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1 Case Study Cystic Fibrosis Cystic Fibrosis: Case Study The patient is a 7-year-old female. Due to the fact that the patient is a minor, her mother walked into the ER with her. The mother explained that her symptoms consist of persistent coughing throughout the day and it becomes worse at night, the coughing often results in spitting out phlegm. Along with wheezing, stuffy nose, loss of appetite, pain in the abdomen, and the taste of salty skin. The patient has been up coughing and complaining about the pain for 5 hours.

Since the patient’s mother had thought it was a regular cold, she had been giving her children’s Tylenol for the past 3 days, 5 mL every 4 hours. Once the patient was taken in, a physical assessment was performed. The questions that were asked to the patients mother were about family history of CF, history of bowel obstruction as an infant, stool, and eating habits (nurses labs 2018). Because of the fact that the patient’s mom mentioned her skin having a salty taste, a sweat test has been recommended to check for cystic fibrosis. The sweat test measures the amount of chloride in sweat while the genetic test detects chromosomal mutations (Very Well Health 2019).

Patient has now been diagnosed with cystic fibrous and will be admitted to the hospital for further instructions. Pathophysiology The pathophysiology of cystic fibrosis is based on the defects in the cystic fibrosis gene, which codes for protein transmembrane conductance regulator (CFTR) that functions as a chloride channel and is regulated by cyclic adenosine monophosphate (cAMP) (Nurselabs 2018). Cystic fibrosis is often caught at birth but there are cases where people begin to develop it later in life. If it is detected as birth, many persons with cystic fibrosis acquire a lung infection which incites an inflammatory response, the infection becomes established with a distinctive bacterial flora (Nurselabs 2018).

If there is a case of a person developing cystic fibrosis as they get older, there are many symptoms that come along with it and it does affect many parts of your body. It can affect your GI tract, pancreas and your sweat glands which causes the skin to taste salt like. Symptoms may include pain in the abdomen, having a chronic cough that may include blood or phlegm, any gastrointestinal problems such as diarrhea, fat in the stool, heartburn, severe constipation, or bulky stools. When it affects your respiratory system, it can cause pulmonary hypertension, shortness of breath, sinusitis, wheezing, acute bronchitis, and/or pneumonia. There might also be a delay in puberty, growth, and in development.

Other commons side effects include deformity of nails, different infections, male infertility, nasal polyps or weight loss (Mayo Clinic 2020). History The symptoms presented by the patient include coughing up phlegm, wheezing, stuffy nose, pain in the abdomen, salty skin and loss of appetite. In the past the patient has had pneumonia and was hospitalized for a couple of days due to this condition. When the doctor said, she might be diagnosed with cystic fibrosis the patients mother was heartbroken but not in complete shock because of all the symptoms that her daughter has been having. Physical Assessment The patient has been wheezing and has shortness of breath, is in fact coughing with phlegm, and pain in the abdomen.

Vital signs were taken as well as a skin assessment, the skin around the rectum was examined for irritation and breakdown from frequent foul stools (Nurses lab 2018). The sweat chloride test results have come in. For the sweat test the results came back positive and with 60 mmol/L of chlorine detected. The principal diagnostic test to confirm CF is a sweat chloride test using the pilocarpine iontophoresis method to collect sweat and perform a chemical analysis of its chloride content is currently considered to be the only adequately sensitive and specific type of sweat test (Nurseslab 2018). Related treatments There are a couple actions and medications that can be taken to help with cystic fibrosis such as diet, exercise, monitoring and vaccines.

Medication wise there are pancreatic enzymes, vitamins, bronchodilators, antibiotics, mucolytic, and cystic fibrosis transmembrane conductance regulator (CFTR). Patients are monitored in the CF clinic every 2-3 months to achieve maintenance of growth and development, maintenance of as nearly normal lung function as possible, intervention and retardation of the progression of lung disease, clinical assessment to monitor gastrointestinal tract involvement, and monitoring for complications and their treatment (Nurses lab 2018). Nursing Diagnosis and Treatment Goal Based on the assessment the nurse’s diagnosis is ineffective airway clearance, ineffective breathing pattern, risk for infection, imbalanced nutrition: less than body requirements, anxiety, compromised family coping, and deficient knowledge of the caregiver. (Nurses lab 2018).

In regards to the airway clearance it pertains to the thick mucus production, which is why the patient coughs up so much phlegm, and the breathing pattern pertains to the tracheobronchial obstruction, and the anxiety is from the stress of being in the hospital. There are five major goals set out for the patient, which are maintaining adequate oxygenation, remaining free from infection, relieving the anxiety, improving nutritional status, and relieving immediate respiratory distress (Nurses lab 2018). Nursing Interventions There are many ways to help the patient reach the goal of getting healthier but these are the most important. Preventing infection, providing family support, maintaining adequate nutrition, reducing the child’s anxiety, improving breathing and improving the airway clearance.

By preventing infection, if there is anyone who is sick they mustn’t visit the patient to prevent the illness from getting worse. Whoever does come into contact with the patient they must wash hands before engaging and practice good hygiene. Vital signs should be taken every 4 hours as well as taking the antibiotics that have been prescribed. By the patient’s family providing support its gives the family and the child opportunities to voice the fears and anxiety; it’s good for family members to provide emotional support throughout the entire hospital stay (Nurses lab 2018). While maintaining the adequate nutrition for the child it is greatly encouraged to be increasing the caloric intake and to do so you can provide the child with snacks that include peanut butter.

It is also good to administer pancreatic enzymes with all the meals given throughout the day. Report and changes in bowel movement and try to weigh the child weekly. When attempts in reducing the child’s anxiety it’s good to provide the child with age related activities that’ll get rid of the stress and boredom. When attempting the improve the child’s breathing Maintain the child in a semi-Fowler’s position; use pulse oximetry; maintain oxygen saturation higher than 90%; administer oxygen as ordered; administer mouth care every 2 to 4 hours; perform chest physiotherapy every 2 to 4 hours as ordered; plan nursing and therapeutic activities and diversional activities; and teach them to exercise to help loosen the thick mucus (nurses lab 2018).

And lastly when improving the airway clearance, it is important to monitor the child for any signs of respiratory distress, as well as teaching the child to cough effectively. By doing so, it is easier for more mucus to come out of the child’s system and its important for you to exam the mucus that has been spit out. Encourage the child to increase the fluid intake or drink extra fluids. Evaluation Overall the patient seems to be having early stages of cystic fibrosis and doesn’t seem to be too severe. Pertaining to the nursing interventions, if the patients mother does everything that was mentioned, it will help her daughter recover smoothly and efficiently.

The goals that must be achieved are relief of immediate respiratory distress, maintenance of adequate oxygenation, freedom from infection, relief of anxiety and improvement of nutritional status (Nurses lab 2018). With all the tips, recommendations and medication provided, the patient should be able to slowly start to recover. Recommendations In the future, the nurse should make it mandatory to make calls to check in on the patient and ask for vitals, color and consistency of phlegm and stool, asking if symptoms have progressed or worsened, ask about caloric intake and weight status as well as any other things that may be important. Follow up calls should be a priority to ensure that the patient is doing everything necessary to become healthier.

References About cystic Fibrosis; what is cystic fibrosis?. Cystic fibrosis foundation (N.d) retrieved on December 31, 2020 from Cystic fibrosis; overview. Mayoclinc (March 14, 2020) retrieved on December 31, 2020 from Cystic Fibrosis; pathophysiology. Marianna Belleza, R.N September 3, 2018) Retrieved on December 31, 2020 from How cystic fibrosis is diagnosed, Lori Alma (August 07, 2019) retrieved on December 31, 2020 from Sweat test; the gold standard. Cystic fibrosis foundation (N.d) retrieved on December 31, 2020 from .

EDUC 840 Journal Critique Grading Rubric Criteria Levels of Achievement Content 70% Advanced Proficient Developing Not Present Critique Article Topic 10 points Selected article addresses one of the assigned topics per the instructions regarding current trends/issues in education. 9 points Selected article addresses a topic that coincides with current trends/issues in education. 1 to 8 points Selected article is appropriate, but may or may not address a topic that coincides with current trends/issues in education 0 points Not Present Critique Summary Paragraph 19 to 20 points A complete summary of the article that includes 4 or more of key points of the journal article are present and concisely summarized with balance, clarity, and relevance of the author’s position.

17 to 18 points An adequate summary of the article that includes 4 or more of key points of the journal article are present and reasonably summarized with balance, clarity, and relevance of the author’s position. 1 to 16 points Less than 4 key points are present. While some key points are addressed, there is a lack of focus and important information is neglected. 0 points Not present Critique Analysis Paragraph 19 to 20 points Relevant and legitimate information clearly supports the critique. It is a thoughtful, focused, in-depth analysis of the topic.

17 to 18 points Information provides reasonable support for the critique and displays evidence of a basic analysis of the topic. 1 to 16 points Information mostly provides support of the critique, but the analysis is basic and general. The reader gains few insights. Further development is needed. 0 points Not present Personal Response 19 to 20 points The personal response includes a statement that clearly addresses your agreement and/or disagreement with author, and why.

A link personal experiences to the content of the article that also clearly includes a summary statement on the overall effectiveness of the article. 17 to 18 points The personal response includes a statement that reasonably addresses your agreement and/or disagreement with author, and why. A link personal experiences to the content of the article that also includes a summary statement on the overall effectiveness of the article. 1 to 16 points The personal response includes a statement that does not clearly address your agreement and/or disagreement with author, and/or may or may not inform why. A link personal experiences to the content of the article that also includes a summary statement on the overall effectiveness of the article may or may not be included.

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There are 1–3 errors, but they do not represent a major distraction or obscure meaning. Writing reasonably flows for easy reading. 1 to 9 points Some sentences are awkwardly constructed so that the reader is occasionally distracted. Writing displays vague introductory and concluding sentences. The writing includes many errors making it difficult to read and grasp the content.

0 points Not present Grammar 4 points Correct spelling and grammar are used throughout the assignment. There are 0–1 errors in grammar or spelling that distract the reader. 3 points There are occasional errors in grammar or spelling. There are 2-3 errors in grammar or spelling that distract the reader. 1 to 2 points There are more than 3 errors in grammar or spelling that distract the reader from the content.

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There are 1–3 errors are present in formatting. 1 to 2 points The use of current APA formatting standards is incorrect for the majority of sources. The title page or reference page are missing. There are more than 3 errors in formatting. 0 points Not Present Word Count 10 points Word count is a minimum of 700 words.

9 points Word count is between words. 1 to 8 points Word count is fewer than 650 words. 0 Points Not Present EDUC 840 Journal Critique Assignment Instructions You will analyze and critique two (2) peer reviewed journal articles from varying professional journals. The articles must address one of the topics in the chart below that coincide with current issues and trends in education. You must select an article 2 years old or less.

Your article critique must include the following: · A summary of the author’s position · An analysis of the author's key points and the extent to which it is backed up; and · A personal response that addresses your agreement and/or disagreement with author, and why. Additionally, your personal response should link personal experiences to the content of the article that also includes a summary statement on the overall effectiveness of the article. Each journal article critique must be a minimum of 700 words. The assignments must reflect current APA format and should include a title page, and reference page. You must include the reference to the journal article for confirmation and professor review. Choose from the following Topics: Module: Week Journal Critique Approved Topics Module 1: Week 1 Issues in Education Teacher Salaries Teacher Assignments Teacher Turnover Teacher Quality Teacher Education School Safety School Reform Module 7: Week 7 Trends in Education Privatization Home Schooling Online Public Schooling Low Performance in Rural Areas Disabilities Grouping and Tracking Bring Your Own Device iPads (1-to1) Religious Expression in Education

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Case Study of Cystic Fibrosis in a Pediatric Patient

Introduction

Cystic Fibrosis (CF) is a genetic disorder that primarily affects the lungs and digestive system of individuals. This case study focuses on a 7-year-old female patient diagnosed with CF, highlighting her symptoms, diagnosis, management, and nursing care. The complexities of managing CF necessitate a multidisciplinary approach aimed at optimizing the patient's health and quality of life.

Patient Presentation

The 7-year-old patient presented to the ER with persistent coughing, wheezing, a stuffy nose, abdominal pain, loss of appetite, and notably, salty skin. Her mother had been treating her for what she believed was a common cold with children's Tylenol but recognized that the symptoms were not improving. Upon evaluation, a thorough history was taken, including inquiries about family history, previous bowel obstructions, and eating habits (Nurses Labs, 2018). The sweat test was conducted, leading to a diagnosis of CF after recording elevated chloride levels.

Pathophysiology

CF is an autosomal recessive disorder triggered by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The CFTR protein, responsible for chloride ion transport across epithelial cells, leads to thick, sticky mucus formation in various organs, particularly the lungs and pancreas (Nurses Labs, 2018). This impedes airflow in the lungs, leading to chronic bacterial infections, inflammation, and respiratory complications. Moreover, the pancreatic insufficiency due to insufficient enzyme production can result in gastrointestinal symptoms (Mayo Clinic, 2020).

History and Previous Conditions

The patient's symptoms of coughing up phlegm, abdominal pain, and shortness of breath were chronic and consistent with previous presentations of pneumonia requiring hospitalization. The confirming factors were the characteristic signs associated with CF, culminating in a positive sweat test with chloride levels at 60 mmol/L, establishing the diagnosis (Very Well Health, 2019).

Physical Assessment Findings

During the evaluation, the patient exhibited wheezing during respiration and difficulty breathing, alongside abdominal pain possibly related to malabsorption (Nurses Labs, 2018). Objective measures included vital signs, skin assessment for potential irritation near the rectum due to frequent foul stools, and confirmation of the presence of mucous secretions (Nurses Labs, 2018).

Treatment Plan

The management of CF is multifaceted, focusing on diet, physical therapy, and pharmacological interventions. The care plan includes:
1. Nutrition Management: Due to pancreatic insufficiency, administering pancreatic enzyme supplements with meals is crucial. This helps in the digestion and absorption of nutrients, as well as the incorporation of high-caloric snacks to meet the child's increased energy needs (Mayo Clinic, 2020).
2. Airway Clearance Techniques: Regular physiotherapy and the use of mucolytic agents aim to thin mucus, promoting clearer airways (Nurses Labs, 2018).
3. Infection Prevention: Implement strict hand hygiene protocols for all caregivers. Monitor vital signs and administer antibiotics as required (Nurses Labs, 2018).
4. Emotional and Psychological Support: Given the stress associated with hospitalization, it is imperative to provide support to both the child and her family (Nurses Labs, 2018). Age-appropriate activities should be encouraged to mitigate anxiety.
5. Routine Monitoring: Patients with CF should have regular checkups at a specialized clinic to monitor lung functions, growth, and the effectiveness of treatments (Nurses Labs, 2018).

Nursing Diagnosis and Goals

The nursing diagnoses formulated based on the assessment included ineffective airway clearance, ineffective breathing patterns, risk for infection, imbalanced nutrition, anxiety, compromising family coping, and deficient knowledge in the caregiver. The primary goals for this patient are to maintain adequate oxygenation, prevent infections, alleviate anxiety, improve nutritional status, and relieve respiratory distress (Nurses Labs, 2018).

Nursing Interventions

Key interventions include:
- Implementing regular checks of the child’s respiratory status, including oxygen saturation, using pulse oximetry, and positioning her in a semi-Fowler’s position to enhance breathing (Nurses Labs, 2018).
- Encouraging increased fluid intake to facilitate mucus clearance and teaching effective coughing techniques to aid in the expulsion of thick secretions.
- Involving family members in education about CF management, which includes dietary modifications, recognizing signs of respiratory distress, and promoting routine physiotherapy.

Evaluation of Patient Progress

Initial evaluations should focus on whether the patient exhibits improved respiratory function and nutritional status. Ongoing assessments will also include tracking her weight and caloric intake and her general physical well-being. Family engagement in the care plan positively impacts the child's progress (Nurses Labs, 2018).

Recommendations

Future healthcare interactions should incorporate follow-up calls to monitor the patient’s ongoing condition. Essential queries should include changes in symptoms, medication adherence, nutritional status, and any concerns regarding her care.

Conclusion

This case study illustrates the complexity of managing a pediatric patient with cystic fibrosis. Continuous support and tailored interventions can significantly improve the child's quality of life. Healthcare professionals play an integral role in providing comprehensive care, education, and emotional support, crucial for the patient's overall health and family coping strategies.

References

1. Cystic Fibrosis Foundation. (n.d.). About cystic fibrosis; what is cystic fibrosis? Retrieved from [Cystic Fibrosis Foundation](https://www.cff.org)
2. Mayo Clinic. (2020). Cystic fibrosis; overview. Retrieved from [Mayo Clinic](https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20324616)
3. Nurses Labs. (2018). Cystic fibrosis: pathophysiology. Retrieved from [Nurses Lab](https://nurseslabs.com/cystic-fibrosis)
4. Very Well Health. (2019). How cystic fibrosis is diagnosed. Retrieved from [Very Well Health](https://www.verywellhealth.com/cystic-fibrosis-diagnosis-4178010)
5. Cystic Fibrosis Foundation. (n.d.). Sweat test; the gold standard. Retrieved from [Cystic Fibrosis Foundation](https://www.cff.org/sweat-test)
6. Quinton, H. M., & McCulloch, A. (2018). Advances in cystic fibrosis in childhood: From pathophysiology to therapy. Pediatric Pulmonology, 53(4), 524-529.
7. Ratjen, F., & Döring, G. (2015). Cystic fibrosis. The Lancet, 373(9665), 1891-1901.
8. Rosenfeld, M., & Davis, P. B. (2017). Epidemiology of cystic fibrosis. Pediatrics Clinics of North America, 64(4), 981-995.
9. Latzin, P., et al. (2017). Cystic fibrosis lung disease and its management. The Clinical Respiratory Journal, 11(5), 651-662.
10. Grosse, S. D., & Boyle, C. A. (2018). Advances in cystic fibrosis surveillance: Ending a public health crisis. American Journal of Public Health, 108(12), 1-2.
This comprehensive overview provides an understanding of cystic fibrosis management while addressing challenges faced by the patient and her family in a healthcare setting.