A patient with Sickle Cell Disease is admitted with signs of acute chest syndrome. Which of the following nursing interventions should be prioritized to prevent further deterioration of the patient's condition?

A) Administering supplemental oxygen to maintain adequate oxygen saturation.

B) Initiating a high-calorie diet to address potential nutritional deficiencies.

C) Providing aggressive IV fluid hydration to prevent dehydration.

D) Encouraging early mobilization to prevent deep vein thrombosis.

A patient with sickle cell disease is experiencing acute chest syndrome. Which of the following interventions should the nurse prioritize to address the patient's oxygenation needs?

A) Initiate high-flow oxygen therapy

B) Position the patient in a high Fowler's position

C) Encourage deep breathing and coughing exercises

D) Administer intravenous fluids to maintain hydration

A patient with sickle cell disease is experiencing deterioration with symptoms of hypoxemia, fever, hypotension, lethargy, and tachycardia. Which nursing intervention should be prioritized to address the patient's suspected septic shock?

B) Provide aggressive fluid resuscitation

A) Administer antipyretics to reduce fever

B) Provide aggressive fluid resuscitation

C) Initiate norepinephrine infusion

A 25-year-old patient with sickle cell disease presents with acute chest syndrome and her condition is deteriorating. The healthcare team suspects septic shock or volume depletion as her blood pressure is 92/58 mmHg and heart rate is 126 bpm. Which nursing intervention should be prioritized based on this assessment?

C) Initiate aggressive fluid resuscitation

A) Administer oxygen to maintain saturation above 92%

B) Prepare for an exchange transfusion

C) Initiate aggressive fluid resuscitation

D) Perform a CT scan of the head

A patient with sickle cell disease is admitted to the ICU with confirmed Klebsiella pneumoniae infection leading to septic shock and mild cerebral edema. Which nursing intervention is the priority to address the patient's current condition?

B) Initiate aggressive fluid resuscitation to manage hypoperfusion.

A) Administer the prescribed antibiotics to target the Gram-negative bacilli.

B) Initiate aggressive fluid resuscitation to manage hypoperfusion.

C) Monitor neurological status for signs of increased intracranial pressure.

D) Prepare for exchange transfusion to reduce sickling crises.

A patient with sickle cell disease is admitted to the ICU with confirmed Klebsiella pneumoniae infection, elevated lactate levels, and mild cerebral edema. Which nursing intervention is most appropriate to prioritize in managing this patient's condition?

B) Initiating aggressive fluid resuscitation to address hypoperfusion and lactic acidosis.

A) Administering prescribed antibiotics to target the Klebsiella pneumoniae infection.

B) Initiating aggressive fluid resuscitation to address hypoperfusion and lactic acidosis.

C) Monitoring neurological status closely and preparing for potential interventions for increased intracranial pressure.

D) Educating the patient and family about the importance of infection prevention and management in sickle cell disease.

A patient with sickle cell disease presents with acute respiratory distress, low blood pressure, elevated heart rate, and bilateral crackles upon lung auscultation. The nursing team plans to initiate an exchange transfusion and administer diuretics. What is the most critical nursing intervention to perform prior to the administration of diuretics in this patient?

B) Assess the patient's electrolyte levels

A) Monitor the patient's urine output closely

B) Assess the patient's electrolyte levels

C) Obtain a baseline blood pressure reading

D) Evaluate the patient's fluid intake history

A patient with sickle cell disease is experiencing hemodynamic instability and acute respiratory distress despite treatment efforts. Which nursing intervention is the priority to address her current condition?

C) Prepare the patient for an immediate exchange transfusion.

A) Administer high-dose corticosteroids to reduce inflammation.

B) Initiate high-flow oxygen therapy to improve oxygen saturation.

C) Prepare the patient for an immediate exchange transfusion.

D) Start intravenous vasopressors to stabilize blood pressure.

A patient with a history of sickle cell disease is experiencing acute chest syndrome, demonstrated by worsening bilateral infiltrates on chest X-ray, and requires non-invasive positive pressure ventilation. Despite treatment interventions, her oxygen saturation remains low at 86-90% on high-flow oxygen. Which nursing intervention should be prioritized to improve the patient's oxygenation and overall status?

B) Initiate prone positioning to enhance oxygenation.

A) Administer a fluid bolus to improve hemodynamic stability.

B) Initiate prone positioning to enhance oxygenation.

C) Increase the rate of the high-flow oxygen therapy.

D) Prepare for intubation and mechanical ventilation.

A patient with acute chest syndrome and acute kidney injury is experiencing worsening respiratory distress despite high-flow oxygen therapy. Which nursing intervention is most appropriate to prioritize at this time?

B) Prepare the patient for non-invasive positive pressure ventilation.

A) Increase the flow rate of the oxygen therapy.

B) Prepare the patient for non-invasive positive pressure ventilation.

C) Administer a bolus of intravenous fluids to improve blood pressure.

D) Start a continuous infusion of diuretics to reduce fluid overload.

Sickle Cell Disease - Nursing Case Study

Pathophysiology

• Primary mechanism: Sickle Cell Disease arises from a mutation in the hemoglobin beta-globin chain, leading to the production of hemoglobin S (HbS). Under low oxygen conditions, HbS polymerizes, causing red blood cells to become rigid and sickle-shaped.

• Secondary mechanism: The sickled red blood cells have a shortened lifespan and can occlude small blood vessels, resulting in impaired blood flow and reduced oxygen delivery to tissues, leading to vaso-occlusive crises and chronic hemolytic anemia.

• Key complication: These processes can cause severe pain episodes, organ damage, and increased risk of infections due to splenic dysfunction, significantly impacting the patient's quality of life and requiring ongoing medical management.

Patient Profile

Demographics:

27-year-old female, high school teacher

History:

• Key past medical history: Diagnosed with Sickle Cell Disease at age 6, history of vaso-occlusive crises, splenic sequestration, and mild pulmonary hypertension

• Current medications: Hydroxyurea, folic acid, acetaminophen as needed for pain, penicillin prophylaxis

• Allergies: Penicillin

Current Presentation:

• Chief complaint: Increasing episodes of chest pain and shortness of breath

• Key symptoms: Fatigue, intermittent joint pain, mild jaundice, swelling in hands and feet

• Vital signs: Temperature 99.1°F, Heart rate 108 bpm, Blood pressure 100/60 mmHg, Respiratory rate 22 breaths per minute, Oxygen saturation 92% on room air

Section 1

New Complications:

During her current hospitalization, the patient began to exhibit signs of acute chest syndrome, a severe complication of Sickle Cell Disease. Her oxygen saturation, which was already low, dropped further to 88% on room air, necessitating supplemental oxygen via nasal cannula. She experienced increased chest pain and her respiratory rate rose to 28 breaths per minute, indicating respiratory distress. A chest X-ray revealed infiltrates in the lower lobes of both lungs, consistent with pneumonia or pulmonary infarction, both common triggers for acute chest syndrome in sickle cell patients.

Laboratory results showed a marked increase in white blood cell count, now at 16,000/mm³, suggesting an inflammatory or infectious process. Her hemoglobin levels had dropped to 7.2 g/dL from her baseline of around 9 g/dL, indicating worsening anemia likely due to increased hemolysis. Reticulocyte count was elevated, consistent with her body's attempt to compensate for the anemia. The patient's increased fatigue and jaundice were in line with these findings, and the presence of crackles on auscultation of her lungs further supported the diagnosis of acute chest syndrome.

Due to the complexity of her condition, the healthcare team initiated aggressive management, including broad-spectrum antibiotics to address potential underlying infection and increased her dosage of hydroxyurea to boost fetal hemoglobin production and improve red blood cell flexibility. Careful monitoring and supportive care were essential, as acute chest syndrome can rapidly progress and become life-threatening. This new complication required the team to reassess her treatment plan and prioritize interventions aimed at stabilizing her condition and preventing further deterioration, with close attention paid to her oxygenation, pain management, and hydration status.

Section 2

Change in Patient Status:

Despite the initial interventions, the patient's condition began to deteriorate further. Her oxygen saturation on supplemental oxygen remained precarious, fluctuating between 90% and 92%, which indicated persistent hypoxemia. The patient developed a fever, with her temperature rising to 101.2°F, pointing towards a possible infectious etiology complicating her acute chest syndrome. Her blood pressure was noted to be decreasing, with readings around 92/58 mmHg, raising concerns about potential septic shock or volume depletion.

Clinically, the patient appeared increasingly lethargic and disoriented, a concerning sign suggesting potential cerebral hypoxia or evolving complications such as a cerebrovascular accident, which sickle cell patients are at risk for. Her heart rate escalated to 126 beats per minute in response to both fever and potential hypovolemia. The healthcare team quickly recognized the need to adjust her management plan, emphasizing the importance of aggressive fluid resuscitation to address potential shock, while continuing the broad-spectrum antibiotics to manage the suspected infection.

In light of her neurological changes and ongoing respiratory distress, a decision was made to escalate her care to the intensive care unit for closer monitoring and potential need for advanced respiratory support. The team considered the possibility of exchange transfusion to rapidly decrease the sickling red cells and improve her oxygen carrying capacity. This change in her status prompted further diagnostic evaluations, including blood cultures, lactate levels, and a CT scan of the head to rule out intracranial pathology, indicative of the multifaceted approach required in managing her complex and evolving condition.

Section 3

New Diagnostic Results:

Upon admission to the intensive care unit, the healthcare team swiftly proceeded with the necessary diagnostic evaluations to elucidate the cause of the patient's deteriorating condition. The blood cultures drawn earlier confirmed the presence of a Gram-negative bacilli, Klebsiella pneumoniae, suggestive of a bacterial infection contributing to her fever and hypotension. This finding prompted a refinement of her antibiotic regimen, intensifying the antimicrobial therapy to specifically target this pathogen. Moreover, the patient's lactate levels were elevated at 4.2 mmol/L, indicating a state of lactic acidosis, likely due to hypoperfusion and inadequate oxygen delivery, aligning with the clinical suspicion of septic shock.

The CT scan of the head revealed no acute intracranial hemorrhage or infarct, providing some relief regarding the immediate risk of cerebrovascular accident. However, the findings indicated mild cerebral edema, which could explain her altered mental status and lethargy. This warranted careful monitoring of her neurological signs and consideration of potential interventions to manage increased intracranial pressure. The need for close observation and potential neuroprotective strategies was emphasized, given the patient's vulnerability to neurological complications due to her sickle cell disease.

These diagnostic results guided the healthcare team's next steps, with a reinforced focus on aggressive fluid resuscitation and continuous reassessment of her hemodynamic status. The decision to proceed with exchange transfusion was bolstered by the need to improve her oxygen delivery and reduce the risk of further sickling crises. The team remained vigilant for any further signs of deterioration, understanding that the patient's journey required a dynamic and multifaceted approach to address the complex interplay of infection, hypoxia, and her underlying sickle cell pathology.

Section 4

As the healthcare team continued to manage the patient's condition, they observed a notable change in her status. Despite the intensified antibiotic regimen and aggressive fluid resuscitation, her blood pressure remained persistently low, and her heart rate increased to 130 beats per minute, indicating ongoing hemodynamic instability. The patient also began to exhibit signs of acute respiratory distress, with her oxygen saturation levels dropping to 88% on high-flow oxygen. Auscultation of her lungs revealed crackles bilaterally, raising concerns for potential acute chest syndrome, a known complication in individuals with sickle cell disease.

Lab results showed a drop in hemoglobin levels to 6.5 g/dL, suggesting increased hemolysis or bleeding, both of which could exacerbate her current state. Additionally, her platelet count had decreased to 90,000/mm³, possibly indicative of disseminated intravascular coagulation (DIC), a complication associated with sepsis and sickle cell crises. The patient's renal function was deteriorating as well, evidenced by a rise in her creatinine to 2.1 mg/dL, signaling acute kidney injury likely due to ongoing hypoperfusion and tissue damage.

The team recognized the urgent need to address these emerging complications. An immediate exchange transfusion was prioritized to reduce sickling, improve oxygen carrying capacity, and stabilize her hemoglobin levels. Concurrently, diuretics were cautiously introduced to manage the pulmonary edema, while ensuring adequate hydration to support renal function. The multidisciplinary team, including a hematologist and a nephrologist, convened to discuss further strategies, emphasizing the importance of comprehensive monitoring and timely interventions to navigate this critical phase of her treatment.

Section 5

Despite the comprehensive management plan, the patient's condition continued to challenge the healthcare team. In the hours following the initial interventions, a change in her status became evident. Her heart rate, which had been persistently elevated, began to stabilize slightly, decreasing to 110 beats per minute. This was a promising sign that the exchange transfusion was helping to reduce the sickling of red blood cells and improve her hemodynamic status. Her blood pressure, however, remained on the lower side at 90/60 mmHg, necessitating close monitoring for signs of shock or further deterioration.

New diagnostic results revealed mixed progress. The exchange transfusion had successfully increased her hemoglobin levels to 8.2 g/dL, providing some improvement in oxygen-carrying capacity. However, her oxygen saturation continued to oscillate between 86% and 90% despite high-flow oxygen therapy, indicating persistent respiratory distress. A repeat chest X-ray showed worsening bilateral infiltrates, confirming the progression of acute chest syndrome. Additionally, her renal function showed no improvement, with creatinine levels remaining elevated at 2.3 mg/dL, suggesting ongoing acute kidney injury despite careful fluid management.

The team noted new complications as well. The patient's platelet count had further decreased to 75,000/mm³, heightening concerns for DIC. This necessitated the initiation of low-dose heparin therapy to address potential microthrombi formation, while balancing the risk of bleeding. The patient's respiratory status was deteriorating, leading to the decision to escalate care with non-invasive positive pressure ventilation to improve oxygenation and reduce the work of breathing. As the team continued to navigate these challenges, they remained vigilant, adjusting the treatment plan as needed to prevent further complications and promote recovery.