Answer the following questions and read the case study.
Explain the pathophysiology, clinical manifestations, and causes of cystic fibrillosis.
Discuss the comorbidities of malnourishment or diabetes, as well as the effects of these conditions on the health of the person/family.
Analyze relevant literature to discuss the functional impairment related this case study.
Discuss the complex nature of caring for this client in the community. Also, reflect on the proactive care that is needed to keep them healthy.
Utilize evidence-based practice to recommend the resources and services needed to care for your client. This includes pharmacological management.
Stephanie Mills is 15 years old and was born with cysticfibrosis.
Stephanie lives with her parents as well as two of her older siblings, Tim (19 and Emily (17).
She meets regularly with the cystic Fibrosis team once every three months. At each visit, she must undergo blood tests and throat samples.
Stephanie is also diabetic and must check her blood sugar three times per day before she eats.
She will require insulin to control her blood sugar levels if it is too high.
She is also very malnourished and only weighs 50 Kgs.
Stephanie is taking multiple vitamins as well as a high-energy and high-protein diet.
Each day she receives around 30 minutes of physiotherapy, inhalers, and other therapies.
Every meal, starting with breakfast, requires her to take several “Creons” mixed with her food. This helps her to digest it.
Stephanie continues her evening with 40 minutes of inhalers and physiotherapy.
Stephanie will need to perform her own breathing exercises, do peak flow tests and administer her medications.
She has to also be on a variety antibiotics which are constantly changing.
While she is encouraged and supported to be active, she struggles to find the energy or time.
The family must take all of her medication and equipment with them when they go on holiday.
Stephanie must follow her daily physio schedule, whether they are going out for the day or on holiday.
This can cause a lot of frustration for the family, and it can also be quite difficult at times.
The family sometimes takes on the responsibility of Stephanie while the rest of the family goes on holiday.
Stephanie’s dad is a full-time retailer manager. Stephanie often does not get home until the late hours of the night.
Alice, Stephanie’s mother, works part-time as a caterer to accommodate Stephanie’s care needs.
Tim is a student during the day, and works as a milk bar manager in the evenings.
Emily, who is in her eleventh year, attends the local highschool and is involved with netball.
The multiple admissions, and inability of mum to go to work have meant that the family has struggled financially to pay for Tim’s university fees.
The case study of Stephanie Mills (15 years old) who was diagnosed at birth with cystic fibrosis is the basis for this paper.
First, we will discuss the pathophysiology and related clinical manifestations of cystic fibrosis.
The paper then examines the comorbidities and impact of malnutrition on the patient’s overall health. Finally, the paper reviews the literature to discuss the functional impairment that was associated with the case study.
It then discusses the complex nature of caring in the community for the patient and the proactive care needed.
It is also recommended to consider the availability of resources and services for patient care, which includes pharmacological management.
Pathophysiology and Clinical Significances of Cystic Fibrosis
Cystic fibrosis, a genetic disorder affecting the exocrine, is a condition that affects the lungs and liver. It can also affect the intestine, liver, kidneys, and pancreas.
Cystic fibrosis is an inherited recessive disorder that is caused by mutation of the gene cystic fibrosis Transmembrane Conductance Regulator (CFTR). CFTR functions normally as a chlorine channel and is regulated via cyclic monphosphate (cAMP). (Cuttin (2015)
Many mutations can occur within the CFTR gene. These mutations can lead to severe or mild effects in the protein formed from the gene.
Common defects include faulty protein folding, short length proteins and proteins that are incapable of using ATP energy.
This mutation limits the movement of iodine (chloride) and thiocyanate across cells membranes.
The main feature of this disease is the abnormalities in cAMP regulation of chloride transport across epithelial cell mucosal surfaces.
The defect results in decreased secretion of chlorine and an increase in reabsorption water and sodium from epithelial cells.
Inflammation and infection can be promoted by increased mucus production due to reduced mucus hydration.
Other exocrine tissues such as the GI tract and pancreases produce more mucus than other exocrine ones (Martin et.al., 2017).
Stoltz, et al. (2015) reported that cystic Fibrosis sufferers have both clinical manifestations and exocrine pancreatic dysfunction.
Other clinical manifestations are also of concern.
The lower airway may be affected by chronic infections such as Haemophilus influenzae, staphylococcus Aureus and Pseudomonas Aeruginosa.
Other symptoms include acute exacerbations (bronchiectasis), allergic bronchopulmonary asthmapergilllosis, and atypical mycobacterial infections.
Coeliac disease, distal intestinal obstruction syndrome, severe constipation Crohn’s Disease, severe constipation, Crohn’s disease, and giardiasis are some of the gastrointestinal complications.
The sweat gland can be affected by electrolyte loss.
Patients may also experience genitourinary problems, bone diseases, such as immobility, pelvic delay and malabsorption, as well as endocrine and pancreatic complications such as insulin deficiencies.
The majority of CF-related deaths are due to respiratory disease.
Cystic Fibrosis manifests itself as poor growth, salty-tasting, and low weight. There are also frequent shortness of breathing, coughing, and thick mucus.
The impact of malnourishment and diabetes on the health of the individual and her family
Both malnutrition and diabetes are health conditions that are well-known for their comorbidities.
Hall (2015) highlighted the many comorbidities associated with diabetes and their impact on the patient’s health.
Uncontrolled hypertension and high blood pressure are two of the most common complications of diabetes.
Combining diabetes and hypertension increases the risk of developing diabetic complications.
Atherosclerosis occurs when blood vessels become narrowed due to clogging.
The patient experiences angina and a reduced blood supply.
This increases the risk of suffering a stroke or heart attack.
Dyslipidemia means that there are abnormally high blood levels of cholesterol.
Such a condition greatly increases your risk of developing cardiovascular diseases.
Non-alcoholicfatty liver disease refers specifically to patients who have high liver enzymes and a larger liver.
40 percent of diabetics suffer from chronic kidney disease, which is damage to the nerves and blood vessels in the body.
Malnutrition can lead to complications such as weight loss, developmental delay and suppressed immunity.
While the short-term effects of developmental delay and immune system impairment can be temporary, growth delays can have long-term implications (Ibrahim et. al., 2017).
Malnutrition can compromise the immune system and make the patient more vulnerable to infections.
A nutrient deficiency can lead to gastrointestinal infections. Growth retardation is caused by nutrient deficiency and infection.
Malnutrition affects bone growth.
One nutrient shortage could lead to another.
Long-term complications like protein deficiencies can result from such deficiencies.
Diabetes has a profound impact on both the family and the patient’s lives. It is also more noticeable than the emotional effects.
As mentioned, anxiety can result from diabetes-related health issues.
Patients and their families can struggle to manage stress and anxiety.
Stephanie can experience grief stages. She is able to communicate her worries to her family members, who may find it difficult to accept the situation.
Because she is unable to spend time with her family because of their work and personal commitments, it can lead to stress and depression.
Family members may also experience stress from the depression of the patient. They should take the time and get to know the patient (James et. al., 2014).
Stephanie has malnutrition and the family is responsible for ensuring proper nutrition intake.
Stephanie’s family is involved in her care (Hockenberry & Wilson 2014).
Study on the Associated Functional Impairment
Stephanie is currently suffering from functional and cognitive impairments due to malnutrition.
Malnutrition can slow down cognitive and motor development.
This can lead to impaired school performance, attention deficit disorder and learning disabilities. It also leads to decreased intellectual ability, reduced problem solving abilities, and decreased school performance (Prado & Dewey 2014).
Stephanie is 15 years of age, so her health conditions might be affecting her academic performance.
Huysentruyt, et. al. (2017) have found that cystic Fibrosis can be associated with functional impairments.
Cystic Fibrosis symptoms vary among patients. They include digestive symptoms such stomach cramps, bloating, constipation and respiratory symptoms such coughing, fever, shortness of breathing, and chest pains.
Patients with these conditions have difficulty maintaining a stable lifestyle.
Stephanie was prescribed Creon as a medicine to treat people who cannot digest food properly.
This condition can lead to mobility difficulties and cystic fibrosis.
Cystic Fibrosis also causes swelling at the ends of fingers, causing them to become longer.
This condition is called clubbing, and it causes difficulty holding onto the fingers.
People with cystic Fibrosis are at greater risk of developing bone thinning.
The disease causes low bone density, which can lead to muscle weakness.
Stephanie is therefore at greater risk of experiencing bone injuries.
Stepahine may face difficulties while eating and performing daily activities such as bathing and washing dishes.
Because Stephanie requires insulin administration, it’s not surprising to find her inactive at times.
Inhalers and nebulisers are used by Stephanie, which suggests that she could have functional difficulties (James, et al. 2014).
Complexity of caring for the person in the community and proactive care intended to keep them well
Cystic Fibrosis is multisystem disease and requires comprehensive care to ensure the best possible patient outcomes.
There are many complexities and challenges involved in caring to patients, which is even more evident when the patient has just turned 18.
In literature, holistic care has been discussed for adolescents with cystic-fibrosis since a long time (Steinkamp, et al. 2015).
Stephanie’s early adulthood is a time when Stephanie experiences significant psychosocial and developmental changes. Stephanie’s health-related quality of living is important.
Stephanie is most difficult to care for because she is socially isolated and does not receive the love and affection she desires.
Her parents are not able or willing to spend time with her and her siblings, which has a negative psychological effect on her.
OpokaWiniarska and her colleagues (2015) have stated that patients suffering from cystic fibrillus need to build strong relationships with family members. The new needs of the patient must be addressed.
It might be difficult for Stephanie to follow her care plan if she does not feel a strong bond with her family.
Cystic fibrosis care requires professional and skilled management. This is why this statement was made.
Recommendation of Resources/ Services that Are Required For Caring for The Client, Including Pharmacological Management
Multiple systemic involvement is required for cystic fibrosis. These specialists are responsible both for treatment and followup.
Stephanie’s primary goals would be to maintain lung function close to normal, administer nutritional therapy, manage complications, and provide emotional and/or social support (Smyth, et al. 2014).
Rosenthal (2014) and Lehne (2014) said that medication management is an important part of cystic Fibrosis care.
This includes mucolytics. mutlivitamins. pancreatic enzymes supplements. Bronchchodilators. Anti-inflammatory agents.
Ivacaftor is a medication that can improve the function of the lungs as well as reduce salt levels in the sweat.
The patient should be referred for chest physical therapy, which loosens the thick mucus from the lungs.
This is assisted by a therapist who also demonstrates breathing techniques.
A recommendation for pulmonary rehabilitation could be made to improve overall well-being.
A community centre can provide rehabilitation that includes physical exercises, mobility enhancements, nutritional counseling, and patient education (Regan and colleagues, 2014).
It is important to provide nutritional support to the patient. A diet chart must be prepared according Stephanie’s needs if she is suffering from malnutrition.
For this purpose, a nutritionist will be consulted. This is to ensure the patient has adequate nutritional intake.
It is possible that she will be advised to consume special fat-soluble vitamins and supplementary nutrients, as well as maintain adequate fluid intake.
Stephaine must be referred to a local support group, who will provide support and help with coping strategies.
Stephanie can meet others with cystic-fibrosis, as well as adolescents. They can share their feelings and experiences.
This is a good way to reduce the risk of depression (Tointon & Hunt 2016).
Family therapy can be very beneficial in strengthening the family relationship of the patient and her loved ones ().
Families should be counselled to make sure they spend time with the patient and not suffer from depression or social exclusion due to health issues.
Adolescent patients need to address their psychosocial issues to ensure they achieve the desired patient outcomes.
The case study was an analysis of a 15-year old patient with cystic Fibrosis and diabetes.
In literature, it is clear that diabetes and malnutrition are common comorbidities.
For the best quality of life, the patient must be treated holistically and managed in an appropriate manner.
A systematic review of the issues surrounding improving adult healthcare transition for young cystic fibrosis patients:
Journal of Child Health Care. 1367493517712479.
Genetics of cystic Fibrosis: from molecular understanding and clinical application.
Nature Reviews Genetics, 16(1) 45-56.
Guyton and Hall Textbook Of Medical Physiology Ebook.
Wong’s Nursing Care of Infants and Children E-Book.
SUN-263: Handgrip Force for Children and Adolescents With Cystic Fibrosis: Effect of Nutritional and Pulmonary Condition.
Clinical Nutrition, 36. S151.
Impact of childhood malnutrition in host defense and infection.
Clinical microbiology Reviews, 30(4), 919-971.
Nursing Care of Children-Ebook: Principles & Practice.
Pharmacology of Nursing Care-Ebook.
Targets for cystic fibrosis lung diseases using ion channels
Journal of Cystic Fibrosis.
Problems faced by patients with cystic Fibrosis as they transition into adulthood.
Advances in Respiratory Medicine. 83(5): 394–400.
Nutrition and brain development during early life.
A prospective cohort study that examined the prevalence of emotional & behavioural difficulties in children who have cystic fibrosis.
Journal of Cystic Fibrosis 13: S114.
European Cystic Fibrosis Society guidelines of care: best practices.
Journal of cysticfibrosis 13, S23-S42.
Cystic fibrosis care through the eyes of patients-A nationwide survey asking for feedback on patient satisfaction and experience with services. The questionnaire was designed to assess patients’ satisfaction.
New England Journal of Medicine, 372, pp. 351-362.
A holistic approach to nursing can improve the quality of life for children with cystic-fibrosis.
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