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Pathophysiology Of Cystic Fibrosis

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Discuss about the Pathophysiology of Cystic Fibrosis.


The present case study analysis is on a patient named Sue who is a 15-year-old indigenous girl living in a remote town in Australia. The patient had been diagnosed with cystic fibrosis in her infancy and had been prescribed a regimen of exercises, antibiotics, inhalers and pancreatic enzymes. She had presented to the local hospital due to complaints of shortness of breath and informed the triage nurse that she has cystic fibrosis. A healthy history assessment undertaken by the nurse revealed that the patient had a two-year history of smoking. In addition, the patient did not adhere to medication regimen for cystic fibrosis and other management strategies outlined. The patient had been living with her mother and six younger brothers and sisters. The present essay would describe the pathophysiology of the presenting complaint. The next section would be an evaluation of the nurse’s role in delivering developmentally suitable nursing care. Growth and developmental theories, family centred care and impact of hospitalisation of the patient on the family as well as the child would be discussed in here. The essay would end with a logical conclusion to the whole discussion.

Cystic fibrosis (CF) is a genetic disease causing sticky, thick mucus for building up in the digestive tract, lungs and other parts of the body, as a result of which there is serious damage to the digestive and respiratory systems. The disease is a condition of the exocrine gland function involving multiple organs. It is a common form of chronic lung disease in young adults and children whereby the patients have a threat to their lives. Cystic fibrosis is a result of a defect formed in the cystic fibrosis transmembrane conductance regulator” gene or CFTR gene. The gene is responsible for controlling the movement of salt and water in and out of the cells of the body. Any mutation or any form of change occurring in the CFTR gene leads the mucus to become stickier and thicker, causing complications to the body. The mucus gets build up in the pancreas, liver, intestines and lungs. Different forms of defects might take place in the CFTR gene, and the damaged gene is inherited in a newborn from the parents (Haack et al., 2013). Normally, patients with cystic fibrosis have acute respiratory complications. As the mucus gets thicker, it obstructs the airways in an undesirable manner, and it becomes difficult for the air to adequately pass at the time of exhalation. This leads to shortness of breath in the patient (Grossman & Grossman, 2005). According to Cantin et al., (2015) breathing discomfort is an integral element of cystic fibrosis. For a patient suffering from cystic fibrosis the disease is related to increased morbidity and poor prognosis, breathing problems are often a result of poor quality of life in patients with cystic fibrosis. In the present case, the patient has been found to be a regular smoker. In addition, she does not adhere to the medication regimen prescribed to her on a regular basis.


Nursing care for patients with cystic fibrosis is an integral element for the person-centred care of patients with the disease. Improved survival for the disease is achieved for the patients if the nurse acknowledges their role in the care delivery process. It is important for a nurse to adhere to the guidelines set for cystic fibrosis management and to ensure that all spheres of care are given adequate attention in due course. It is the role of the nurse to educate the patient about the importance of the care plan and management regimen laid out and coordinate all the provisions of care services in support of the patient and the family members (Tointon & Hunt, 2016).

Application of growth and developmental theories in nursing have been long considered for practice against the context of rising demand for adequate care plan for achieving better outcomes. One of the key theories of human development that can be suitably applied for nursing care of patients is the theory of human development proposed by Jean Piaget (Hockenberry & Wilson, 2014). Application of this theory for the present case scenario would be appreciable. As per Piaget, the interaction of the patient with surrounding environment plays a key role in the development. In the context of healthcare, children who are critically ill are to be given care by the nurses to the degree that facilitates and promotes better patient outcomes. The nurse must have a knowledge of the psychology of Sue and must develop an adequate understanding of the development phase that the child is in for delivering the required care services. This would be beneficial for the nurse to assess the stage of development in which the patient is in for planning the correct level of stimulation, that would at the end facilitate progress to a large extent instead of the deterioration in fulfilment of developmental tasks (Hockenberry et al., 2016).


According to developmental theories of Jean Piaget and Erik Erikson, in the case of children suffering from chronic diseases, the impact of the social, emotional and cognitive development occurs in a certain way on the prognosis of the disease. Future developments in the nursing care process are guided by the way the developmental stage of the patient is addressed. The developmental of a child can be divided into different stages, namely, infant, toddler, preschooler, school-age and adolescent, based on the theories put forward by these scientists. The adolescent phase encompasses of age 12 to 18 years. In the present context, Sue is an adolescent girl, 15 years of age, and her care process given by the nurse must be provided in a specific manner considering her age and developmental stage. The nurse must ensure that the environment in which she is admitted to the healthcare setting for breathing troubles is safe and secured for her. Privacy is to be maintained at the time of nursing care. A separate room might be considered for her during her stay at the hospital. The patient needs to be given adequate information about her presenting conditions and must be educated about the treatment regime laid out. Both verbal, as well as written instructions, are to be provided are to be provided to the patient regarding her care plan. Continuation of education regarding her symptoms and the risk factors is pivotal since at the adolescent stage one usually is able to understand the underlying principles of care (Butcher et al., 2013).


In the adolescent stage of development, one develops the new sense of self or identity. It is a common tendency for an adolescent patient to try out different new strategies for maintaining a good health condition. It is a quest to understand what fits best for the patient in the context of family expectations. The family context for the patient is to be clearly identified if adequate care is to be provided. In the present case, the influence of her family on Sue might be deep impacting. The relationship that Sue shares with her family is to be considered in order to achieve better patient outcomes. The challenge of being admitted to a health care setting adds to the crisis to the developmental crisis of a patient in the adolescent stage. The nurse is to aid in resolving this crisis and making the patient feel secured and safe in the new environment (Saewyc, 2000).

As opined by Potter et al., (2016) nursing care must meet the needs of the patient and provide opportunities to involve the patient as well as the family members in the process of care planning. A family centred care approach is pivotal for enabling the patient to adhere to the medication regimen and other management strategies. The nurse is able to meet most of the needs of a patient; however, it is the continual involvement of the parents and family members that enable a patient to achieve best outcomes. Parental involvement is crucial at the time of hospitalisation as well as after discharge. In the present context, the nurse must educate Sue’s mother about her involvement in the care process in order to reduce the risks of further health complications pertaining to cystic fibrosis. She must be made aware of the drastic health impacts of not adhering to the medication regimen as Sue is found not to adhere to her care regimen. Further, her mother must be educated on the harmful impact of smoking on cystic fibrosis and must be supported to help Sue quit smoking. It is seen that Sue has six siblings and therefore it is common for her mother to give less attention to Sue’s management plan for cystic fibrosis. Her mother must therefore be educated on social management practices so that Sue gets adequate care and support (Mattila et al., 2014).


Impact of hospitaliation on patients as well as family members is a singifican area of nursing care. It has been indicated through research the perceptions of patients regarding admission to health care settings as well as knowledge and attitude of the patient and family members regarding the care setting plays a role in the way patient goals are achieved and the recovery process of the care. It is the responsibility of the nurse to provide emotional support to the patient for reducing any chances of trauma and anxiety. A rich pool of nursing literature has thrown light upon the fact that care process is facilitated if the patient facing trauma, fear and anxiety is reduced to a considerable extent. The nurse must, therefore, consider effective strategies for reducing any form of anxiety or fear that Sue has been facing after being admitted to the setting. This process must be culturally sensitive and ethnically safe (Barling et al., 2014). The patient in the present case is an indigenous patient staying at a remote location in Australia. The nurse must, therefore, be aware of the cultural practices of the indigenous population that are considered for providing emotional support and care. The traditional ways of providing comfort and affection that are usually considered by indigenous population are to be considered in the present context so that the patient can receive ethnically suitable care approach (Giger, 2016).

From the above case study analysis, it can be concluded that nursing practice for an adolescent patient needs to encompass the integral care components that are safe and sound on a person-centred basis. Cystic fibrosis is a severe disease that requires regular monitoring and adherence to management regimen. For the present case, Sue is suffering from health complications arising due to mismanagement of her care plan that aggravates the underlying pathophysiological conditions of cystic fibrosis. It is the duty of the nurse to provide an integrated and comprehensive care to the patient that involves the patient as well as her family in the major decisions of care delivery.



Barling, J. A., Stevens, J. A., & Davies, K. M. (2014). The reality of hospitalisation: stories from family members of their hospital experience for adolescents and young adults living with and dying from cancer. Contemporary nurse, 46(2), 150-160.

Butcher, H. K., Bulechek, G. M., Dochterman, J. M. M., & Wagner, C. (2013). Nursing interventions classification (NIC). Elsevier Health Sciences.

Cantin, A. M., Hartl, D., Konstan, M. W., & Chmiel, J. F. (2015). Inflammation in cystic fibrosis lung disease: pathogenesis and therapy. Journal of Cystic Fibrosis, 14(4), 419-430.

Giger, J. N. (2016). Transcultural nursing: Assessment and intervention. Elsevier Health Sciences.

Grossman, S., & Grossman, L. C. (2005). Pathophysiology of cystic fibrosis implications for critical care nurses. Critical care nurse, 25(4), 46-51.

Haack, A., Aragão, G. G., & Novaes, M. R. C. G. (2013). Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases. World Journal of Gastroenterology: WJG, 19(46), 8552.

Hockenberry, M. J., & Wilson, D. (2014). Wong's nursing care of infants and children. Elsevier Health Sciences.

Hockenberry, M. J., Wilson, D., & Rodgers, C. C. (2016). Wong's essentials of pediatric nursing. Elsevier Health Sciences.

Mattila, E., Kaunonen, M., Aalto, P., & Åstedt?Kurki, P. (2014). The method of nursing support in hospital and patients' and family members' experiences of the effectiveness of the support. Scandinavian journal of caring sciences, 28(2), 305-314.

Potter, P. A., Perry, A. G., Stockert, P., & Hall, A. (2016). Fundamentals of nursing. Elsevier Health Sciences.

Saewyc, E. M. (2000). Nursing theories of caring: A paradigm for adolescent nursing practice. Journal of Holistic Nursing, 18(2), 114-128.

Tointon, K., & Hunt, J. (2016). How holistic nursing can enhance the quality of life of children with cystic fibrosis. Nursing Children and Young People, 28(8), 22-25

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