some of the symptoms of this effect. In the Reproductive system, cystic fibrosis increases or eliminates the mucous membranes in the vas deferens and prostate gland leading to infertility in men (Averill et al., 2015). Increasing or reducing mucus membranes in women reduces their capacity to conceive but not as severely as in men (Anderson et al., 2015).
Conclusion
Humans need to breath at all times, even when in dusty, smoky or polluted environments. A complex mucus membrane, the ASL, protects the respiratory system from injury from the debris and chemicals, or from infection by the germs prevalent in the air breathed in. Cystic fibrosis is a genetic disease that affects the homeostasis of the ALS by either limiting its ability to protect the respiratory system or causing it to interfere with respiration. The disease also affects the reproductive system, leading inter alia to infertility and the digestive system leading to indigestion. The disease is genetically predisposed and cannot be cured. Diagnostic tests are commonly undertaken on vulnerable children and symptomatic adults who, if diagnosed with the condition are placed on symptomatic treatment. The genetic disposition of cystic fibrosis, its lack of treatment, and the various adverse effects creates an urgent need for better research in its causes, prevention, and management.
References
Anderson, R., Pearce, A., Graham, C. A., & Ingham, R. (2015). 289 How do men and women with cystic fibrosis think their illness and associated experiences affects their body image, sexuality, relationships and their ideas about parenthood?. Journal of Cystic Fibrosis , 14 , S132
Averill, S., Lubner, M. G., Menias, C. O., Bhalla, S., Mellnick, V. M., Kennedy, T. A., & Pickhardt, P. J. (2017). Multisystem Imaging Findings of Cystic Fibrosis in Adults: Recognizing Typical and Atypical Patterns of Disease. American Journal of Roentgenology , 209 (1), 3-18
Blahd, W. (2017). How the lungs and respiratory system work. Retrieved from https://www.webmd.com/lung/how-we-breathe
Cutting, G. R. (2015). Cystic fibrosis genetics: From molecular understanding to clinical application. Nature Reviews Genetics , 16 (1), 45–56. doi: 10.1038/nrg3849
Li, L., & Somerset, S. (2014). Digestive system dysfunction in cystic fibrosis: challenges for nutrition therapy. Digestive and Liver Disease , 46 (10), 865-874
Mayo Clinic. (2016, October 13). Cystic fibrosis. Retrieved from https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700
Muhlebach, M. S., Zorn, B. T., Esther, C. R., Hatch, J. E., Murray, C. P., Turkovic, L., ... & Wolfgang, M. C. (2018). Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children. PLoS Pathogens , 14 (1), e1006798
NIH. (2018). Cystic Fibrosis. Retrieved from https://www.nhlbi.nih.gov/health-topics/cystic-fibrosis
Tang, X. X., Ostedgaard, L. S., Hoegger, M. J., Moninger, T. O., Karp, P. H., McMenimen, J. D., ... & Welsh, M. J. (2016). Acidic pH increases airway surface liquid viscosity in cystic fibrosis. The Journal of Clinical Investigation , 126 (3), 879-891
Zimmermann, K. A. (2018, February 12). Respiratory system: Facts, function and diseases. Retrieved from https://www.livescience.com/22616-respiratory-system.html
