Question:

Take a look at the case study and answer all the questions.

Explain the pathophysiology, clinical manifestations, and causes of cysticfibrosis.

Discuss the comorbidities associated with diabetes and malnourishment as well the consequences for the health of the patient and his/her family.

Analyze the relevant literature and discuss any functional impairments in this case study.

Think about the challenges of caring and the proactive care needed to keep this client healthy.

You can use evidence-based practices to recommend resources/services needed for client care. This includes pharmacological management.Case Study

Stephanie Mills, 15, was born with cystic fibrosis.

Stephanie lives with her family, including her parents and Emily and Tim 19.

She attends regular meetings with her cystic fibrosis team at least once every three month and must have blood tests performed and throat swabs taken at each visit.

Stephanie also has diabetes. She must check her blood sugar levels before each meal.

In order to keep her blood sugar levels in check, Stephanie will need to be insulin-treated if she has high blood sugar.

She is also undernourished and weighs in at 50kgs.

Stephanie is currently taking multiple vitamins and a high-energy, low-protein diet.

She receives approximately 30 minutes of physiotherapy each day and takes inhalers.

Beginning with breakfast, she must take a few “Creons” mixed with her food. This helps her digest her food.

Stephanie spends 40 minutes inhaling, physiotherapy, or nebulisers at night.

Stephanie has to practice her breathing exercises and get her peak flow test readings.

She has to be on a range of antibiotics and they change from time to time.

Although encouraged to be active, she has trouble finding the time or energy.

When the family goes on holiday, all of Stephanie’s medication and equipment should be with her.

Stephanie’s routine of physio each morning, and evening must be followed whether they are out on vacation or going out.

This can make it very difficult for everyone and can lead to frustration.

Because of the challenges involved, Stephanie is often cared for by the entire family while her siblings go on holiday.

Stephanie’s dad is a full-time retailer manager. Stephanie often does not get home until well into the evenings because he works long hours.

Alice, Stephanie’s mom, works part-time as a chef to help with Stephanie’s care.

Tim goes to University during the school day and works at the local dairy bar in the night.

Emily, a local high school student, is currently in year 11 and enjoys playing netball.

With the many admissions and the inability of mom to work, the family has been having financial problems paying for Tim’s tuition.

Answer to Question: CNA116 Introduction To Nursing

Introduction

The case study is that of Stephanie Mills (age 15), who was diagnosed with cystic Fibrosis at birth.

The first part of the analysis discusses the pathophysiology, clinical manifestations, and other aspects of cysticfibrosis.

Next, the paper examines the impact of diabetes and malnutrition on patient health. Finally, the paper reviews relevant literature to discuss functional impairment.

After that, it discusses the difficulties of caring and proactive care required for the patient.

The last recommendation is to identify the services and resources that are necessary for the care of the patient, including pharmacological administration.

Pathophysiology And Clinical Manifestations of Cystic Fever

Cystic fibrosis can be described as a genetic disorder affecting the exocrine system. This condition affects mainly the lungs, liver, intestine and kidneys.

Cystic fibrosis (CFTR) is an inherited autosomal-recessive disorder. It results from mutations in the cystic fibrosis transmembrane conducting regulator (CFTR). CFTR normally functions as a channel for chloride, and is controlled by cyclic Monophosphate (cAMP).

Many mutations can occur within the CFTR genome, which can cause mild to severe problems in the protein produced by the gene.

Common defects include faulty folding and short protein lengths, as well as proteins that are not able to use ATP.

This mutation affects the ability of thiocyanate, chloride, and iodine to move across the cell membrane.

This disease is distinguished by the abnormalities in cAMP regulated chloride transport across epithelial and mucosal cells.

The defect results in reduced chloride secretion, and also increased sodium and water reabsorption across epithelial tissues.

Increased mucus production and inflammation is caused by decreased mucus hydration, along with an increase in lining fluid.

The mucus secretions from the pancreases of sweat glands and respiratory tract, as well as other exocrine tissue, are viscose. (Martin et.al., 2017).

Stoltz et. al. (2015) state that the majority of cystic fibrosis patients suffer from clinical manifestations associated with lung disease and exocrine-pancreatic insufficiency.

Additionally, there are many other clinical manifestations of cystic fibrosis that are serious.

There are many signs that can occur in the lower respiratory tract, including chronic infections with organisms such staphylococcusaureus, Haemophilus Influenzae, and Pseudomonas.

Other signs and symptoms include bronchiectasis (acute exacerbations), allergic bronchopulmonary Aspergilllosis (allergy bronchopulmonary aspergilllosis) and atypical Mycobacterial infection. Complications of the upper airway tract include pansinusitis and nasal polyposis.manifestations of the liver include gall stones, portal hypertension and bile duct stricture.

Coeliac disease is a condition that can cause gastrointestinal complications. These comorbidities may include severe constipation (distal intestinal obstruction syndrome), Crohn’s, Crohn’s, coeliac disease, severe diarrhea, Crohn’s disease, and Giardiasis.

Problems with the sweat gland include electrolyte deficiencies in sweat.

Patients may also experience genitourinary complications, bone disease, such as immobility, a delay in puberty, or malabsorption and/or endocrine failure, such as an insulin deficiency.

It is important to note that CF is a leading cause of death.

Cystic Fibrosis can be characterized by poor growth and salty-tasting hair.

The Comorbidities of Diabetes And Malnourishment: The Impact On The Health Of The Person and Her Family

Two of the most serious health conditions are malnutrition, and diabetes.

Hall (2015) identified the comorbidities and their effects on health.

A majority of diabetes patients suffer from uncontrolled hypertension, high blood pressure, and other conditions.

Hypertension and diabetes can increase the risk for complications.

Atherosclerosis is an increase in the risk of complications such as high blood pressure and uncontrolled blood glucose levels. Clogging causes blood vessels to narrowed.

Poor blood supply can lead to angina.

Patients are also more likely to suffer a stroke or heart attack.

Dyslipidemia describes a condition that has abnormally high blood lipid levels.

Such a condition significantly increases the likelihood of developing cardiovascular disease.

Non-alcoholicfatty liver diseases is when patients have high liver enzymes and a larger liver.

A further 40% of diabetics suffer from chronic renal disease. This is where damage occurs to the nerves of the body, blood vessels and kidneys.

Malnutrition increases the likelihood of complications, such as weight loss, development delay, and suppressed immuno system.

Although developmental delay and impaired immune system function are temporary complications, growth delays have long-term implications (Ibrahim and co-authors, 2017).

Malnutrition can cause a weak immune system that makes the patient susceptible to contracting infectious diseases.

Infections such as gastrointestinal problems can cause nutrient deficiencies. Growth retardation can also be caused by nutrient shortages.

Malnutrition reduces total bone growth.

One nutrient shortage could lead to another.

This can lead to long-term complications, such as protein deficiencies.

Diabetes has a major impact on the life of both the patient as well as the family. The emotional and physical effects are more severe.

Anxiety is a result of the diabetes complications.

Both the patient as well as the family members can experience anxiety and stress.

Stephanie may experience stages of grief. Therefore, it is natural that she shares her concerns with family members who might find it difficult or impossible to understand the situation.

Due to her professional and personal obligations, Stephanie might feel stressed out and depressed.

The depression that a patient is suffering can cause stress in the family members. Family members should also take time to get to know the patient’s situation (James et. al., 2014.).

Stephanie is suffering from malnutrition. It is the responsibility to ensure proper nutrition.

Stephanie’s family was involved in the management and self-management diabetes.

Study of the Associated Functional Impairment

Stephanie was diagnosed with malnutrition which causes cognitive and functional impairments.

Malnutrition is a problem that can affect brain development and delay motor and cognitive development.

These impairments include learning disabilities, learning disabilities, attention deficit disorder, impaired school performance, and decreased problem solving ability (Prado & Dewey (2014)).

Stephanie is 15 years of age, so her academic performance may be affected by her health condition.

Huysentruyt (et al.) (2017) claim that cysticfibrosis may be closely linked with functional impairments.

Cystic fibrosis symptoms may vary between patients. These include symptoms such as constipation, stomach ache, bloating, and constipation. Also, respiratory symptoms such a shortness and fever can occur.

Cystic fibrosis can cause a patient to have difficulty living a normal lifestyle.

Stephanie was taking Creon, which is a prescribed medication for people who are not able or unable to digest foods in a normal way.

This is caused by cystic Fibrosis, which can cause problems in mobility.

Cysticfibrosis causes swelling of the fingers at the ends.

This condition is called “clubbing” and can cause patients difficulty with holding their fingers.

Cystic Fibrosis sufferers are at greater risk of developing thinning bones.

People with the disease experience low bone density and muscle weakness.

Stephanie is at risk of sustaining bone injuries.

Stepahine could face difficulties with her daily living activities, such as washing and bathing.

Stephanie is dependent on insulin administration so it is easy to spot her in inactive conditions.

Additionally, she uses inhalers/nebulisers. This could indicate that she might have functional problems (James et. al., 2014.).

Complexity of Caring For A Person In The Community. Proactive Care To Keep The Client Healthy

For the best patient outcomes, cystic Fibrosis must be treated as a multisystem disorder.

Caregiving patients can be difficult and complex. The present case is an example of this.

Comprehensive care for adolescents living with cysticfibrosis has been well-researched in the literature since long (Steinkamp et. al., 2015).

Stephanie’s health-related quality life is very important in the early years of her adulthood. Stephanie is going through developmental and psychosocial transitions.

Stephanie is a socially isolated patient who does not get the care she deserves.

Because her parents are not able or willing to spend time with her and her siblings, the psychological effects on the patient are mostly negative.

OpokaWiniarska et.al. (2015) suggested that patients with cystic fibris should have strong relationships with loved ones and be able to communicate the new needs to them.

Stephanie might have difficulty adhering to her care plan if she does not feel secure with her family.

This is because of the potential for mismanagement. Cystic Fibrosis care must be managed professionally and skillfully.

Recommendations on Resources/ Services that Are Required For Caring for The Client, Including Pharmacological Management

The multisystemic involvement and responsibility of care specialists for treatment and follow-up care is essential for cystic fibrosis.

Stephanie’s care plan will focus on maintaining lung function and administering nutritional therapy. It also aims to manage any complications. (Smyth et. al., 2014).

Rosenthal and Lehne (2014) pointed out the important role of medication management in cystic fibrillis care plans.

These medications could include mucolytics. Mutlivitamins. Pancreatic enzyme supplements. Bronchodilators. Anti-inflammatory drugs.

Ivacaftor, a medication that improves the functioning of the lungs, and decreases the amount salt in sweat, might be administered to the patient.

The patient will be referred to chest therapy. This is done by loosening the thickening of the mucus.

As well as demonstrating breathing techniques, a therapist helps with this process.

It might be recommended that pulmonary rehabilitation is done to improve overall health.

Rehabilitation can be done in a community centre. It would include exercise, nutrition counseling, patient education, as well as mobility and physical exercises (Regan and al., 2014.).

The patient needs nutritional support. Therefore, Stephanie should have a diet chart that is tailored to her body.

A nutritionist can be consulted to help the patient get the right nutritional intake.

She might be advised, among other things, to consume fat-soluble vitamins as well as supplementary nutrition.

Stephaine should be referred by the local support group to receive coping strategies.

Stephanie can connect with others and adolescents living with cystic fibrillis to share feelings and experience.

This is a very effective way to avoid depression (Tointon & Hunt (2016)).

Family therapy is a good option to strengthen the bond between the patient, her family and friends ().

Family members will be counselled on how to spend more time with the patient, in order to avoid social exclusion or depression that can result from health problems.

Adolescents need to be addressed with psychosocial issues in an effort to reach the desired patient outcomes.

Conclusion

This paper focused on the case of a 15year-old girl with cystic fibrillis, malnutrition, and diabetes.

Literature supports the diagnosis and treatment of patients with diabetes and malnutrition.

To improve quality of patient’s life and manage multifactoral health complications, it is necessary to use a comprehensive holistic approach.

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