Introduction
Cystic fibrosis is an autosomal recessive condition that is caused by the mutation of the cystic fibrosis trans-membrane conductance regulator. The mutation affects the absorption of chloride ions which impairs the movement of sodium and water at the cellular level. This results in production of abnormally thick secretions that cause inflammation and damage to the cell. This affects the functioning of the organs. It is one of the most common genetic disorders that affects Europeans. Statistics indicating that one out of 2381 people in the United Kingdom suffer from the condition.
The complexity of the disease calls for a holistic approach in the treatment and the management of the condition. A multidisciplinary team is often needed to treat and manage cystic fibrosis. Frequent evaluations for complications arising from the condition need to be carried out in addition to monitoring the progression of the disease. The multidisciplinary team should be comprised of a specialist in respiratory conditions, a physiotherapist, a psychologist, a pharmacist, a social worker and a microbiologist. Care of patients with cystic fibrosis calls for co-ordination among the members of the multidisciplinary team in order to continually provide high quality care that will result in better outcomes among the patients suffering from cystic fibrosis.
The treatment and management of cystic fibrosis is lifelong process. It involves chest physiotherapy two times a day in order to prevent the accumulation of thick secretions in the airway. Patients have to take prophylactic antibiotics to fight bacterial infections that can have devastating effects on the outcome. Pancreatic enzymes and fat soluble vitamins have to be administered orally since cystic fibrosis patients have digestive complications. Their digestive system cannot digest fats. Some patients also need steroids and bronchodilators. The treatment of cystic fibrosis is an involving endeavor that is expensive. In spite of the increase of the life expectancy of cystic fibrosis patients to 27 years, poor outcomes are still associated with the condition in Ireland where prevalence of cystic fibrosis is the highest in the world.
It is estimated that 3 out of 10,000 people in Ireland are suffering from cystic fibrosis. The country also has the highest proportion of families in which more than one child is suffering from the condition.The strain of cystic fibrosis in Ireland is the deadliest form predisposing the individuals suffering from the condition to a difficult life that may result in an early death. Social and cultural aspects are believed to be some of the major factors that contribute to the poor outcomes associated with the disease. The exact extent of their influence remains debatable but the connection between poor outcomes and these aspects shall be discussed in this essay.
Discussion
Socio economic status is construct comprising of several dimensions that is often used as an indicator of the effect of the environment and culture on an individuals’ health. In a study that defined the socio economic status of the participants in terms of race, income, level of education and ethnicity, it was found that a low socio economic status was likely to be associated with a reduction in pulmonary function as well as faltering during growth among patients suffering from cystic fibrosis. This study was carried out in Ireland. It emphasizes the findings of another study which showed that 20% of patients were likely to fail to return to their baseline condition following an exacerbation in spite having access to treatment.
Investigations into the factors that lead to this showed that this was as a result of the following factors: a lack of medical insurance and poor adherence to the treatment regimen which are both indicators of low socioeconomic status.
In another study carried out by researchers from the University of Nottingham, it was found that death as a result of cystic fibrosis was more prevalent among females and socially disadvantaged just as much as it was 50 years ago. This is in spite the fact that medical advances that have been made in the recent past have resulted in the average age at which individuals suffering from cystic fibrosis to 27 years, up from 6 months in 1959. The researchers made their conclusions following an analysis of the records of cystic fibrosis deaths from 1959 to 2008. The records showed that the death in age groups of between 0-4 years and 25-29 years was higher among females as compared to males. The team concluded that this difference could be attributed to environmental factors or varying access to health care services that are vital for the management of symptoms.
The findings of this study correspond with findings of studies that were carried in the United States where the prevalence rate of cystic fibrosis is three times lower than in Ireland. While the cystic fibrosis strain that is found in USA is different from the one that is found in Ireland, it has been found that there is a correlation between poor lung function and low socio economic status. During the study, the researcher split the participants into three groups: socially advantaged, socially disadvantaged and not socially disadvantaged. At the end of the study, it was found that children suffering from cystic fibrosis who were drawn from the socially advantaged households had a better FEV1, BMI and weight as compared to the patients who were drawn from the other two groups.
While the findings may be from an American study, the aspects raised in the study could apply to Ireland. It is possible that socially disadvantaged families live in poorly heated and ventilated houses which further exaggerates the severity of symptoms of cystic fibrosis. It is also quite common to find that socially disadvantaged parents have a lower level of education which limits their understanding of the management of the condition. Such parents work for long hours hence have little time to ensure that the members of their families who are suffering from cystic fibrosis adhere to their treatment regimen. This results in poor nutrition hence the loss of weight and low BMI.
Cystic fibrosis is mostly treated and managed by a lifelong dependence on oral, nebulized antibiotics. Research indicates that patients with chronic Pseudominas aeruginosa infections have a worse outcome than the patients who are not infected. First time acquisition of Pseudominas aeruginosa is often aggressively treated using antibiotics. In some cystic fibrosis centers, intravenous antibiotic treatment lasting for 3-4 months per year can be prescribed to treat Pseudominas aeruginosa infections. As the disease progresses, so does the intensity of the treatment. Patients awaiting organ transplants require noninvasive ventilator support, feeding via tubes and oxygen support. This makes the cost of treating cystic fibrosis quite high. In Ireland, some of the families have up to two children with cystic fibrosis which puts a tremendous strain on financial resources. In some cases, the financial status of the family prevents them from getting medical cover. They have to rely on free treatment services that can be accessed from non-governmental organizations. Lack of funds is associated with a worse outcome in patients suffering from cystic fibrosis.
In another study, the researchers found that cystic fibrosis patients who contract Burkholderia cepacia and Pseudominas aeruginosa have worse outcomes than those who do not contract either of these bacterial infections. The participants in the study were divided into three groups: those who had Burkholderia cepacia and Pseudominas aeruginosa infection, those who only had Pseudominas aeruginosa and those who had neither of these infections. It was found that those who had both the infections had a 5.4% decrease in lung function as compared to 1.6% decrease in lung function in patients with none of these infections. Burkholderia cepacia is difficult to treat because it is resistant to most antibiotics. This can particularly be devastating to patients who have limited family resources since it significantly increase the cost of their treatment. It has been shown that limited funding limits access to vital antibiotics in government run facilities in Ireland. This further exaggerates the outcomes that are associated with cystic fibrosis among patients whose socioeconomic status is low.
Currently, there are very few government run facilities that have isolation units that entirely dedicated to the isolation of cystic fibrosis patients. This can be harmful to cystic fibrosis patients since infections such as Burkholderia cepacia are highly contagious and the lack of isolation only compounds the problem. A report on the standard of care for cystic fibrosis patients indicates that in patient care of cystic fibrosis patients calls for availability of sufficient beds at the hospital at all times. The beds for the patients must be in separate rooms with a toilet and bathroom en suite. Patients with Burkholderia cepacia should not be allowed to interact. Currently, there is a limited number of centers in Ireland that can accord this level of care. Most patients opt to manage the condition from their homes due to the fear of poor management in hospitals. Even with the best efforts, this limits the availability of monitoring services that are available to the patient. Delayed admission times at non-governmental institutions only puts the patients at a greater risk.
In Ireland, most of the cystic fibrosis centers rely on donations from the public and organizations. Government funding is limited. While the efforts of these organizations have significantly improved the outcomes associated with the disease over the last 50 years, the limited funding obtained from the government affects the care that they can provide. Some of the drugs that are useful in the management of cystic fibrosis are quite expensive. The organizations cannot access these drugs on behalf of the individuals who need them as a result of limited funding.
The Cystic Fibrosis Registry of Ireland is one of the organization that has over the years collected and analyzed data on cystic fibrosis in order to improve the quality of care in the country. The data collected by the organization relates to incidence, prevalence and treatment of the condition. The organization was founded in 2001. It is mostly funded by HSE but the amount of funding the organization receives has remained static over the years. The gathering of health intelligence which significantly informs the care of patients suffering from cystic fibrosis is crippled by this. An increase in the funding given to the registry would help in the realignment of the data obtained so that is up to par with the data that is collected by similar organizations in the United States of America and other parts of North America.
It is only as recently as last year that the government came up with a proposal that will help in faster diagnosis as well as improved access to care in the country. According to the plan, the government will establish a centre for rare diseases such as cystic fibrosis which will serve as a platform for the co-ordination of care and research about cystic fibrosis. The plan was launched against the backdrop of an admission that there is a lack of proper knowledge and research in Ireland that would inform management and care for the patients that are affected by the condition. This admission particularly holds true among low income citizens who have limited knowledge about the condition.
The effect of this is particularly pronounced among children whose diagnosis could be delayed since the parents lacked vital information about the signs that are associated with the disease. This affects the onset of the process of seeking intervention which ultimately determines the outcome of the intervention. As such, cases that are diagnosed later in the life of the child become more expensive and difficult to manage since by the time they are diagnosed, the disease has progressed.
In one study, it was found that gender does influence the prognosis of cystic fibrosis. However, females have a poorer quality of life as compared to males. Their chest symptoms and emotional functions appear to be more severely affected by the disease as compared to males. Males were found to have a poor quality of life with regard to their body image. These findings corroborate what Nottingham researchers found out in a study carried out in the UK. They found that being a female and having a lower socioeconomic status was associated with poorer outcomes among cystic fibrosis patients.
Another study showed that outcomes among Asians in the United Kingdom were worse than outcomes that are associated with other racial groups. Asian females were found to have worse FEV 1 as compared to their male counterparts within the same age group. The researcher attributed to a number of factors which include but are not limited to socioeconomic status, inadequacy of healthcare services among Asian, language barriers and genetic factors. There are no such studies that have been carried out to this effect in Ireland but given that the factors at play remain constant regardless of the geographic location, it is possible that same factors would emerge in cystic fibrosis patients from other races in Ireland. The exact link between gender and outcomes in cystic fibrosis is not entirely understood but it is speculated that an interplay of environmental, cultural and genetic factors is responsible for the poor outcomes among females.
It is important to address the disparities brought about by social and cultural aspects in the outcomes among patients suffering from cystic fibrosis are to be improved. There is a need to improve access to education and health care services among cystic fibrosis patients regardless of their social and cultural status. There is also a need to broaden research efforts in Ireland in order to understand the extent of the effect of social and cultural aspects on outcomes hence tailor appropriate responses.
In conclusion, socio and cultural aspects interact with other factors in affecting outcomes in cystic fibrosis patients. The role of socioeconomic status with regard to income, education, access to health care and information and availability of government funding cannot be ignored.
References
Balmer, D. F., Schall, J., & Stallings, V. (2008 ). Social disadvantage predicts growth outcomes in preadolescent children with cystic fibrosis. Journal of cystic fibrosis , np.
Barr, H. L., Britton, J., Smyth, & Fogarty. ( 2011). Association between socioeconomic status, sex, and age at death from cystic fibrosis in England and Wales (1959 to 2008): cross sectional study. BMJ, 343 .
Cuthbert, A. (2011). New horizons in the treatment of cystic fibrosis. British Journal of Pharmacology, 173-183.
Doring, G., & Hoiby, N. (2008). Early intervention and prevention of lung disease in cystic fibrosis: A European consensus. Journal of cystic fibrosis, 67-91.
Kerem, E., Conway, S., Elborn, S., & Heijerman, H. (2005). Standards of care for patients with cystic fibrosis: a European consensus. Journal of cystic fibrosis, 7-26.
McCormick, J., Mehta, G., Olesen, H., Viviani, L., Macek, J., & Mehta, A. (2010). Comparative demographics of the European cystic fibrosis population: a cross‐sectional database analysis. Lancet, np.
Murray, K. (2012). An analysis of factors contributing to poor outcomes in Cystic fibrosis. Scottish Medical Journal, 23-30.
Verma, N., Bush, A., & Buchdahl, A. (2005). Is there still a gender gap in cystic fibrosis? . Chest, np.
