Bruce with Cystic Fibrosis - Case Study - Nursing Assignment Help

Assignment Task

Case Study Analysis Example
Introduction
This essay will be analysing the case of a 13-year-old boy named Bruce with cystic fibrosis (CF), a life-limiting genetic condition that affects the mucus production in the body, largely affecting the lungs and the gastrointestinal system (Fraser et al., 2017). Bruce lives with his family in Merredin, Western Australia (WA), regularly having tune ups at Perth Children’s Hospital (PCH). He has a history of procedural anxiety and pancreatic insufficiency from his CF, and is scheduled for a tune-up and one week stay in hospital for exacerbation of his CF and an insertion of a new Peripherally Inserted Central Catheter (PICC). Despite his supportive parents, Bruce appears withdrawn and reluctant to be in hospital, uninterested in eating or involving himself in his care, however after 24hrs of antibiotics he states he has stated his cough is improving. With this information this essay will discuss the pathophysiology, management and health promotion for Bruce and his family.

Pathophysiology
Cystic fibrosis is caused by a mutation in the cystic fibrosis transmembrane regulator (CFTR) protein gene, and is an autosomal recessive disorder. The CFTR protein ordinarily functions as a channel across epithelial cell membranes, and controls the chloride ion transportation and therefore the movement of water in and out of the cell, affecting the flow of mucus in the body (Fraser et al., 2017). Normally, the CFTR gene interacts with inflammasome, transports anions, and regulates the epithelial sodium channel (Elborn, 2016). In a person with cystic fibrosis, there is hyper-inflammation of proteasome, reduced or absent anion transport, and hyper-reabsorption of sodium, and impaired innate immunity, leading to mucous anomalies (Elborn, 2016). This results in mucus retention and chronic infections, and because of this, morbidity and mortality are often caused by bronchiectasis, progressive respiratory impairment, and small airway obstructions. Issues with the CFTR gene can affect malabsorption in the pancreas (Elborn, 2016). In the case study it states that Bruce has a history of pancreatic insufficiency as a complication of his CF, due to the thick mucus in the pancreas blocking pancreatic enzymes from entering the small intestine, so the digestive tract begins to pass partly undigested food, affecting nutrition status (Cystic Fibrosis Foundation, n.d.).

Nursing Assessment and Management
To prioritise and manage Bruce’s care, the nursing staff first need to assess Bruce using a variety of tools and assessments to develop a nursing plan. One of these assessments is the Children’s Early Warning Tool [CEWT], a tool that assesses for clinical deterioration in patients (Cassidy et al., 2019). Bruce’s observations are as follows: Temperature 38.7, Heart rate [HR] 120 beats per minute (bpm), Oxygen saturation 95% at room air, blood pressure [BP] 100/70mmHg and respiratory rate [RR] 26 breaths per minute with mild distress. According to the CEWT for children 12 years and above, Bruce scores a 4 on the chart which indicates he needs increased frequency of observations, review of oxygen requirements, treatment to clear the mucus in his airways, and a review from the ward doctor.
To treat exacerbations of CF, IV antibiotics is commonly prescribed, often through a PICC line (May et al., 2018). Bruce has a blocked PICC so requires an insertion of a new PICC. His procedural anxiety means sedation or general anaesthesia should be considered for the insertion of his PICC line. Antibiotics are a constantly used therapy for CF patients, and after 24 hours of antibiotics Bruce has already noted an improvement, so ensuring IV access is maintained is important to fight off recurring infections.

Bruce’s increased HR and RR indicate ineffective airway clearance, and a respiratory assessment should be done to assess for adventitious or decreased breath sounds to confirm this (Swearingen, 2015). To improve airway clearance, the use of bronchodilators and chest physiotherapy is a daily regimen aiding patients to mobilise and clear the thick secretions in the airways (Kamat, 2017). Medications via a nebuliser is another treatment option to clear secretions, and it is important to sit Bruce up to maximise the inhalation of the medication and improve effectiveness of his cough to clear airways (Kamat, 2017).
Nutrition is a major aspect for cystic fibrosis management, especially in patients with pancreatic insufficiency such as Bruce. A diet high in calories, proteins, salts, and medium to high in fats is necessary (Swearingen, 2015). Patients with CF need at least 120-130% of the recommended daily allowance of caloric intake, and the fat intake should make up 35-40% of this, compared to 30% fat intake for those without CF (Kamat, 2017). Pancreatic enzymes should be administered with meals and snacks as prescribed, as replacement of enzymes is necessary for proper digestion and absorption of nutrients, and is important for growth and in fighting infection (Standen, 2019). As well as the enzymes, non-fat soluble multivitamins are an important part of managing Bruce’s care. Considering a dietician referral for Bruce and his family regarding Bruce’s requirements during his exacerbation of CF should also be considered (Kamat, 2017).
Bruce will be spending his life treating his illness at home and at hospital, so it is important that we address his procedural anxiety and reluctance towards hospitalisation for future treatments. Providing psychosocial support to Bruce and his family is important so that we can intervene before Bruce’s anxiety worsens and his family become reluctant to receive care. Introducing our roles and communicating with empathy and active listening techniques will establish a safe atmosphere for Bruce and his family, relieving some anxiety (Swearingen, 2015). CF social workers can help families deal with not only psychological aspects of managing Bruce’s care but financial ones as well, especially considering they live regional and need to travel for Bruce’s tune-ups.

Health Promotion
In a few years’ time Bruce will be transitioning to adult health care, and Bruce and his family need preparation for his change. Transitioning from paediatric care moves adolescents with chronic conditions to adult health care in a manner that maximises quality care in an age-appropriate service, occurring over time involving the paediatric and adult health care agencies (Singh et al., 2020). By the end of this process, Bruce should be able to actively participate in self-managing his CF and making decisions in his care.
Perth Children’s Hospital has three stages to transition, the first stage being the introductory/planning stage that begins approximately around 12-14 years old, however this stage can vary (Perth Children’s Hospital, n.d.). This stage introduces Bruce and his family to the transition process, working out what Bruce needs to know about himself and his medical condition. The next stage, the preparation stage, is usually during the ages of 14-16 years, and develops a transition plan in partnership with Bruce and his parents. Bruce will also begin to learn the skills needed for caring for himself at this stage. The final stage is the transfer stage, usually between 16-18 years of age, and begins when Bruce is looking after himself with confidence and is ready to transfer, and information is exchanged between the health care team at PCH and adult health services (Perth Children’s Hospital, n.d.).
For the initial stage of transition, educating Bruce on the care being provided and why, and encouraging his parents to talk through the care they give him at home is important to provide Bruce with information about himself that he will need for adult care and to manage his own care when he’s older and ready (Coyne et al., 2018). Referrals to CF social work will also help provide Bruce and his parents support on how to prepare for a higher level of independence in Bruce’s care in a smoother transition (Kamat, 2017).

Conclusion
To conclude, cystic fibrosis is a chronic, life-limiting genetic condition that requires daily care and a lot of contact with health services throughout someone’s life. For this hospital presentation and tune-up, Bruce needs to have a clear airway maintained and treatment provided for the increased mucus in his lungs, and he needs to be considered for sedation or general aneasthesia while his PICC line is inserted to try and lessen the stress from his procedural anxiety. He needs nutritional supplements and assessments on his nutritional status, and psychosocial support given to him and his family for both Bruce’s withdrawn presentation and in preparing him and his parents for the transition to adult health care, a process that needs to be introduced early so that planning can begin and nobody feels unprepared when the time for transition does come.

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