Cystic fibrosis - Nursing Case Study

Pathophysiology

• Primary mechanism: Cystic fibrosis is primarily caused by mutations in the CFTR gene, leading to defective chloride ion channels on epithelial cells. This results in impaired chloride and water transport across cell membranes, causing thick and sticky mucus secretions.

• Secondary mechanism: The accumulation of viscous mucus in the lungs and digestive tract obstructs airways and pancreatic ducts, promoting chronic respiratory infections and malabsorption of nutrients due to pancreatic enzyme insufficiency.

• Key complication: Persistent lung infections and inflammation lead to progressive bronchiectasis and respiratory failure, while gastrointestinal issues can cause malnutrition and growth delays, emphasizing the need for early intervention and comprehensive management.

Patient Profile

Demographics:

28-year-old female, respiratory therapist

History:

• Key past medical history: Diagnosed with cystic fibrosis at age 3, recurrent pulmonary infections, pancreatic insufficiency, mild liver disease

• Current medications: Ivacaftor, pancreatic enzyme supplements, azithromycin, salbutamol inhaler, vitamin D supplements

• Allergies: Penicillin (rash)

Current Presentation:

• Chief complaint: Increased cough and shortness of breath over the past two weeks

• Key symptoms: Persistent cough with thick mucus, fatigue, wheezing, mild chest pain, reduced appetite

• Vital signs: Temperature 100.4°F, heart rate 112 bpm, respiratory rate 24 breaths per minute, blood pressure 110/70 mmHg, oxygen saturation 89% on room air

Section 1

Following the initial assessment, the patient, a 28-year-old female with a history of cystic fibrosis, presented with increased cough and shortness of breath. Her vital signs indicated a low-grade fever, tachycardia, and tachypnea, with oxygen saturation at 89% on room air. Given her history of recurrent pulmonary infections and current symptoms, a high suspicion of an exacerbation of cystic fibrosis-related lung disease was raised. A chest X-ray was performed, revealing bilateral infiltrates and areas of consolidation, suggestive of a potential bacterial pneumonia overlaid on her chronic bronchiectasis.

Further laboratory diagnostics included sputum cultures, which identified growth of Pseudomonas aeruginosa, a common pathogen in cystic fibrosis exacerbations. Blood tests showed leukocytosis with a left shift, indicating an acute bacterial infection. Additionally, the patient’s liver function tests revealed a mild elevation in liver enzymes, which could correlate with her history of mild liver disease but necessitated further monitoring. Arterial blood gas analysis showed a partial pressure of oxygen (PaO2) of 55 mmHg, indicating significant hypoxemia, and a compensated respiratory acidosis with a pH of 7.35 and a PaCO2 of 50 mmHg, reflecting chronic respiratory compromise.

With these findings, the clinical team initiated targeted antibiotic therapy, avoiding penicillin due to her allergy, and opted for a combination of intravenous ceftazidime and tobramycin to address the Pseudomonas infection. The patient was also started on supplemental oxygen therapy to maintain adequate oxygen saturation levels and was encouraged to continue her respiratory therapy regimen to aid in mucus clearance. This management approach aimed to stabilize her respiratory status, treat the acute infection, and prevent further complications, while also necessitating close monitoring for potential side effects from the antibiotics and changes in her liver function.

Section 2

As the treatment progressed over the next 48 hours, the patient initially showed some improvement in her respiratory status, with a slight increase in oxygen saturation levels to 93% while on supplemental oxygen. However, despite the antibiotics, her fever persisted, and she began to experience increased fatigue and malaise. A repeat chest X-ray showed no significant improvement in the areas of consolidation, raising concerns about the effectiveness of the current antibiotic regimen or the potential development of antibiotic resistance, particularly given the known challenges in treating Pseudomonas aeruginosa infections in cystic fibrosis patients.

Concurrently, her liver function tests showed further elevation in liver enzymes, with alanine aminotransferase (ALT) rising to 85 U/L and aspartate aminotransferase (AST) reaching 90 U/L. This trend necessitated consideration of potential hepatotoxicity from the antibiotic therapy or a progression of her underlying liver disease. Additionally, the patient reported mild right upper quadrant abdominal discomfort, which could correlate with her liver status and required further evaluation.

Given these developments, the clinical team faced a critical decision point: whether to adjust the antibiotic regimen to target the persistent infection more effectively while minimizing potential liver damage. Further consultation with a hepatologist was deemed necessary to evaluate the liver function changes and guide safe continuation of therapy. Additionally, the team considered implementing adjunctive therapies such as corticosteroids to address inflammatory components of her condition. These clinical decisions required careful balancing of risks and benefits, with ongoing monitoring for any new complications, particularly signs of sepsis or worsening respiratory failure.

Section 3

New Diagnostic Results:

As the clinical team proceeded with further investigations, they decided to obtain a sputum culture to reassess the microbial landscape and determine the sensitivity profile of the infectious agent. The results confirmed the presence of Pseudomonas aeruginosa, but with an added complexity: the strain showed resistance to the initial antibiotic regimen, necessitating an urgent change in treatment strategy. Based on the susceptibility profile, the team opted to initiate a combination of a higher-dose ceftazidime and tobramycin, both of which demonstrated effectiveness against the resistant strain.

Meanwhile, an abdominal ultrasound was conducted to evaluate the patient's reported right upper quadrant discomfort and the elevated liver enzymes. The imaging revealed mild hepatomegaly with some fatty infiltration, suggesting non-alcoholic fatty liver disease (NAFLD) as a potential underlying condition, exacerbated by the current antibiotic regimen. A hepatologist recommended adjusting the medication plan to include liver-protective measures, such as reducing the dose of potentially hepatotoxic drugs and considering the use of ursodeoxycholic acid to support liver function.

These new diagnostic insights prompted the clinical team to carefully monitor the patient's response to the adjusted antibiotic therapy and liver management plan. The complexity of her condition required ongoing vigilance for any signs of further complications, such as sepsis or acute respiratory distress, while also aiming to preserve her liver health and improve her overall clinical status. The team remained hopeful that the tailored treatment approach would lead to gradual improvements, necessitating close follow-up with repeat imaging and laboratory assessments to track her progress.

Section 4

As the clinical team monitored the patient's condition following the adjusted treatment regimen, they noted an initial improvement in her respiratory symptoms. Her cough had become less frequent, and the sputum production had decreased, suggesting a positive response to the new antibiotic combination. However, during a routine assessment, the patient began to exhibit signs of increased fatigue and mild jaundice, prompting a reevaluation of her liver function. Subsequent lab tests revealed a notable rise in her liver enzyme levels: ALT at 150 U/L and AST at 130 U/L, both significantly above normal ranges, indicating worsening liver function despite the protective measures put in place.

In response to these findings, the clinical team collaborated closely with the hepatologist to further optimize the liver management plan. The decision was made to discontinue any non-essential medications that could contribute to hepatotoxicity and to increase the dose of ursodeoxycholic acid. Additionally, the team considered the possibility of alternative antibiotic therapies with a reduced hepatic impact, carefully weighing the risks and benefits to ensure effective infection control while minimizing liver stress.

Throughout this period, the nursing staff played a crucial role in closely monitoring the patient's vital signs and overall status. They diligently assessed for any additional signs of complications, such as increased abdominal pain or changes in mental status, which could indicate hepatic encephalopathy. The patient's blood pressure remained stable at 120/75 mmHg, and her heart rate was slightly elevated at 95 beats per minute, reflecting her ongoing battle with infection. The team remained vigilant, recognizing the importance of balancing aggressive infection management with the protection of her liver health, thus setting the stage for continued close observation and potential further adjustments in her treatment plan.

Section 5

As the clinical team continued to monitor the patient's condition, they noted a subtle change in her status that warranted further investigation. On the third day following the adjustment in her treatment regimen, the patient began to experience pruritus, which raised concerns about her liver function. Concurrently, her fatigue seemed to worsen, and she reported a vague sense of abdominal discomfort. These symptoms prompted the team to order additional diagnostic tests, including a comprehensive metabolic panel and an ultrasound of the liver to assess for any structural changes. The metabolic panel revealed an elevated bilirubin level of 2.5 mg/dL and a slight increase in alkaline phosphatase to 140 U/L, suggesting a possible obstruction or worsening of hepatic function.

The ultrasound, however, did not show any significant obstructive pathology, ruling out common bile duct stones or acute liver lesions. This information led the clinical team to hypothesize that the ongoing liver dysfunction might be related to cholestasis, potentially linked to her underlying cystic fibrosis. In light of this, the nursing staff worked closely with the medical team to implement additional supportive measures. These included maintaining strict fluid balance to prevent dehydration and providing nutritional support tailored to her current needs, with an emphasis on adequate caloric intake to combat her increasing fatigue.

Despite these challenges, the patient remained engaged in her treatment plan and continued to show resilience. Her respiratory symptoms remained stable, with her oxygen saturation consistently above 94% on room air, indicating effective management of her pulmonary status. The team acknowledged the complexity of her case, recognizing the need for ongoing interdisciplinary collaboration. By maintaining a careful balance between managing her respiratory infection and addressing her liver complications, the team aimed to improve her overall quality of life, preparing for potential future interventions or adjustments in her therapeutic approach.