G6PD - Nursing Case Study
Pathophysiology
• Primary mechanism: Glucose-6-phosphate dehydrogenase (G6PD) deficiency impairs the hexose monophosphate pathway, reducing NADPH production. This deficiency compromises the ability of red blood cells (RBCs) to regenerate reduced glutathione, crucial for detoxifying reactive oxygen species (ROS).
• Secondary mechanism: Accumulation of ROS leads to oxidative damage of RBCs, causing hemoglobin denaturation and precipitating Heinz bodies formation. Damaged RBCs are sequestered and destroyed in the spleen, resulting in hemolytic anemia.
• Key complication: Hemolytic anemia can lead to severe jaundice and acute kidney injury due to the excessive release of bilirubin and hemoglobin breakdown products, necessitating prompt medical intervention to prevent life-threatening outcomes.
Patient Profile
Demographics:
45-year-old male, construction worker
History:
• Key past medical history: Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency, chronic hemolytic anemia, recurrent infections
• Current medications: Folic acid supplements, regular blood transfusions
• Allergies: Sulfa drugs, fava beans
Current Presentation:
• Chief complaint: Severe fatigue and jaundice
• Key symptoms: Dark urine, pallor, shortness of breath, abdominal pain
• Vital signs: Blood pressure 90/60 mmHg, heart rate 130 bpm, respiratory rate 28 breaths/min, temperature 101.3°F, oxygen saturation 89% on room air
Section 1
Change in Patient Status:
As the medical team initiated treatment, including oxygen supplementation and intravenous fluids to address the patient's hypovolemia and hypoxia, his condition showed only minimal improvement. Within a few hours, the patient's oxygen saturation remained critically low at 85% despite high-flow oxygen therapy, indicating a possible worsening of his respiratory status. Additionally, the patient's heart rate increased to 145 bpm, and his blood pressure dropped further to 85/55 mmHg, signaling hemodynamic instability. The patient also began exhibiting signs of confusion and restlessness, suggesting cerebral hypoxia or possible central nervous system involvement due to severe anemia and hypovolemia.
Close monitoring revealed significant changes in laboratory values. Hemoglobin levels dropped sharply to 6.5 g/dL, and a complete blood count indicated an elevated reticulocyte count, characteristic of a compensatory response to hemolytic anemia. Serum lactate dehydrogenase (LDH) and indirect bilirubin levels were markedly elevated, consistent with ongoing hemolysis. Additionally, renal function tests showed a rising creatinine level, now at 2.4 mg/dL, confirming the development of acute kidney injury. These findings necessitated urgent blood transfusions and consideration of renal support therapies to prevent further deterioration.
The rapid progression of symptoms and the emergence of acute kidney injury prompted the healthcare team to reassess the therapeutic approach. It was critical to determine whether additional factors, such as an undiagnosed infection or a hemolytic crisis trigger, were contributing to the patient's deteriorating condition. The interdisciplinary team prepared to initiate broad-spectrum antibiotics while awaiting further diagnostic results, including blood cultures and imaging studies, to rule out infectious causes and assess the extent of splenic involvement or other potential sources of acute hemolysis. This change in status required advanced clinical reasoning to prioritize interventions that could stabilize the patient and prevent multi-organ failure.
Section 2
As the interdisciplinary team continued to manage the patient's care, new diagnostic results arrived, providing additional insights into the complexity of his condition. Blood cultures returned positive for Escherichia coli, indicating a concurrent septicemia likely exacerbating the patient's hemodynamic instability and contributing to his severe anemia. Imaging studies, including an abdominal ultrasound, revealed splenomegaly with areas suggestive of infarction, a potential trigger for the hemolytic crisis. This finding correlated with the elevated LDH and indirect bilirubin levels, confirming the ongoing destruction of red blood cells.
The presence of sepsis necessitated an immediate adjustment of the treatment plan. The healthcare team prioritized the initiation of broad-spectrum antibiotics, specifically targeting gram-negative organisms, while maintaining rigorous supportive care. Despite these efforts, the patient's condition continued to decline, with urine output decreasing to less than 0.5 mL/kg/hr, further evidence of worsening acute kidney injury. The creatinine level climbed to 3.2 mg/dL, pushing the team to consider renal replacement therapies to support the failing kidneys.
This cascade of complications demanded advanced clinical reasoning to balance the need for aggressive intervention against the risk of exacerbating the patient's fragile state. The medical team debated the timing and intensity of renal support, weighing the risks of fluid overload against the necessity of correcting electrolyte imbalances and removing toxins. As the patient's oxygen saturation stubbornly hovered around 82%, discussions also centered on the potential need for mechanical ventilation to ensure adequate oxygenation and reduce cerebral hypoxia. The critical nature of these decisions underscored the complexity of the case, requiring a meticulous approach to prevent the progression to multi-organ failure.
Section 3
As the interdisciplinary team continued to navigate this intricate clinical scenario, the patient's status took another concerning turn, signaling new complications. Overnight, the patient developed acute respiratory distress, with a respiratory rate climbing to 35 breaths per minute and an oxygen saturation dropping further to 78% on high-flow oxygen. Auscultation of the lungs revealed diffuse crackles, raising alarms for potential acute respiratory distress syndrome (ARDS) secondary to sepsis. A stat chest X-ray was ordered, which showed bilateral pulmonary infiltrates, confirming suspicions of developing ARDS. This respiratory compromise compounded the existing hemodynamic instability, complicating the management of his oxygenation and ventilation needs.
In parallel, laboratory results indicated a further drop in hemoglobin to 6.5 g/dL, necessitating urgent blood transfusions to address the ongoing hemolytic anemia. However, the patient's glucose-6-phosphate dehydrogenase (G6PD) deficiency posed a challenge in selecting appropriate blood products and transfusion protocols to minimize hemolysis risk. Additionally, the elevated lactate level of 4.5 mmol/L suggested worsening tissue hypoxia and prompted the team to reassess the adequacy of perfusion and cardiovascular support.
Faced with these developments, the healthcare team engaged in intensive clinical reasoning to prioritize interventions. The need for mechanical ventilation became increasingly apparent, yet the risk of barotrauma and further hemodynamic compromise necessitated a carefully titrated approach. The team also deliberated the initiation of continuous renal replacement therapy (CRRT) to manage fluid overload, correct electrolyte disturbances, and support renal function without exacerbating the patient's respiratory status. These decisions, made in a high-stakes environment, underscored the necessity for synchronized interdisciplinary collaboration to prevent progression to multi-organ failure and optimize the patient's chances of recovery.
Section 4
As the interdisciplinary team implemented their carefully crafted interventions, the patient's response offered both challenges and insights into the evolving clinical picture. Mechanical ventilation was initiated using a lung-protective strategy with low tidal volumes to mitigate the risk of barotrauma. Initial settings included a tidal volume of 6 mL/kg of ideal body weight and a positive end-expiratory pressure (PEEP) of 10 cm H2O, with close monitoring of peak and plateau pressures. Despite these efforts, the patient's oxygenation improved only marginally, with oxygen saturation hovering around 82%. Meanwhile, arterial blood gas analysis revealed a pH of 7.30, PaCO2 of 50 mmHg, and a PaO2 of 55 mmHg, indicating a mixed respiratory and metabolic acidosis that required further optimization of ventilatory settings and bicarbonate therapy.
Concurrently, the decision to initiate continuous renal replacement therapy (CRRT) was executed with precision. The therapy was tailored to gently manage fluid overload and correct the patient's metabolic derangements, including hyperkalemia with a potassium level of 5.9 mmol/L and a creatinine level of 2.8 mg/dL. The patient's hemodynamic status remained tenuous, with mean arterial pressures fluctuating between 60-65 mmHg despite vasopressor support with norepinephrine at 0.08 mcg/kg/min. This necessitated discussions about further escalating vasopressor therapy or considering adjunctive agents like vasopressin to optimize perfusion without compromising renal or cardiac function.
Despite these interventions, new complications emerged that required immediate attention. The patient developed a new-onset fever of 39.2°C, raising concerns for a secondary infectious process or an exacerbation of the underlying sepsis. Blood cultures were urgently drawn, and broad-spectrum antibiotics were re-evaluated to ensure adequate coverage. The combination of respiratory distress, hemodynamic instability, and now possible infectious exacerbation highlighted the complexity of this case and the need for ongoing, dynamic clinical reasoning. Each member of the healthcare team remained vigilant, knowing that the next steps would be critical in determining the trajectory of the patient's recovery or decline.
Section 5
As the healthcare team grappled with the complexities of the patient's condition, new diagnostic results provided critical insights that further complicated the clinical picture. A follow-up chest X-ray revealed bilateral infiltrates consistent with acute respiratory distress syndrome (ARDS), but also a small right-sided pleural effusion that had not been previously noted. This finding prompted a re-evaluation of the patient's fluid status and the potential need for thoracentesis, especially given the patient's ongoing hypoxemia. Additionally, a repeat complete blood count showed a declining hemoglobin level of 7.2 g/dL, raising concerns for hemolysis, possibly exacerbated by the patient's underlying G6PD deficiency. These hematological changes necessitated a careful balance between transfusion thresholds and the risk of transfusion-related complications, particularly in the context of potential alloimmunization.
Simultaneously, the results of the blood cultures returned positive for Klebsiella pneumoniae, indicating a multidrug-resistant organism. This finding required immediate modification of the antibiotic regimen to include meropenem, in addition to colistin, to ensure comprehensive antimicrobial coverage. The emergence of this resistant infection highlighted the need for stringent infection control measures and increased the risk of septic shock, given the patient's already compromised hemodynamic status. Despite the ongoing norepinephrine infusion, the patient's mean arterial pressure dropped to 58 mmHg, prompting the addition of vasopressin at 0.03 units/min to stabilize perfusion.
The interdisciplinary team recognized that these new complications demanded advanced critical thinking and clinical judgment to navigate the intersecting challenges of respiratory support, hemodynamic management, and infection control. With each intervention, the team remained acutely aware of the delicate balance required to support the patient's fragile hemodynamic state while avoiding further metabolic derangements or additional organ dysfunction. As the team continued to monitor the patient's response, they prepared for the possibility of further complications, knowing that their ongoing vigilance and adaptability would be crucial in shaping the patient's path forward.