31 Aug 2022

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Cystic Fibrosis (CF): Causes, Symptoms & Treatment

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Cystic Fibrosis (CF) is currently one of the main genetic disorders that are affecting thousands of people in the United State of America (USA) and various parts of the world. CF is caused by a defect on a gene on chromosome 7 whose main role is to encode the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) ( Agrawal et al ., 2017) . Any defect in CFTR results in a buildup of mucus on the lungs and pancreas, which may lead to a fatal lung infection ( Agrawal et al ., 2017) . Consequently, a person may find it difficult to breathe due to lung damage or respiratory failure. Some of the major health problems that are associated with CF include sinus disease, intestinal obstruction, and liver diseases ( Agrawal et al., 2017) . Also, there are cases where men with CF have been diagnosed with male infertility ( Agrawal et al ., 2017) . A significant number of people are suffering from CF in the USA. According to Agrawal et al. (2017), more than 30,000 of people who live in the USA are suffering CF compared to the global figure of 70,000. The USA is leading in terms of the people with CF, making the disorder a significant public health concern and a huge financial burden for families across the country. 

Even though CF is believed to be a rare disease, it has significant social and economic implication on individuals and the country as a whole. Apart from affecting the general health and wellbeing of affected persons, it also destabilizes patients’ families due to long hospitalizations and use of a lot of resources (Hassan, Bonafede, Limone, Hodgkins, & Sawicki, 2018). Patients who have been diagnosed with CF spend at least 20 days in hospitals (Hassan et al ., 2018). High health resource utilization can lead to poverty among affected families. However, the primary concern is the increasing cost of managing CF. The average cost of CF care per individual in the USA is estimated to be $48,000, which increases with the disease severity (Vadagam & Kamal, 2018). For instance, at severe stages of the disease, managing CF can cost about $343,900 annually (Vadagam & Kamal, 2018). CF is costly, and it can lead to adverse financial implications for families. Thus, CF should be explored due to its substantial social and economic implications. 

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Modern medical technologies have helped in managing CF in the USA. Specifically, telehealth medical technologies have been largely and successfully used to monitor the progress of patients remotely, especially regarding how they comply with the medication requirements ( Tagliente, Solvoll, Murgia, & Bella, 2016) . Telemonitoring has reduced the cost of managing CF while at the same time it saves time ( Tagliente et al ., 2016) . This is because the patients don’t spend time and money to visit the doctors at the health facilities, but rather receive consultation and care as a follow-up to their initial visit and treatment at the hospital ( Tagliente et al ., 2016) . Nonetheless, apart from the Medicaid program, state and federal government does not have other initiatives aimed at addressing the problems of CF. Therefore, CF is one of the most complicated genetic disorders that should be explored to find ways of reducing its social and economic impact, as well as its adverse effect of health and wellbeing of patients. The term paper helps the public in understanding the disease, which is now affecting a significant number of the US population. Importantly, the paper provides useful information that can be used by people to effectively manage CF while reducing the negative social and economic implications. Cystic fibrosis is a genetic disorder that affects millions of American citizens, either directly or indirectly, and its adverse effects can be reduced through the use of medical technologies and effective government programs. 

The many types of complication that are associated with CF are one of the reasons why it regarded as a complicated and dangerous genetic disorder. Unlike other disorders that have a few complications, CF's pathophysiology is characterized by many health problems. A significant number of people who are suffering from CF are likely to experience respiratory failure, mainly because of pneumonia ( Rafeeq & Murad, 2017) . At the same time, an increased obstruction of airway affects the functioning of the respiratory system because the air cannot easily pass during the exhalation. The problem in the respiratory system is caused by a defective CFTR, which releases sticky and thick mucus that blocks the pathway ( Rafeeq & Murad, 2017) . Consequently, patients with CF are vulnerable to lung infections, particularly pseudomonas. Also, the infiltration of neutrophil leads to the massive release of elastase that destroys the surrounding tissues. The defective neutrophil also releases a lot of nucleic acids that results in mucus hyper-viscosity ( Rafeeq & Murad, 2017) . Therefore, CF mainly affects the respiratory system, resulting in further health complications. Also, it can cause digestion abnormalities and the imbalance of minerals in human blood because of the excessive loss of salt, leading to health problems like fatigue and dehydration. 

CF is one of the diseases that do not have a cure. Nonetheless, many treatment methods are currently being used to reduce its complications. According to Rafeeq & Murad (2017) , to minimize complications and infections in the respiratory system, antibiotics such as azithromycin and levofloxacin can be used to treat CF. At the same time, oral rehydration can be used to prevent intestinal blockage. Physiotherapy to clear airway secretions is another common treatment method for CF ( Edmondson & Davies, 2016). Physiotherapy is associated with the improved prognosis of CF for the last two decades, especially because of its effectiveness. Besides, people with CF are encouraged to engage in regular physical exercise to reduce the effect of the disorder on various body systems. Patients with CF are also encouraged to take food rich in supplemental vitamins ADEK, fluoride, and Zinc ( Rafeeq & Murad, 2017). Hence, many interventions are used to treat CF. 

Apart from many complications and health problems, CF is linked to the substantial social and economic impact on individuals, families, government, and society at large. Hassan et al . (2018) argue that treating and managing CF require intensive health care resources, which may lead to both social and economic impact. The high cost of medication and health care services and long hospitalization of patients with CF leads to family instability (Hassan et al ., 2018). Families whose members are suffering from CF are always dysfunctional because of financial constraints and social stress, including depression, and reduced satisfaction. In the case of a young patient with CF, the family is likely to experience the feeling of guilt due to isolation. Marriage breakups or instability is also common when a child has been diagnosed with CF. The treatment and management of CF demand a lot of time and finance from parents, which may result in reduced affection and intimacy among couples. The average days of hospitalization for patients who are suffering from CF are 20 days that is likely to increase based on the severity of the disease (Hassan et al ., 2018). CF, therefore, affects the stability of families and marriages. 

The economic impact of CF is a problem faced by families and the government due to the high cost of treating and managing the disorder. According to Vadagam & Kamal (2018), the global average annual cost of taking care of a patient who is suffering from CF is 22,102, and the hospitalization bill accounts for about 75%. Comparably, in the USA, the average cost for treating CF per patient is $48,000, which is a clear indication that the disorder is causing a financial burden among many affected Americans (Vadagam & Kamal, 2018). The cost of treating and managing CF is not statistic as it increases with the severity of the diseases. Besides, the cost is affected by other factors such as age and the body systems affected by the disorder. For instance, based on the analysis that was done by Van Gool, Norman, Delatycki, Hall, & Massie (2013), the cost of CF in treating patients with poor lung functioning is seven times compared to their counterparts with normal function lungs. Also, children who are diagnosed with CF impose higher economic burden than older patients (Vadagam & Kamal, 2018). As a result, the main economic impact of CF is the increased financial burden on family members who are expected to foot hospital bills and other health care costs. A Family is likely to end up in poverty when one of its members is diagnosed with CF. 

However, the social and economic impact of CF can be reduced through the use of modern medical technologies. Telemedicine is one of the technologies that have improved the treatment and management of CF significantly. Gur, Nir, Telesho, Bar-Yoseph, Manor, Diab, and Bentur (2016) argue that effective treatment of CF can only be received in certain specialized health centers, especially in large cities. As a result, the geographical barrier is hindering the treatment of CF. The problem of the geographical barrier can be overcome through the use of telemedicine technologies. Specifically, according to Gur et al . (2016), cell phone application such as SMS and MMS can conveniently and cost-effectively be used to enhance self-management of CF while acting as medication reminders and a source of supportive messages. The use of telemedicine increase medication adherence, and it reduces the cost of CF treatment substantially ( Tagliente et al ., 2016). Adherence to medication has been one of the major problems in managing and treating CF, and it has mostly been solved by telemedicine. Telemedicine can be used to target young patients who are techno-savvy yet find it challenging to manage CF. The use of mobile phone applications improves communication between patients and health professionals, leading to improved health outcomes. Besides, telemedicine helps in monitoring symptoms. Telemedicine, therefore, is essential in the treatment and management of CF. 

Although CF is one of the significant public health concerns, both federal and state governments are doing little to address the problem. Medicaid is the main program that has been initiated by the federal government to address the problem. Unfortunately, Medicaid only covers a small percentage of people who are suffering from CF. Even though the federal government is determined to ensure that Medicaid covers millions of Americans with CF, some states impose limitations, making it hard for a significant number of people to access the services. Besides, Medicaid is characterized by many restrictions. For instance, it is mandatory for the service providers to be contracted with Medicaid before a patient with CF receives health care services. Hence, Medicaid is locking out millions of people with CF, increasing the cost of treating the disease. However, CF Foundations are helping the affected families to deal with the challenges they encounter, especially by providing social support and creating awareness (Homa, Sabadosa, Marrow & Marshall, 2015). Thus, the government should initiate more programs to help families overcome the financial challenges associated with CF. Importantly, the government should collaborate and partners with drug manufacturers to reduce the cost of life-changing CF drugs. Alternatively, the government can subsidize the production of CF drugs. Both state and federal government has a role to play in reducing the prevalence of CF, especially by making its treatment affordable to many Americans. 

In conclusion, Cystic Fibrosis (CF) is one of the health care concerns in the USA, as a significant number of people is suffering from diseases. Any defect in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) results in CF, leading to health problems such as sinus disease, intestinal obstruction, and liver diseases. At the same time, people who are suffering from CF are likely to experience respiratory failure. Medical experts are yet to find the cure for CF, even though treatments methods like the use of antibiotics and oral rehydration. Physical exercise and eating food that is rich in fluoride and zinc also reduces the risk of CF. The disease has a significant social and economic impact. CF does not only lead to poverty, but it can also result in dysfunctional families because its treatment and management require a lot of time and resources. Besides, the cost of treating and managing CF is high, especially at the advanced stage of the diseases. However, the cost can be reduced through the use of telemedicine, which also improves the effectiveness of managing the disease. Many hospitals now rely on telemedicine to manage and treat CF. Exploring health issues like CF helps patients and society at large. 

To reduce the impact of CF, the government should intervene by initiating programs that can help in reducing the cost of treating the diseases. Millions of Americans are not able to afford the treatment cost. Although many middle-income and high-come persons can afford CF treatment and management, a substantial number of low-income Americans struggling to pay for the medication. Thus, to reduce the financial burden of treating and managing CF, Medicaid should be expanded to cover any patient who is suffering from CF in the country. At the same time, the government should collaborate with local communities to implemented CF awareness programs aimed at educating the public. Public awareness is one of the most effective strategies that can be used to prevent the increasing prevalence of CF. Besides, it is through public awareness and education that people would be motivated to go for early diagnosis and treatment of the disease. Apart from using the community to create awareness, both public and private institutions should be supported and empowered to provide CF awareness and training, especially to their workforce of employees. Public awareness can help in preventing genetic diseases like CF significantly. Patients and families need to get both social and economic support to cope with the adverse effects of CF. The treatment and management of CF require a lot of resources, which sometimes may not be available in low-income households. Individuals, organizations, and the government should support families whose members are suffering from CF. Apart from financial support, social and moral support is needed to cope with CF. 

References 

Agrawal, A., Agarwal, A., Mehta, D., Sikachi, R. R., Du, D., & Wang, J. (2017). Nationwide trends of hospitalizations for cystic fibrosis in the United States from 2003 to 2013. Intractable & rare diseases research , 6 (3), 191-198. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5608929/ 

Edmondson, C., & Davies, J. C. (2016). Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications. Therapeutic advances in chronic disease , 7 (3), 170-183. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4907071/ 

Gur, M., Nir, V., Teleshov, A., Bar-Yoseph, R., Manor, E., Diab, G., & Bentur, L. (2017). The use of telehealth (text messaging and video communications) in patients with cystic fibrosis: A pilot study. Journal of telemedicine and telecare , 23 (4), 489-493. Retrieved from https://sci-hub.tw/https://doi.org/10.1177/1357633X16649532 

Hassan, M., Bonafede, M. M., Limone, B. L., Hodgkins, P., & Sawicki, G. S. (2018). The burden of cystic fibrosis in the Medicaid population. ClinicoEconomics and Outcomes Research: CEOR , 10(1) , 423-431. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6065469/ 

Homa, K., Sabadosa, K. A., Marrow, L. C., & Marshall, B. C. (2015). Experience of care from the perspective of individuals with cystic fibrosis and families: results from 70 CF Foundation-accredited programs in the USA. Journal of Cystic Fibrosis , 14 (4), 515-522. Retrieved from https://www.sciencedirect.com/science/article/pii/S1569199314003087 

Rafeeq, M. M., & Murad, H. A. S. (2017). Cystic fibrosis: current therapeutic targets and future approaches. Journal of translational medicine , 15 (1), 84-93. Retrieved from https://translational-medicine.biomedcentral.com/articles/10.1186/s12967-017-1193-9 

Tagliente, I., Solvoll, T., Murgia, F., & Bella, S. (2016). Telemonitoring in cystic fibrosis: a 4-year assessment and simulation for the next 6 years. Interactive journal of medical research , 5 (2). Retrieved from https://www.researchgate.net/publication/301815768_Telemonitoring_in_Cystic_Fibrosis_A_4-year_Assessment_and_Simulation_for_the_Next_6_Years 

Vadagam, P., & Kamal, K. M. (2018). Hospitalization costs of cystic fibrosis in the United States: a retrospective analysis. Hospital Practice , 46 (4), 203-213. Retrieved from https://sci-hub.tw/10.1080/21548331.2018.1505407 

Van Gool, K., Norman, R., Delatycki, M. B., Hall, J., & Massie, J. (2013). Understanding the costs of care for cystic fibrosis: an analysis by age and health state. Value in health , 16 (2), 345-355. Retrieved from https://www.sciencedirect.com/science/article/pii/S1098301512042684 

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StudyBounty. (2023, September 15). Cystic Fibrosis (CF): Causes, Symptoms & Treatment .
https://studybounty.com/cystic-fibrosis-cf-causes-symptoms-and-treatment-essay

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