Case Study on Cystic Fibrosis

Question: Discuss about the Case Study on Cystic Fibrosis. Answer: Cystic Fibrosis is a malady that occurs due to prevalence of genetic disorder. This disorder is widespread in Australia and is effecting a large number of individuals especially children. One in 25 people are carrier of cystic fibrosis and 1 in 2500 children suffers from it. Additionally, 3000 children are currently suffering from this genetic disorder (Douglas et al., 2015). This malady occurs in children when both the parents are carriers of it and usually affects the offspring by altering the autosomal recessive genes. This disease becomes more prevalent due to ineffective knowledge and insufficient access to the remedial treatment. Indigenous people suffer from both the conditions leading to ineffective regulation of required treatment. An analysis on this scenario has been conducted with the case study of Sue who is a 15 years old girl and is suffering from cystic fibrosis since birth (Cutting, 2015). The essay undertakes the implications of cystic fibrosis in Sue who is 15 years old and is from indigenous background. This essay outlines the pathophysiology of cystic fibrosis, its effect on the growth and development of the children in accordance with the behavioural theory of psychology. Furthermore, analysis has been conducted on the need of family-oriented treatment and effect of the hospitalization on Sue. Case Study: This case study is of Sue who is a 15 years old indigenous girl suffering from cystic fibrosis and has been provided with medication of inhalers, pancreatic enzymes and antibiotics and has been informed to undertake physical exercises. Additionally, it has also been analysed that she is smoking for past two years and has six younger brothers and sisters. Indigenous people still do not have optimal awareness level and updated system for the undertaking of effective treatment. Furthermore, she is not undertaking her prescribed medication appropriately. This essay covers each aspect related with the case study of Sue and the implications of cystic fibrosis. Pathophysiology: Cystic Fibrosis is one of the genetic disorders that lead to clotting of the dead tissues that can be fibrous or connective in nature. Clot undertakes the formation of vacant cavity that contains liquid secretion or mucus and is thin walled. Cystic fibrosis in human beings occurs due to the presence of a defective gene that undertakes the collection of thick mucus in different organs of the body but especially occurs in lungs. In addition to this, the mucus clogs the airway passage of the body by blocking the trachea further contributing to the bacterial and viral growth in the target organ. These bacteria and virus attacks the normal functioning of the organ leading to substantial damage to lungs and ultimate respiratory failure. Additionally, cystic fibrosis degrades the digestive system by inhibiting the digestive enzymes to break glucose into glycogen as a process of absorption (Haq et al., 2015). Cystic fibrosis occurs due to mutation of autosomal recessive genes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Furthermore, CFTR protein helps in regulating chloride and thiocyanate ions balance that further cause transportation of epithelial fluid abnormality among the organs of the body. Additionally, CFTR protein also undertakes the formulation of digestive fluids, mucus and sweat which gets prohibited by the cystic fibrosis leading to collection of them and ultimate formation of a cyst (Michl et al., 2016). Growth and Development Theory: Among all the psychological theories of the growth and development, the theory that goes along with the case study of Sue is Behavioural Theory. The concept of this theory has been given and amended by various theorists such as Watson, Skinner, Thorndike and Pavlov. According to this, theory, lifestyle, cognitive skills and interactions with environment affects the physical behaviour in the human beings. A similar condition has been seen in case of Sue who is a 15 year old indigenous girl and is having a two years old case history of smoking. Furthermore, this can be formulated that undertaking of smoking at such tender age can be regulated by copying the other members of the community. Here the other members of the community who smokes are stimuli and the smoking of Sue is a reaction to such stimuli (Crain, 2015). According to Peet and Hartwick (2015), appropriate awareness and targeted knowledge must be instilled among children at a very young age. At tender age, children tend to learn and cope up with the knowledge in a much better way than an elder person. Ideally, instillation of knowledge must start occurring among children from 7 years of age onwards. By this time, the brain is developed enough to understand the maladies and implications related to them. Sue is 15 years old and is a capable enough of understanding cystic fibrosis and its implications but she is incompetent due to the prevailing signs and symptoms of it. Furthermore, under such cases, family and extended family play an essential role of effective decision-making in place of Sue (Peet Hartwick, 2015). Family-Centred Care: Sue requires patient-oriented and family-oriented care for the optimal regulation of treatment. A group of condition-oriented multidisciplinary or multiagency team is required to be formed containing different healthcare professionals such as nurses, pulmonologists, general physician, general practitioners, nutritionist, social worker, psychologist, respiratory or physical therapist, pharmacist, otolaryngologist, gastroenterologist, endocrinologist, counsellors, childcare team and many others. Besides this, family-oriented treatment is also essential as Sue is young and incompetent of undertaking life saving decisions in accordance with her condition. Additionally, it is essential to preserve the ethical and moral beliefs of the family that are build upon the social, cultural, psychosocial, economic and financial experiences (Pietz, 2016). As per the analysis of Malone et al. (2017), the family-oriented intervention model must be implemented by neonatal paediatric nurses formulating effective relations between the them, Sue and family, by analysing the needs and requirement of Sue, analysing the strengths and weakness of the family and patient while undertaking the treatment, respecting the ideologies and dignity of the diversity of the family in terms of social, cultural, spiritual, psychosocial, racial and others wellbeing. Additionally, optimal consent is required to be enhanced in Sue and her family members regarding the effective knowledge on the prevailing condition and associated intervention. Furthermore, as cystic fibrosis is a disease that occurs due to genetic disorder, all six younger brothers and sisters are equally prone of acquiring the disease from Sue or parents (Malone et al., 2017). Family and extended family play an important role in maintaining the mental wellbeing of a patient. Family must provide a patient with optimal somatic and mental support and care in order to prevent the patient from any sort of side effects of such severe malady such as depression, dementia or others. Furthermore, culture influences the decision-making while the regulation of the treatment. The family may prefer treatment of Sue from the healthcare professionals with same indigenous background. In addition to this, cross-culture communication can also prohibit the optimal expression of awareness affecting the decision-making related to intervention (Fidika et al., 2015). Effects of Hospitalization: Association to a malady and hospitalization are two main crises that a child bears and this has drastic effects on the somatic and mental wellbeing of them. This brings severe stress among the patient as well as associated family members. Certain factors such as stress, anxiety, irritation, confusion and others are certain factors that come along with the hospitalization. Furthermore, a child is already suffering from somatic condition and new environment of hospital burdens them with mental sufferings as well. Sue may also suffer from dilemma of being separated from her parents, fear of pharmacological and non-pharmacological interventions, unfamiliarity with the new environment of the hospital, irritation due to weakness, pain, worried due to uncertainty and many others (Hockenberry Wilson, 2014). According to Vitale et al. (2014), the family also suffers from a critical condition experiencing various factors such as depression, disbelief, guilt, anxiety, anger, fear, confusion, frustration and others. This makes the whole situation much complex and confusing for the family members as well. The siblings starts missing their elder sibling, parents separates the unhealthy child from healthy ones in fear of contraction of illness, no awareness enhances the confusion and complications towards remedial interventions, the healthy sibling may start feeling jealous of the unhealthy one due to the amount of care she gets from the family members and other associated people and many others (Vitale et al., 2014). Keeping all these points in mind, it is essential to formulate patient-oriented and family-oriented care to Sue and her family. In addition to this, it is essential to understand the mental pressure Sue and her family is suffering from, and optimal decision-making must be undertaken in accordance with the thought processing of both patient and associated family. The remedial treatment that is a concoction of pharmacological and non-pharmacological interventions must be provided in accordance with the predilection of family members (Mosquera et al., 2014). Conclusion: The indigenous people are most deprived ones in experiencing any sort of facility formulated, implemented and regulated by the legislation of a country. In addition to this, any severe health condition degrades the situation more as they cannot avail effectual remedial treatment as per the requirement by the condition. The legislation and experienced healthcare professionals must undertake optimal steps to ameliorate the gap between the indigenous patients and the optimal safeguard intervention. This report determines the pathophysiology of the critical genetic malady called as cystic fibrosis determining the possible cause of initiation in the body at such tender age among young children. Furthermore, patient and family oriented care must be provided to both patient and associated family in terms of pharmacological and non-pharmacological interventions keeping the behavioural theory in mind. In addition to this, the degrading effects of the hospitalization must be undertaken for con sideration while implementation of remedial treatment to a child. Effective palliative care in accordance with the effectual communication must be undertaken in order to provide comfort to Sue and her family. Furthermore, healthcare professionals must undertake informal dealing with Sue and her family in order to clear the confusion and no awareness in them and further providing relief in accordance with their existing beliefs. References: Crain, W. (2015).Theories of development: Concepts and applications. Psychology Press. Cutting, G. R. (2015). Cystic fibrosis genetics: from molecular understanding to clinical application.Nature Reviews Genetics,16(1), 45-56. Douglas, T., Jordan, B., Priddis, L., Anderson, V., Sheehan, J., Kane, R. T., ... Shields, L. (2015). 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