A 25-year-old female patient with cystic fibrosis has a significant decline in lung function, with an FEV1 dropping to 55% of the predicted value, accompanied by a sputum culture revealing Pseudomonas aeruginosa. Her white blood cell count is elevated, and her serum albumin is low. Which of the following nursing interventions is most appropriate to prioritize in her current care plan?

C) Administer systemic antibiotics as prescribed to target the Pseudomonas infection.

A) Increase the frequency of her chest physiotherapy sessions to improve airway clearance.

B) Initiate a high-calorie, high-protein diet to address her nutritional status.

C) Administer systemic antibiotics as prescribed to target the Pseudomonas infection.

D) Educate the patient on the importance of consistent use of pancreatic enzyme supplements.

A patient with cystic fibrosis has a recent decline in FEV1 to 55% of the predicted value and a sputum culture showing colonization with Pseudomonas aeruginosa. What is the most appropriate initial nursing intervention to address the patient's current respiratory status?

B) Increase the frequency of chest physiotherapy to enhance sputum clearance.

A) Administer bronchodilators as prescribed to open the airways.

B) Increase the frequency of chest physiotherapy to enhance sputum clearance.

C) Encourage the patient to increase fluid intake to thin secretions.

D) Recommend the use of a high-frequency chest wall oscillation vest.

A patient with a chronic respiratory condition is experiencing a mixed response to treatment, including a low-grade fever, decreased oxygen saturation, persistent fatigue, and reduced appetite. Her sputum culture continues to show Pseudomonas aeruginosa. Which of the following nursing interventions should be prioritized to address the patient's current clinical status?

C) Arrange for a consultation with a dietitian to address nutritional deficiencies.

A) Encourage the patient to participate in light physical activity to improve oxygenation.

B) Administer prescribed antipyretics to manage the low-grade fever.

C) Arrange for a consultation with a dietitian to address nutritional deficiencies.

D) Increase the frequency of respiratory therapy sessions to enhance airway clearance.

A patient with chronic respiratory issues and a recent infection due to Pseudomonas aeruginosa is being reassessed after showing mixed response to treatment. Given her decreased oxygen saturation, persistent fatigue, and poor nutritional status, which nursing intervention is the most appropriate to implement immediately?

B) Administer supplemental oxygen to maintain adequate oxygen saturation levels.

A) Increase the frequency of respiratory therapy sessions to enhance airway clearance.

B) Administer supplemental oxygen to maintain adequate oxygen saturation levels.

C) Schedule a follow-up appointment with the primary care physician to review treatment.

D) Educate the patient about the importance of adhering to her current medication regimen.

A patient presents with increased fatigue, new onset of chest pain, and decreased oxygen saturation. Crackles are heard in the lower lung fields, and her white blood cell count has risen, indicating a potential infection. Which nursing intervention is the priority to prevent further respiratory compromise?

B) Initiate supplemental oxygen therapy to maintain adequate oxygenation

A) Administer prescribed intravenous antibiotics immediately

B) Initiate supplemental oxygen therapy to maintain adequate oxygenation

C) Encourage the patient to increase oral fluid intake to thin secretions

D) Position the patient in a high Fowler's position to facilitate lung expansion

A patient presents with increased fatigue, chest pain on deep breaths, a fever of 101.3°F, and an oxygen saturation of 87% on room air. Crackles are noted in the lower lung fields, and a chest X-ray reveals patchy infiltrates. Which of the following interventions should the nurse prioritize to address the patient's immediate needs?

B) Initiate oxygen therapy to improve oxygen saturation levels.

A) Administer prescribed intravenous antibiotics to address potential infection.

B) Initiate oxygen therapy to improve oxygen saturation levels.

C) Prepare the patient for a CT scan to evaluate for a pulmonary embolism.

D) Start enteral feeding to address nutritional deficiencies.

A patient with cystic fibrosis is experiencing an exacerbation with mucus plugging and hypoxemia. Despite initial stabilization with antibiotics and nutritional support, her condition has worsened. Which priority nursing intervention would best address her current respiratory status?

A) Increase the frequency of chest physiotherapy sessions

B) Administer additional oral fluids to thin secretions

C) Arrange for immediate intubation

D) Initiate a low-sodium diet to reduce fluid retention

A patient with cystic fibrosis is experiencing increased respiratory distress and hypoxemia despite supplemental oxygen. The healthcare team has adjusted the treatment plan to include more targeted antibiotic therapy and enhanced airway clearance techniques. What is the priority nursing intervention to address the patient's respiratory condition?

D) Increase the frequency of nebulized hypertonic saline treatments.

A) Administer bronchodilator therapy to reduce airway obstruction.

B) Encourage the patient to take deep breaths and perform incentive spirometry.

C) Prepare the patient for intubation and mechanical ventilation.

D) Increase the frequency of nebulized hypertonic saline treatments.

A patient with cystic fibrosis is showing signs of improvement after 24 hours of intensified antibiotic therapy and enhanced airway clearance techniques. Despite this, her nutritional status remains poor with low albumin levels. Which nursing intervention is most appropriate to address the patient's nutritional needs?

B) Collaborate with a dietitian to develop a high-calorie, high-protein diet plan.

A) Increase the frequency of nebulized treatments to further improve lung function.

B) Collaborate with a dietitian to develop a high-calorie, high-protein diet plan.

C) Initiate non-invasive ventilation to reduce respiratory effort and conserve energy.

D) Encourage the patient to perform chest physiotherapy independently to enhance self-care.

A patient with cystic fibrosis is showing signs of partial improvement following an adjusted antibiotic regimen and enhanced airway clearance techniques. Despite these interventions, the patient's albumin levels remain low, and there is no significant weight gain. What is the most appropriate nursing action to address the patient's nutritional concerns?

B) Collaborate with a dietitian to develop a high-calorie, high-protein diet plan tailored to the patient's needs.

A) Increase the frequency of nebulized treatments to assist with better respiratory function and caloric intake.

B) Collaborate with a dietitian to develop a high-calorie, high-protein diet plan tailored to the patient's needs.

C) Monitor the patient's fluid intake closely to prevent fluid overload and ensure proper hydration.

D) Recommend the implementation of a daily exercise regime to improve appetite and overall strength.

Cyctic fibrosis - Nursing Case Study

Pathophysiology

• Primary mechanism: Cystic fibrosis is primarily caused by mutations in the CFTR gene, leading to dysfunctional or absent CFTR protein channels, which are crucial for chloride ion transport across epithelial cell membranes. This disruption impairs the regulation of salt and water movement, resulting in thick, sticky mucus production in various organs.

• Secondary mechanism: The accumulation of viscous mucus predominantly affects the respiratory system, obstructing airways and making them susceptible to chronic bacterial infections. This environment promotes persistent inflammation and progressive lung damage, a hallmark of the disease.

• Key complication: The obstruction and infection in the lungs lead to decreased pulmonary function over time, often progressing to respiratory failure. Additionally, thick mucus can block the pancreatic ducts, impairing digestion and nutrient absorption, which can result in malnutrition and growth issues.

Patient Profile

Demographics:

14-year-old female, student

History:

• Key past medical history: Diagnosed with cystic fibrosis at age 2, recurrent respiratory infections, pancreatic insufficiency

• Current medications: Dornase alfa, pancreatic enzyme supplements, azithromycin, inhaled tobramycin

• Allergies: Penicillin

Current Presentation:

• Chief complaint: Increased cough and difficulty breathing

• Key symptoms: Persistent productive cough, increased sputum production, fatigue, poor appetite, mild abdominal pain

• Vital signs: Temperature 100.4°F, heart rate 110 bpm, respiratory rate 24 breaths per minute, blood pressure 108/70 mmHg, oxygen saturation 89% on room air

Section 1

New Diagnostic Results:

Upon further examination and testing, the patient's recent pulmonary function tests (PFTs) reveal a significant decline in her lung function, with a forced expiratory volume in one second (FEV1) dropping to 55% of the predicted value, down from her baseline of 70% just two months ago. This decline indicates a worsening obstruction in her airways, raising concerns about potential exacerbation or new colonization of pathogenic bacteria. A sputum culture is conducted, revealing the presence of Pseudomonas aeruginosa, a common and often challenging pathogen in cystic fibrosis patients, known for its resistance to multiple antibiotics.

Additionally, laboratory results show an elevated white blood cell count of 14,000/µL with a left shift, supporting the suspicion of an active infection. Her serum albumin level is found to be low at 3.2 g/dL, suggesting possible malnutrition and poor protein intake or absorption. Given these findings, the healthcare team is prompted to consider adjustments in her treatment regimen to address the infection aggressively and to reassess her nutritional status. The results compel the team to evaluate the effectiveness of her current inhaled antibiotics and consider systemic antibiotics to better manage the Pseudomonas infection, while also contemplating potential modifications to her pancreatic enzyme therapy and dietary plan to improve her nutritional status.

These diagnostic results underscore the importance of a multidisciplinary approach, integrating respiratory therapy, nutritional support, and aggressive infection control to manage the complexities of cystic fibrosis. The healthcare team must exercise clinical reasoning to balance the immediate needs of combating the pulmonary infection while ensuring long-term management strategies are in place to support her overall health and development. This strategic response will aim to stabilize her condition and prevent further deterioration, setting the stage for the next steps in her treatment plan.

Section 2

Change in Patient Status:

Two weeks into the revised treatment regimen, the patient exhibits a mixed response, prompting a re-evaluation by the healthcare team. Initially, she shows signs of improvement in terms of respiratory symptoms; her cough has decreased in frequency and severity, and she reports less dyspnea during daily activities. However, during a routine follow-up appointment, her vital signs reveal a low-grade fever of 100.4°F, and her oxygen saturation has decreased to 90% on room air. These findings, coupled with her persistent fatigue and decreased appetite, suggest that despite some symptom relief, the underlying infection and nutritional issues may not be fully resolved.

Further laboratory tests are ordered to assess her current status. Repeat PFTs show a slight improvement in FEV1 to 58%, but this modest gain is not as significant as expected. A repeat sputum culture still indicates the presence of Pseudomonas aeruginosa, albeit in reduced quantities. Her white blood cell count remains elevated at 13,500/µL, and there is no significant change in her serum albumin, which remains low at 3.3 g/dL. These lab results suggest a partial response to the systemic antibiotics but highlight ongoing challenges with infection control and nutritional support.

In light of these findings, the healthcare team must employ clinical reasoning to refine the treatment strategy further. The decision is made to adjust her antibiotic therapy, possibly incorporating a combination therapy approach to target the resistant Pseudomonas more effectively. Additionally, a consultation with a dietitian is arranged to explore more aggressive interventions for her nutritional status, such as the initiation of nutritional supplements or adjustments to her pancreatic enzyme dosing. The team recognizes the importance of close monitoring and continuous reassessment to adapt her care plan dynamically, aiming to optimize her respiratory function and overall health while mitigating the risk of further complications.

Section 3

Two weeks following the adjustment in her treatment plan, the patient presents with a further change in her status that necessitates immediate attention. During a routine check-in, she reports increased fatigue and a new onset of chest pain, particularly when taking deep breaths. Her oxygen saturation has further declined to 87% on room air, and her fever has risen to 101.3°F. Her respiratory rate has increased to 28 breaths per minute, and there are noticeable crackles upon auscultation of the lower lung fields. These symptoms are concerning for a possible exacerbation of her condition or the development of a secondary complication such as pleuritis or a pulmonary embolism.

In response to these alarming developments, the healthcare team orders a series of diagnostic tests, including a chest X-ray, which reveals patchy infiltrates consistent with worsening infection or inflammation. A CT scan of the chest is recommended to further evaluate for potential complications such as a pulmonary embolism, given her increased respiratory distress and chest pain. Concurrently, blood cultures and additional sputum cultures are collected to assess for any changes in the microbial flora or the emergence of new pathogens that could be contributing to her declining condition.

The patient's latest laboratory results indicate a further increase in her white blood cell count to 15,200/µL, suggesting an acute inflammatory response or infection. Her serum albumin remains low at 3.1 g/dL, highlighting ongoing nutritional deficiencies despite previous interventions. These findings prompt the healthcare team to consider more aggressive antimicrobial therapy, including intravenous antibiotics, and to reassess her nutritional management, possibly incorporating enteral feeding options. The team must carefully balance these interventions with close monitoring of her respiratory status to prevent further deterioration, aiming to stabilize her condition and prevent additional complications.

Section 4

Following the administration of intravenous antibiotics and nutritional support adjustments, the patient initially shows signs of stabilization; however, within 48 hours, her condition takes an unexpected turn. Her oxygen saturation remains critically low at 85% despite supplemental oxygen, and her respiratory distress worsens, with a respiratory rate now climbing to 32 breaths per minute. Repeat auscultation reveals more pronounced crackles and wheezing throughout both lung fields, indicating significant airway obstruction and possible mucus plugging, common in cystic fibrosis exacerbations. The patient's heart rate has increased to 115 beats per minute, reflecting her body's compensatory response to hypoxemia and stress.

New diagnostic results from the CT scan indicate multiple areas of atelectasis, suggestive of mucus plugging, rather than a pulmonary embolism. These findings are consistent with cystic fibrosis-related complications, necessitating an immediate adjustment in her treatment plan. The blood cultures have returned positive for Pseudomonas aeruginosa, a common pathogen in cystic fibrosis exacerbations, confirming that the current antibiotic regimen may need further adjustment to effectively target this resistant organism.

In response to these developments, the healthcare team initiates more targeted antibiotic therapy and increases the intensity of her airway clearance techniques, including chest physiotherapy and nebulized hypertonic saline. A high-calorie, high-protein enteral nutrition plan is also implemented to address her persistent malnutrition and support her increased metabolic demands. Close monitoring of her respiratory status continues, with plans to escalate to non-invasive ventilation if her oxygenation fails to improve. These interventions are critical in preventing further respiratory decline and stabilizing her clinical status, setting the stage for potential recovery while remaining vigilant for any emerging complications.

Section 5

As the healthcare team focuses on the response to the intensified interventions, they closely monitor the patient's clinical progress. Within 24 hours of implementing the adjusted antibiotic regimen and enhanced airway clearance techniques, there are some encouraging signs. The patient's respiratory rate has decreased slightly to 28 breaths per minute, and her oxygen saturation has improved to 88% with the aid of supplemental oxygen. However, she still requires frequent nebulized treatments and chest physiotherapy sessions, as her lung auscultation continues to reveal diffuse crackles and occasional wheezes.

Laboratory tests indicate a slight decrease in inflammatory markers, such as C-reactive protein (CRP), suggesting a partial response to the targeted antibiotics against Pseudomonas aeruginosa. The patient's white blood cell count has stabilized, remaining within the upper limit of normal, which supports the notion that the infection is starting to come under control. Despite these positive trends, the patient's nutritional status remains precarious. Her albumin levels are low, reflecting ongoing protein depletion, and her weight has not yet shown significant improvement. These findings underscore the need for continued nutritional support and careful monitoring of her caloric intake and metabolic needs.

While the interventions have led to some stabilization, the healthcare team remains vigilant for any new complications or setbacks. Given the patient's complex condition and the nature of cystic fibrosis, they are prepared to escalate care as necessary. The next steps include reassessing the effectiveness of the current treatment plan, considering the potential need for non-invasive ventilation, and ensuring that the patient's nutritional goals are met to support her recovery. The team prepares to address any emerging issues promptly, maintaining a comprehensive approach to her care that is both proactive and adaptable to changing clinical circumstances.