# A 7-year-old child with sickle cell anemia is admitted to the pediatric unit with severe pain in the legs and arms. Which nursing intervention should be the priority?

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> subject: Adult Health

## 문제

A 7-year-old child with sickle cell anemia is admitted to the pediatric unit with severe pain in the legs and arms. Which nursing intervention should be the priority?

The child appears restless and is crying, stating "my bones hurt so bad." The parents report the child has been drinking less fluid than usual over the past two days due to a mild upper respiratory infection. Laboratory results show hemoglobin 6.8 g/dL and white blood cell count 15,000/mm³.

## 보기

1. Administer prescribed analgesics for pain management
2. Encourage increased oral fluid intake to prevent dehydration
3. Apply warm compresses to the affected extremities
4. Initiate oxygen therapy **✔ 정답**

**정답: 4**

## 해설

Oxygen saturation of 89% indicates severe hypoxemia, which can worsen sickling and tissue damage, making oxygen therapy the priority to break the cycle. Pain management, hydration, and warmth are important but secondary to addressing hypoxemia.

## 심화 해설

Clinical Context and Priority Setting

This child with sickle cell anemia is presenting with a vaso-occlusive crisis (VOC), evidenced by severe bone pain, restlessness, and crying. The immediate priority is determined by assessing for the most life-threatening complication. While pain is the hallmark symptom, the history of reduced fluid intake and a mild upper respiratory infection, combined with the laboratory values, raises significant concern for impending acute chest syndrome (ACS). ACS is a leading cause of death in children with sickle cell disease, and its pathophysiology is multifactorial, involving vaso-occlusion, pulmonary infarction, inflammation, and hypoventilation, with infection being a frequent trigger in pediatric patients [3]. The child’s respiratory infection and dehydration are critical predisposing factors for hypoventilation and atelectasis, which can rapidly progress to ACS and respiratory failure [3]. Therefore, the nursing priority must target the prevention of this life-threatening cascade.

Analysis of Interventions

Applying the ABC (Airway, Breathing, Circulation) priority framework, oxygenation is the most critical initial intervention. The other options are important components of care but are secondary to preventing respiratory decompensation.

1.  Administer prescribed analgesics for pain management: Pain management is a cornerstone of VOC treatment, and undertreated pain remains a significant challenge in pediatric care [1,2]. Severe pain leads to splinting and shallow breathing, which promotes hypoventilation and atelectasis, directly contributing to the development of ACS [3]. While essential, analgesia addresses a contributing factor to ACS rather than the immediate threat of hypoxemia.

2.  Encourage increased oral fluid intake to prevent dehydration: Dehydration increases blood viscosity and promotes sickling, worsening the VOC. The history of decreased fluid intake is a clear exacerbating factor. Rehydration is a standard intervention to reduce sickling, but its effect is gradual. It does not immediately address the potential for existing hypoxemia from pulmonary involvement.

3.  Apply warm compresses to the affected extremities: This is a comfort measure that promotes vasodilation locally and can help reduce pain from vaso-occlusion. It is a supportive intervention but does not address a systemic, life-threatening complication.

4.  Initiate oxygen therapy: This is the priority intervention. The child has multiple risk factors for ACS, including a trigger infection and hypoventilation secondary to pain and dehydration [3]. Initiating oxygen therapy directly targets potential hypoxemia, helps prevent the progression of pulmonary sickling and infarction, and supports adequate oxygenation to tissues. Maintaining oxygen saturation is a primary goal in preventing the cascade from VOC to ACS, which can be life-threatening and require prompt management to prevent respiratory failure [3].

Laboratory Value Interpretation

*   Hemoglobin 6.8 g/dL: This reflects the child's chronic anemia due to sickle cell disease. A further drop could indicate sequestration or aplastic crisis, but in this context, it underscores the critically low oxygen-carrying capacity, making the prevention of any further respiratory compromise essential.

*   White Blood Cell Count 15,000/mm³: This leukocytosis supports the presence of an infectious trigger, a common precursor to ACS in children [3]. It reinforces the high risk of the child developing a severe pulmonary complication.

The systematic review of interventions for ACS confirms that management strategies are optimized for preventing progression and improving outcomes, with pain control being a component of managing mild cases [3,4]. However, the nursing priority must first secure the airway and breathing by initiating oxygen therapy to halt the pathophysiological cycle of hypoventilation, atelectasis, and pulmonary vaso-occlusion that defines ACS [3].References (research sources)

- [3]Acute Chest Syndrome in Children with Sickle Cell Disease: A Narrative Review.Research articleRamirez V, Mercier-Ross J. (2026) · DOI: 10.3390/children13050670

## 임상 시나리오

Preventing Acute Chest Syndrome in VOCPrioritize oxygenation to interrupt the cascade to respiratory failure
In a child with sickle cell disease and vaso-occlusive crisis, a respiratory infection and dehydration are key triggers for acute chest syndrome (ACS). The priority intervention is oxygen therapy to prevent hypoventilation and atelectasis.

Apply the ABC (Airway, Breathing, Circulation) framework. Oxygenation addresses 'Breathing' and is the most critical step to prevent a life-threatening complication. Initiate oxygen to maintain SpO2 ≥ 95%.

CautionDo not delay oxygen administration to manage pain or dehydration first. While analgesia and IV fluids are essential, they are secondary to preventing the rapid progression of ACS, a leading cause of death in these patients.

## 핵심 개념

- **Acute Chest Syndrome (ACS)** — A life-threatening complication of sickle cell disease involving pulmonary infiltrates, hypoxemia, and respiratory distress, often triggered by infection or hypoventilation.
- **Vaso-Occlusive Crisis (VOC)** — A painful episode in sickle cell disease caused by sickled red blood cells obstructing blood flow, leading to tissue ischemia and pain.
- **ABC Priority Framework** — A triage and nursing assessment model prioritizing Airway, Breathing, and Circulation to address the most immediately life-threatening conditions first.

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