Emergency Department Workup for 16-Year-Old Female with Chest Pain and Sickle Cell Disease
This patient requires immediate emergency transfer to an acute care setting with rapid triage, as acute chest syndrome (ACS) is a leading cause of death in sickle cell disease with up to 13% all-cause mortality, and ACS must be actively excluded in every sickle cell patient presenting with chest pain. 1, 2
Immediate Actions (Within 30 Minutes)
Critical Initial Steps
- Administer first analgesic dose within 30 minutes of triage with parenteral opioids (morphine) for chest pain using scheduled around-the-clock dosing or patient-controlled analgesia rather than as-needed dosing 2
- Obtain baseline oxygen saturation immediately and initiate continuous pulse oximetry monitoring, maintaining SpO2 at baseline or ≥96% (whichever is higher) 3, 2
- Notify hematology immediately upon presentation—this is a critical action that should not be delayed 2
- Initiate aggressive intravenous hydration immediately, as sickle cell patients have impaired urinary concentrating ability and dehydrate easily, which precipitates sickling 2
Mandatory Laboratory Studies
Order immediately upon arrival 2:
- Complete blood count with reticulocyte count to establish baseline hemoglobin level
- Comprehensive metabolic panel to assess renal function and electrolytes
- Blood type and crossmatch in preparation for potential transfusion
- Blood cultures if temperature ≥38.0°C or any signs of sepsis, as infections are a leading cause of morbidity and mortality in sickle cell disease 2
Essential Imaging
- Chest radiograph is the gold standard imaging modality to identify new segmental infiltrate, which defines ACS when combined with respiratory symptoms 4, 5
- Obtain chest X-ray immediately even if initial presentation lacks respiratory symptoms, as ACS can develop after initial presentation for a pain event 3
Acute Chest Syndrome Exclusion Criteria
ACS is defined by new segmental infiltrate on chest radiograph plus any of the following 3, 5:
- Fever >38.5°C
- Cough or wheezing
- Hypoxemia (PaO2 <60 mmHg or SpO2 decline)
- Tachypnea
- Lower respiratory tract symptoms
- Chest pain
Cardiac Evaluation
Acute coronary syndrome must also be excluded in this adolescent patient, as AMI occurs in sickle cell patients at relatively early age without traditional risk factors, and death from ACS in sickle cell disease is significantly high compared to age-, sex-, and race-matched controls 1
Obtain:
- 12-lead electrocardiogram
- Troponin level to rule out myocardial ischemia 1
Treatment Protocol While Completing Workup
Respiratory Management
- Administer supplemental oxygen to maintain SpO2 above baseline or ≥96%, as hypoxia precipitates sickling and worsens vaso-occlusion 2
- Initiate incentive spirometry every 2 hours immediately for all admitted patients, especially those with chest pain, as this significantly reduces ACS development 3, 2
Antimicrobial Therapy
- Initiate empiric antibiotics covering atypical pathogens if temperature ≥38.0°C or signs of sepsis after obtaining blood cultures 3, 4
Pain Control
- Provide aggressive pain control with parenteral opioids, as delays in pain management are associated with worse morbidity 2
- Use scheduled dosing rather than PRN to maintain adequate analgesia 3, 2
Disposition Criteria
Maintain a low threshold for ICU or high-dependency unit admission for 2:
- Respiratory distress present
- Increasing oxygen requirements
- History of previous ACS
- Comorbidities present
- Signs of clinical deterioration
Common Pitfalls to Avoid
- Do not delay analgesia while completing workup—first dose must be within 30 minutes 2
- Do not assume normal initial chest X-ray excludes ACS—infiltrates may develop 24-48 hours after presentation 4
- Do not overlook cardiac causes—this age group can develop AMI without traditional risk factors 1
- Avoid overhydration which can lead to pulmonary edema, but ensure adequate hydration to prevent sickling 3
- Do not discharge without hematology consultation and clear follow-up plan 2