A 17-year-old female with cystic fibrosis is admitted to the pediatric intensive care unit with significant respiratory distress, fever, hypotension, and tachycardia. Her laboratory results show severe hypoxemia, respiratory acidosis, and elevated white blood cell count. What is the nurse's priority intervention to address the immediate respiratory concerns for this patient?

B) Administer supplemental oxygen to improve oxygen saturation levels.

A) Initiate broad-spectrum intravenous antibiotics to target potential Pseudomonas aeruginosa infection.

B) Administer supplemental oxygen to improve oxygen saturation levels.

C) Begin aggressive airway clearance techniques to manage mucus plugging.

D) Prepare for potential intubation and mechanical ventilation due to respiratory failure.

A 17-year-old female with cystic fibrosis is admitted to the pediatric intensive care unit with severe respiratory distress and signs of systemic infection. Given the initial assessment findings, which of the following interventions should the nurse prioritize first to stabilize the patient?

B) Initiate supplemental oxygen therapy to improve the patient's severe hypoxemia.

A) Administer broad-spectrum intravenous antibiotics to address the suspected Pseudomonas aeruginosa infection.

B) Initiate supplemental oxygen therapy to improve the patient's severe hypoxemia.

C) Begin aggressive airway clearance techniques to manage her productive cough and mucus plugging.

D) Consult endocrinology to manage her erratic blood glucose levels due to pancreatic insufficiency.

A patient with multi-drug resistant Pseudomonas aeruginosa infection is exhibiting signs of septic shock and impending respiratory failure. Despite initial interventions, her condition is deteriorating, as indicated by worsening respiratory acidosis and hypotension. Which of the following nursing interventions is the highest priority to stabilize the patient's condition?

C) Initiate norepinephrine infusion to improve blood pressure and maintain perfusion.

A) Increase the rate of IV fluid resuscitation to address hypotension.

B) Administer the adjusted antibiotic regimen promptly to control the infection.

C) Initiate norepinephrine infusion to improve blood pressure and maintain perfusion.

D) Escalate to invasive mechanical ventilation to manage respiratory failure.

A patient with confirmed multi-drug resistant Pseudomonas aeruginosa infection and signs of septic shock is receiving non-invasive positive pressure ventilation and norepinephrine support. Despite these interventions, the patient's respiratory acidosis is worsening. As the nurse, what is the next most appropriate immediate action to address the patient's deteriorating respiratory status?

B) Initiate invasive mechanical ventilation to provide better respiratory support.

A) Increase the rate of intravenous fluid administration to improve hemodynamics.

B) Initiate invasive mechanical ventilation to provide better respiratory support.

C) Administer a bronchodilator to reduce airway resistance and improve ventilation.

D) Request an urgent re-assessment by the respiratory therapist to adjust ventilator settings.

A patient with severe cystic fibrosis exacerbation and septic shock is experiencing acute confusion and disorientation. Given her critical condition, which intervention should the nurse prioritize to address the potential underlying cause of her altered mental status?

B) Initiate continuous renal replacement therapy to manage fluid overload and acute kidney injury.

A) Increase the dose of norepinephrine to stabilize blood pressure further.

B) Initiate continuous renal replacement therapy to manage fluid overload and acute kidney injury.

C) Administer a bolus of normal saline to improve intravascular volume.

D) Prepare the patient for an immediate CT scan of the brain to assess for intracranial pathology.

A patient with cystic fibrosis has been admitted with a severe exacerbation complicated by multidrug-resistant infection and septic shock. Despite non-invasive positive pressure ventilation and vasopressor support with norepinephrine, the patient's condition continues to deteriorate, evidenced by labile blood pressure, elevated heart rate, decreased urine output, elevated lactate, and acute confusion. Which of the following nursing interventions is the most critical to prioritize to address the underlying pathophysiology of this patient's condition?

A) Initiate renal replacement therapy to manage fluid overload and acute kidney injury.

B) Increase the dose of norepinephrine to maintain adequate blood pressure and perfusion.

C) Administer a high-calorie, high-protein diet to support metabolic demands.

D) Implement continuous neurological assessments to monitor for septic encephalopathy.

A patient with severe cystic fibrosis exacerbation is receiving norepinephrine and fluid resuscitation but continues to have fluctuating blood pressure and signs of poor perfusion. Despite normal CT findings, the patient exhibits confusion and fluctuating levels of consciousness. Which nursing intervention is most critical to address her current condition?

B) Prepare to administer vasopressin as an adjunctive therapy to norepinephrine.

A) Increase the norepinephrine infusion rate to stabilize blood pressure.

B) Prepare to administer vasopressin as an adjunctive therapy to norepinephrine.

C) Initiate high-flow oxygen therapy to improve cerebral perfusion.

D) Perform a neurological assessment to evaluate the need for seizure prophylaxis.

A patient with a severe cystic fibrosis exacerbation is experiencing circulatory collapse despite norepinephrine and fluid resuscitation. The healthcare team is considering the addition of vasopressin. Concurrently, the patient shows signs of septic encephalopathy and has been started on continuous renal replacement therapy (CRRT) due to deteriorating renal function. As the nurse caring for this patient, which intervention should be prioritized to address the patient's hemodynamic instability and neurological status?

B) Administer vasopressin and monitor for improvements in hemodynamic status.

A) Increase the norepinephrine dosage to improve blood pressure stabilization.

B) Administer vasopressin and monitor for improvements in hemodynamic status.

C) Initiate a bolus of hypertonic saline to correct potential electrolyte imbalances.

D) Consult with the neurology team for immediate EEG results to assess seizure activity.

A 25-year-old female with cystic fibrosis and suspected gastrointestinal bleeding presents with worsening respiratory status despite high-flow nasal cannula oxygen therapy. Her arterial blood gas shows a pH of 7.28, PaCO2 of 52 mmHg, and PaO2 of 58 mmHg. Her hemoglobin level has dropped to 7.5 g/dL. Given these findings, what is the most appropriate immediate nursing intervention?

A) Prepare for endotracheal intubation to address her respiratory failure.

B) Administer a blood transfusion to address her acute anemia.

C) Initiate non-invasive ventilation to improve her oxygenation.

D) Consult with gastroenterology to prioritize the endoscopic procedure.

A patient with cystic fibrosis is experiencing worsening hypoxemia and acidosis, with arterial blood gas results indicating a pH of 7.28, PaCO2 of 52 mmHg, and PaO2 of 58 mmHg. Despite high-flow nasal cannula oxygen therapy, her oxygen saturation remains at 85%. Considering the patient's fragile hemodynamic status and potential for destabilization, what is the most appropriate immediate nursing intervention to optimize her respiratory status while preparing for potential escalation of care?

C) Prepare for non-invasive ventilation to provide ventilatory support and improve gas exchange.

A) Increase the flow rate on the high-flow nasal cannula to maximize oxygen delivery.

B) Administer a bronchodilator to address potential airway obstruction and improve gas exchange.

C) Prepare for non-invasive ventilation to provide ventilatory support and improve gas exchange.

D) Initiate immediate intubation and mechanical ventilation to correct the acidosis and hypoxemia.

cystic fibrosis - Nursing Case Study

Pathophysiology

• Primary mechanism: Cystic fibrosis is caused by mutations in the CFTR gene, leading to dysfunctional CFTR protein channels. These channels fail to transport chloride ions across epithelial cell membranes, resulting in thick, viscous mucus.

• Secondary mechanism: The buildup of thick mucus obstructs airways and pancreatic ducts, promoting chronic lung infections and impairing digestive enzyme release, which reduces nutrient absorption.

• Key complication: Persistent lung infections due to mucus retention create a cycle of inflammation and tissue damage, eventually leading to respiratory failure, the most severe and life-threatening complication.

Patient Profile

Demographics:

17-year-old female, high school student

History:

• Key past medical history: Diagnosed with cystic fibrosis at age 2, history of recurrent pulmonary infections, pancreatic insufficiency, CFRD (Cystic Fibrosis-Related Diabetes), nasal polyps, and liver disease.

• Current medications: Ivacaftor, pancreatic enzyme supplements, insulin, azithromycin, high-dose ibuprofen, inhaled tobramycin, dornase alfa, and vitamin supplements (A, D, E, K).

• Allergies: Penicillin (causes rash and hives).

Current Presentation:

• Chief complaint: Severe shortness of breath and productive cough with greenish sputum.

• Key symptoms: Respiratory distress, persistent cough, weight loss, fatigue, abdominal pain, and frequent greasy stools.

• Vital signs: Temperature 39.2°C (102.6°F), heart rate 132 bpm, respiratory rate 36 breaths per minute, blood pressure 88/54 mmHg, oxygen saturation 82% on room air.

Section 1

Initial Assessment Findings:

Upon admission to the pediatric intensive care unit, the initial assessment of the 17-year-old female reveals significant respiratory distress. Auscultation of the lungs indicates diffuse crackles and wheezing bilaterally, with diminished breath sounds at the bases, suggesting possible atelectasis or mucus plugging. Her productive cough is persistent, with copious amounts of thick, greenish sputum, raising concerns for a potential Pseudomonas aeruginosa infection, a common pathogen in cystic fibrosis patients. The patient is tachypneic with a respiratory rate of 36 breaths per minute and is using accessory muscles to breathe, indicating increased work of breathing. She is febrile with a temperature of 39.2°C (102.6°F) and exhibits signs of systemic infection or sepsis, evidenced by hypotension (blood pressure 88/54 mmHg) and tachycardia (heart rate 132 bpm).

Laboratory results reveal a white blood cell count of 18,000/mm³, with a left shift indicating an acute bacterial infection. Arterial blood gas analysis shows severe hypoxemia with a PaO2 of 52 mmHg and respiratory acidosis with a pH of 7.28, pointing to compromised gas exchange and possible respiratory failure. Her sputum culture is pending, but the clinical team anticipates multi-drug resistant organisms given her history. Furthermore, pancreatic insufficiency is exacerbated, evident by frequent, greasy stools and weight loss, and her blood glucose levels are erratic, with episodes of hyperglycemia and hypoglycemia complicating her CFRD management. Electrolyte imbalances are noted with hypochloremia and hyponatremia, likely due to excessive sodium loss through sweat and malabsorption.

These findings necessitate urgent interventions, including supplemental oxygen to maintain adequate oxygen saturation, initiation of broad-spectrum intravenous antibiotics tailored to cystic fibrosis pathogens, and aggressive airway clearance techniques. A multidisciplinary approach is vital, involving pulmonology, endocrinology, and nutrition, to address her complex needs. The clinical team must anticipate potential complications such as respiratory failure, septic shock, and exacerbation of her pancreatic insufficiency, requiring advanced critical thinking and prompt, evidence-based decision-making to stabilize her condition.

Section 2

Following the initial interventions, the patient was placed on high-flow nasal cannula oxygen therapy, which improved her oxygen saturation to 91%. Despite this, her respiratory distress persisted, highlighting the severity of her pulmonary involvement. The team initiated a regimen of intravenous antibiotics, including ceftazidime and tobramycin, to target suspected Pseudomonas aeruginosa infection. To further alleviate mucus obstruction, frequent chest physiotherapy and nebulized hypertonic saline treatments were started. However, within 12 hours, her clinical status began to deteriorate.

New diagnostic results from a follow-up arterial blood gas analysis indicated worsening respiratory acidosis with a pH of 7.24 and a PaCO2 of 58 mmHg, suggesting impending respiratory failure. Concurrently, the sputum culture confirmed the presence of a multi-drug resistant Pseudomonas strain, necessitating a reassessment of the antibiotic regimen. Her blood pressure remained low at 85/50 mmHg, despite fluid resuscitation, and her heart rate increased to 140 bpm, raising concerns for septic shock.

These developments prompted the clinical team to escalate respiratory support to non-invasive positive pressure ventilation to decrease the work of breathing and improve alveolar ventilation. The antimicrobial therapy was adjusted to include colistin, based on sensitivity results. Given the signs of septic shock, vasopressor support with norepinephrine was initiated to maintain adequate perfusion. The patient's erratic blood glucose levels required intensive monitoring and sliding scale insulin therapy adjustments, managed by the endocrinology team. These changes underscore the need for dynamic and coordinated care to address her complex and evolving clinical picture.

Section 3

Following the escalation to non-invasive positive pressure ventilation and the adjustment in antimicrobial therapy, the patient's initial response showed a slight improvement in her work of breathing and oxygenation, with oxygen saturation rising to 93%. However, subsequent assessment revealed new complications indicative of further clinical deterioration. Despite the addition of norepinephrine, her blood pressure remained labile, fluctuating between 80/45 mmHg and 90/55 mmHg, with a persistently elevated heart rate of 145 bpm. These findings, alongside a drop in urine output to less than 20 mL/hour, suggested worsening septic shock with possible acute kidney injury.

Further laboratory investigations revealed a significant elevation in serum creatinine to 2.3 mg/dL from a baseline of 0.9 mg/dL, alongside an increase in blood urea nitrogen to 42 mg/dL. Additionally, her lactate level rose to 4.8 mmol/L, corroborating the suspicion of inadequate tissue perfusion. These findings necessitated the initiation of renal replacement therapy planning, while continuing to titrate vasopressor support to stabilize hemodynamics. The multidisciplinary team, including nephrology, was brought in to evaluate the potential need for continuous renal replacement therapy (CRRT) to manage her acute renal impairment and fluid overload.

As the clinical team worked to address these critical issues, the patient's mental status showed signs of acute confusion and disorientation, raising concerns for possible cerebral hypoperfusion or septic encephalopathy. A CT scan of the brain was ordered to rule out potential intracranial pathology. This development highlighted the complexity of her condition and the critical need for ongoing reassessment and adaptation of the management plan to address the multifaceted challenges posed by her severe cystic fibrosis exacerbation complicated by multidrug-resistant infection and systemic shock.

Section 4

As the critical care team continued to monitor the patient closely, her condition persisted in its complexity. Despite the administration of norepinephrine and attempts to optimize fluid resuscitation, her hemodynamic status remained precarious. The patient's blood pressure continued to fluctuate, now occasionally dipping to as low as 75/40 mmHg, with her heart rate escalating to 155 bpm. Her skin appeared mottled and cool to the touch, indicative of worsening peripheral perfusion. These signs suggested the need for escalation in vasopressor therapy, and the team considered the addition of vasopressin to better support her circulatory collapse.

Concurrently, the CT scan of the brain returned with no evidence of acute intracranial pathology, ruling out any significant hemorrhagic or ischemic events. However, her ongoing confusion and fluctuating levels of consciousness were concerning. Neurology was consulted to evaluate for septic encephalopathy and to consider the potential contributions of electrolyte imbalances or metabolic derangements. The team also initiated continuous EEG monitoring to assess for subclinical seizure activity, given her neurological status.

In light of the persistent fluid overload and deteriorating renal function, the nephrology team proceeded with the initiation of continuous renal replacement therapy (CRRT). This decision was made to address her rising creatinine, now at 2.8 mg/dL, and to manage her fluid balance more effectively. The initiation of CRRT aimed to stabilize her metabolic status and potentially improve her mental status by mitigating uremic toxins. The complexity of her case underscored the need for ongoing multidisciplinary collaboration and vigilant monitoring as the healthcare team navigated the evolving landscape of her severe cystic fibrosis exacerbation and its associated complications.

Section 5

As the critical care team continued their efforts to stabilize the patient, a new set of diagnostic results brought additional challenges to light. The laboratory tests indicated a significant drop in her hemoglobin levels to 7.5 g/dL, likely due to ongoing gastrointestinal bleeding that had been initially suspected but not confirmed. This new finding necessitated immediate intervention to prevent further hemodynamic instability. The team promptly initiated a type and crossmatch for packed red blood cells, planning for a transfusion to address the acute anemia. Simultaneously, gastroenterology was consulted to perform an emergent endoscopy, aiming to identify and manage the source of the bleeding.

Meanwhile, her respiratory status raised further concerns. Despite being on high-flow nasal cannula oxygen therapy, her oxygen saturation levels had decreased to 85%, indicating worsening hypoxemia. Arterial blood gas analysis revealed a pH of 7.28, PaCO2 of 52 mmHg, and PaO2 of 58 mmHg, confirming a mixed respiratory and metabolic acidosis. The critical care team deliberated on escalating her respiratory support to non-invasive ventilation or even intubation, given her deteriorating pulmonary function. This decision was complicated by her fragile hemodynamic status and the potential for further destabilization with sedative administration.

The unfolding complexities demanded heightened vigilance and advanced clinical reasoning from the team. The need to balance aggressive resuscitation efforts with careful management of her cystic fibrosis-related complications, such as thickened secretions and impaired gas exchange, was paramount. As they prepared for potential escalation of care, the team remained acutely aware of the delicate equilibrium required to navigate her multifaceted condition, emphasizing the critical importance of multidisciplinary collaboration in optimizing her outcomes.