Cystic Fibrosis: Assessment, Physiological Effects, Risks, Ethical Considerations, and Treatment

QUESTION

Case Study

J.R. is a 13-year-old boy diagnosed with cystic fibrosis (CF) who is being seen in the outpatient clinic for biannual evaluation. He lives in the house with his parents and has a sister C.R. 7-year-old who also has cystic fibrosis. J.R. reports that he has not felt well, that he has no energy, is coughing more and has difficulty breathing. He explains that he has been absent from school for a week.

Questions:

1. Discuss what additional data nursing should obtain from J.R. and of their parents.

2. Briefly describe the condition, physiological effect on the following systems:

– respiratory

– gastrointestinal

– player

– skin

– electrolyte balance

3. Identify the factors that put J. R. at risk for developing respiratory infections.

4. Discuss the ethical and social principles that may be affected by the patient’s chronic condition as they progress through the various stages of growth and development.

Case Study Progress:

J.R. is later admitted to the hospital for suspected respiratory infection and exacerbation of CF.

His estimate includes vital signs: BP 115/76, P 85, R 28 and Temp. 101.8oF (38.8oC). Sat. O2: 88% in ambient air. Weight: 30 KG, Color: pale and dry skin with cyanotic nails and drum fingers. Capillary refill of 3 seconds. Labored breathing, productive cough, snoring, pain scale 3-4 (scale 10) when coughing. Barrel-shaped chest, looks thin with decreased muscle mass. Urinary and fecal elimination in the morning without problem. Anxious patient and answers questions in short sentences.

5. Indicate with an X which System is affected and why. Provide a short nursing note in narrative format (DAR format).

□ Neurological:

□ Respiratory:

□ Cardiovascular:

□ Gastrointestinal:

□ Genitourinary:

□ Musculoskeletal:

□ Skin:

□ Psychosocial:

□ Pain:

Nursing narrative note:
6. What is the most common microorganism that causes respiratory infection in children with CF and why?

Medical orders:

– Ceftazidine (Fortaz) 2G IV q 8h

– Gentamicin (Gentak) 100 mg IV q 8h

– Vancomycin (Vancocin) 450 mg IV q 8h
7. Are the previous prescribed doses in safe ranges? Comment on each medication, the doses and justify with information from a nursing medication reference book.

8. After treatment, the child improves and is discharged. Discuss the health promotion aspects to prevent exacerbation of the condition that should be emphasized with J.R.’s parents.

ANSWER

Cystic Fibrosis: Assessment, Physiological Effects, Risks, Ethical Considerations, and Treatment

Introduction

This case study focuses on J.R., a 13-year-old boy with cystic fibrosis (CF), who presents with symptoms of respiratory infection and exacerbation of his condition. In this essay, we will discuss additional data that nursing should obtain, describe the physiological effects of CF on various body systems, identify risk factors for respiratory infections, explore ethical and social principles affected by J.R.’s chronic condition, evaluate the nursing note, review the common microorganism causing respiratory infections in CF, assess the safety of prescribed medication doses, and discuss health promotion aspects for preventing exacerbation of CF.

Obtaining Additional Data

Nursing should gather more information from J.R. and his parents to gain a comprehensive understanding of his condition. This includes details about the duration and severity of his symptoms, any recent changes in his medication or treatment plan, adherence to treatment, exposure to sick contacts, and any triggers or factors that could have contributed to the exacerbation. It is also essential to inquire about the symptoms and current condition of J.R.’s sister, C.R., who also has CF, as she could be a potential source of infection.

Physiological Effects on Body Systems

Respiratory: CF primarily affects the respiratory system, leading to thick, sticky mucus production, impaired mucociliary clearance, and increased susceptibility to respiratory infections.
Gastrointestinal: CF can cause pancreatic insufficiency, leading to malabsorption of nutrients, steatorrhea, and poor weight gain.
Pulmonary: In CF, chronic inflammation and recurring infections can result in progressive lung damage, leading to respiratory symptoms and decreased lung function.
Skin: The sweat glands in individuals with CF produce excessively salty sweat, impacting skin integrity and electrolyte balance.
Electrolyte Balance: CF disrupts the balance of electrolytes, particularly chloride and sodium, leading to abnormal transport across cell membranes and subsequent imbalances.

Risk Factors for Respiratory Infections

Several factors put J.R. at risk for respiratory infections. These include the impaired mucociliary clearance due to thick mucus, reduced lung function, chronic inflammation, and exposure to pathogens in healthcare settings, schools, and the community. Shared living conditions with his sister, who also has CF, increases the risk of cross-infection and transmission of respiratory pathogens.

Ethical and Social Principles

As J.R. progresses through the various stages of growth and development, ethical and social principles are affected. These include autonomy, as he needs to gradually assume responsibility for his own care; beneficence, as healthcare providers strive to promote his well-being; non-maleficence, ensuring that interventions do not cause harm; and justice, ensuring equitable access to care and resources despite his chronic condition.

Nursing Note Assessment

Neurological:
Respiratory: X (Affected due to labored breathing, productive cough, decreased lung function)
Cardiovascular:
Gastrointestinal:
Genitourinary:
Musculoskeletal:
Skin: X (Affected due to pale and dry skin, cyanotic nails)
Psychosocial: X (Anxious patient)
Pain: X (Pain scale 3-4 when coughing)

Nursing Narrative Note

J.R. is a 13-year-old boy with CF admitted for suspected respiratory infection and exacerbation of his condition. He presents with labored breathing, productive cough, and decreased lung function. Physical assessment reveals pale and dry skin with cyanotic nails. The patient appears anxious, answering questions in short sentences. He reports pain scale of 3-4 when coughing. Prompt intervention and treatment are initiated to improve his respiratory status and alleviate discomfort.

Safe Medication Doses

Ceftazidime (Fortaz) 2G IV q 8h, Gentamicin (Gentak) 100 mg IV q 8h, and Vancomycin (Vancocin) 450 mg IV q 8h are commonly used antibiotics in the treatment of respiratory infections. The prescribed doses should be verified with a nursing medication reference book to ensure they fall within safe ranges based on the patient’s weight, age, renal function, and the specific guidelines provided by the reference book.

Health Promotion for Preventing Exacerbation

After J.R.’s treatment and discharge, it is crucial to emphasize health promotion strategies to prevent future exacerbations. This includes adherence to prescribed medications, airway clearance techniques, proper nutrition and pancreatic enzyme replacement therapy, regular exercise, avoidance of respiratory irritants, frequent hand hygiene, and vaccination against respiratory pathogens such as influenza and pneumococcal infections.

Conclusion

This case study highlights the comprehensive care required for a child with CF. Nursing assessment should gather additional data to better understand the current condition and potential sources of infection. CF affects multiple body systems, necessitating holistic care and monitoring. Ethical and social principles come into play as the patient navigates different stages of growth and development. Safe medication doses should be verified using appropriate references. Finally, health promotion strategies are crucial to preventing exacerbations and optimizing long-term outcomes for individuals like J.R. with CF.