# A 35-year-old African American client with sickle cell anemia is admitted to the emergency department with severe chest pain, shortness of breath, and a temperature of 101.8°F (38.8°C). The client reports the pain started suddenly 2 hours ago and rates it as 9/10. Which nursing intervention should be the priority?

> source: MyMerci (mymerci.kr)  
> url: https://mymerci.kr/pages/nclex_q.php?qn_id=540792  
> language: ko  
> subject: Adult Health

## 문제

A 35-year-old African American client with sickle cell anemia is admitted to the emergency department with severe chest pain, shortness of breath, and a temperature of 101.8°F (38.8°C). The client reports the pain started suddenly 2 hours ago and rates it as 9/10. Which nursing intervention should be the priority?

The client appears anxious and is using accessory muscles to breathe. Oxygen saturation is 88% on room air, blood pressure is 90/60 mmHg, and heart rate is 120 bpm. The client has a history of multiple hospitalizations for sickle cell crises.

## 보기

1. Administer prescribed antibiotics immediately to treat infection
2. Prepare the client for emergency blood transfusion to increase oxygen capacity
3. Initiate high-flow oxygen therapy and establish IV access for aggressive fluid resuscitation **✔ 정답**
4. Position the client in Trendelenburg position to improve circulation and venous return

**정답: 3**

## 해설

Priority is high-flow oxygen and IV fluids to address hypoxemia (O2 saturation 88%) and hypotension, preventing further sickling. Antibiotics or transfusion may be needed later but are not immediate priorities.

## 심화 해설

Clinical Priority Analysis

The client presents with acute chest syndrome (ACS), a life-threatening complication of sickle cell disease characterized by a new pulmonary infiltrate, fever, and respiratory symptoms. The immediate threat is hypoxemia, as evidenced by an oxygen saturation of 88% on room air, tachypnea with accessory muscle use, and severe chest pain. The concurrent hypotension (90/60 mmHg) and tachycardia (120 bpm) suggest hemodynamic instability, likely due to a combination of hypoxemia-induced cardiac strain, potential sequestration, and the systemic inflammatory response triggered by the vaso-occlusive crisis .

Rationale for Priority Intervention

The priority nursing intervention is to initiate high-flow oxygen therapy and establish IV access for aggressive fluid resuscitation. In ACS, pulmonary vascular sickling and microvascular occlusion lead to ventilation-perfusion mismatch and intrapulmonary shunting, rapidly progressing to life-threatening hypoxemia. Oxygen delivery is critical to reverse tissue hypoxia, reduce further hemoglobin S polymerization, and prevent a cascade of worsening sickling. Simultaneous, aggressive IV fluid administration is essential because dehydration and hemoconcentration exacerbate sickling by increasing blood viscosity and slowing microvascular flow. Establishing IV access also provides a route for subsequent analgesic and antibiotic administration. The overarching principle in trauma and acute care is the immediate stabilization of airway, breathing, and circulation, which aligns directly with this intervention .

Analysis of Alternative Options

- Option 1 (Administer prescribed antibiotics immediately): While infection is a common trigger for ACS and antibiotic administration is a key component of treatment, it is not the immediate priority over correcting life-threatening hypoxemia and hypovolemia. The client’s immediate survival depends on oxygenation and circulatory support first .

- Option 2 (Prepare for emergency blood transfusion): Transfusion therapy, such as simple or exchange transfusion, is a definitive treatment for ACS to increase oxygen-carrying capacity and reduce the percentage of hemoglobin S. However, it is a secondary intervention that requires preparation, including type and crossmatch, and is initiated after initial stabilization with oxygen and fluids. The clinical guidelines emphasize that transfusion strategies are applied based on the individual case context and severity, not as the first-line emergency response .

- Option 4 (Position in Trendelenburg position): This is contraindicated. The Trendelenburg position would further compromise respiratory function by pushing abdominal contents against the diaphragm, worsening the work of breathing in a client already using accessory muscles. It does not address the underlying pathophysiology of hypoxemia or vaso-occlusion and may dangerously increase preload in a heart already stressed by tachycardia and potential myocardial injury from microvascular occlusion .

Pathophysiological Integration

The client’s presentation illustrates a severe systemic inflammatory response to microvascular occlusion. The sudden chest pain and fever reflect pulmonary infarction and cytokine release. The cardiovascular strain, with hypotension and reflex tachycardia, may involve a component of acute myocardial stress. In sickle cell disease, recurrent microinfarctions and chronic inflammation can alter the myocardial substrate, making the heart more susceptible to injury during an acute crisis, a concept explored in frameworks for myocardial injury management that consider etiology-specific confounders . While not a classic allergic coronary syndrome, the principle of a systemic hypersensitivity-like reaction causing cardiovascular compromise in a susceptible host underscores why rapid, multi-system stabilization—oxygenation and volume resuscitation—is the non-negotiable first step .

## 임상 시나리오

Clinical Management of Acute Chest Syndrome (ACS) in Sickle Cell Disease

Acute chest syndrome is a leading cause of death in sickle cell disease. Immediate recognition and aggressive intervention are critical. The priority is simultaneous respiratory and hemodynamic support.

Immediate Nursing Priorities

- **Oxygenation:** Apply high-flow oxygen via non-rebreather mask to target SpO2 >95%. Hypoxemia promotes hemoglobin S polymerization, worsening the crisis.

- **Fluid Resuscitation:** Establish large-bore IV access and initiate isotonic crystalloid boluses (e.g., 0.9% Normal Saline). Aggressive hydration reduces blood viscosity and slows sickling.

- **Analgesia:** Administer prescribed opioids (e.g., morphine, hydromorphone) intravenously for severe pain. Avoid IM injections due to erratic absorption and bleeding risk.

- **Antibiotics:** Administer broad-spectrum antibiotics promptly after cultures are drawn, as infection is a common trigger.

Ongoing Monitoring and Interventions

- Continuously monitor SpO2, respiratory rate, and work of breathing. Be prepared for non-invasive or mechanical ventilation if respiratory failure develops.

- Monitor hemodynamic status closely (BP, HR, urine output) to guide fluid therapy and detect shock.

- Prepare for red blood cell transfusion or exchange transfusion if hypoxemia or anemia is severe and unresponsive to initial measures.

- Avoid Trendelenburg position; maintain semi-Fowler's or High Fowler's to optimize lung expansion.

Key Clinical Pearls

- ACS can progress rapidly from mild symptoms to respiratory failure within hours.

- Fever, chest pain, and a new pulmonary infiltrate on CXR confirm the diagnosis, but treatment should not be delayed for imaging.

- A drop in hemoglobin and platelet count alongside rising LDH can signal severe ACS or multiorgan failure.

## 핵심 개념

- **Acute Chest Syndrome (ACS)** — A life-threatening complication of sickle cell disease involving pulmonary infiltrates, fever, and respiratory symptoms, often triggered by infection or vaso-occlusion.
- **Vaso-occlusive Crisis** — A painful episode in sickle cell disease caused by sickled red blood cells obstructing microvasculature, leading to tissue ischemia and inflammation.
- **Hemoglobin S Polymerization** — The process where deoxygenated sickle hemoglobin molecules aggregate into rigid polymers, distorting red blood cells into a sickle shape.
- **Ventilation-Perfusion Mismatch** — An imbalance between alveolar ventilation and pulmonary capillary blood flow, causing impaired gas exchange and hypoxemia.

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