What is the management approach for a patient with sickle cell crisis presenting with chest pain?

Management of Sickle Cell Crisis with Chest Pain

Patients with sickle cell disease presenting with chest pain require immediate emergency transfer to an acute care setting with aggressive treatment for potential acute chest syndrome (ACS), including rapid pain control with parenteral opioids, oxygen therapy, incentive spirometry, antibiotics, and early involvement of hematology for consideration of transfusion. 1

Immediate Assessment and Triage

Critical Recognition

• Chest pain in sickle cell crisis may represent acute chest syndrome, a life-threatening complication that can present acutely or develop after initial presentation for a pain event, with or without fever 2, 1
• ACS is characterized by new segmental infiltrate on chest radiograph (which may not be visible initially) accompanied by lower respiratory tract symptoms, chest pain, and/or hypoxemia 2, 1
• Patients may deteriorate rapidly with progression to pulmonary failure and death, making early recognition critical 2

Initial Diagnostic Workup

• Obtain baseline oxygen saturation immediately, as decreasing SpO2 provides early warning of worsening ACS 1
• Order chest radiograph as the gold standard imaging modality, though infiltrates may not be visible on first presentation 2, 3
• Obtain complete blood count to establish baseline hemoglobin level 1
• Obtain blood cultures if fever is present (temperature ≥38.0°C) 1

Aggressive Pain Management

Parenteral Opioid Therapy

• Administer parenteral opioids such as morphine immediately for severe pain using scheduled around-the-clock dosing or patient-controlled analgesia (PCA), not as-needed dosing 2, 1
• PCA techniques show superior outcomes with lower overall morphine consumption compared to continuous infusion 4
• Delays in addressing and undertreatment of sickle cell pain are common and must be avoided 2

Pain Management Pitfalls

• Because there are rarely objective physical findings with severe pain in sickle cell disease, patients may be stigmatized when seeking pain relief 2
• Studies document disparities in pain management between racial groups, with children of color receiving less pain medication 2
• Inadequately treated thoraco-abdominal pain leads to hypoventilation and reduced inspiratory effort, which can precipitate or worsen ACS 2

Respiratory Support Protocol

Oxygen Therapy

• Administer oxygen to maintain SpO2 above baseline or 96% (whichever is higher) 1
• Continue oxygen monitoring until saturation is maintained at baseline in room air 1
• Reserve oxygen administration for hypoxic patients rather than routine use 5

Incentive Spirometry

• Implement incentive spirometry every 2 hours to encourage deep inspiratory effort and prevent atelectasis 2, 1
• This intervention is critical as hypoventilation from pain is a major causative factor of ACS 2
• Consider continuous positive airway pressure, high-flow nasal oxygen, or nasopharyngeal airway for patients with increasing respiratory distress 1

Hydration Management

Fluid Administration Strategy

• Provide aggressive hydration while carefully monitoring fluid balance to prevent overhydration 1
• Oral hydration is preferred when possible, but administer intravenous fluids if oral intake is inadequate 1
• Use 5% dextrose solution or 5% dextrose in 25% normal saline rather than normal saline, as the haemoglobinopathy causes hyposthenuria with reduced ability to excrete sodium load 5
• Avoid excessive hydration, which can lead to pulmonary edema 1

Antimicrobial Therapy

Infection Management

• Initiate antibiotics if temperature reaches ≥38.0°C or if there are signs of sepsis 1
• Infection (viral, bacterial, Mycoplasma, or Chlamydia) is a specific causative factor in many ACS cases 2
• Infection was a contributing factor in 56% of deaths from ACS in a large multicenter study 6
• Vigorous antibiotic therapy is required as infections are the leading cause of morbidity and mortality in sickle cell disease 5

Transfusion Decisions

Indications for Transfusion

• Simple or exchange transfusions are often necessary for severe cases of ACS 1
• Discuss transfusion decisions urgently with hematology, considering the 7-30% risk of alloimmunization 1
• For patients with bilateral lung infiltrates, automated red cell exchange (RCE) is preferred over simple transfusions, as bilateral infiltrates indicate severe, progressive disease requiring rapid reduction of HbS levels to <30% (ideally <20%) 1

Transfusion Protocol

• Use phenotypically matched transfusions to improve oxygenation, which has shown a 1% rate of alloimmunization 6
• Avoid simple transfusion alone if the patient has high baseline hemoglobin, as this increases viscosity and worsens vaso-occlusion 1
• Do not delay exchange transfusion while waiting for simple transfusion to work in patients with bilateral infiltrates 1

Temperature and Environmental Control

• Maintain normothermia as hypothermia leads to shivering and peripheral stasis, which increases sickling 1
• Use active warming measures if needed 1

Monitoring and Escalation of Care

ICU Admission Criteria

• Maintain a low threshold for admitting patients to high dependency or intensive care unit after presentation, depending on severity and patient comorbidities 2
• Consider ICU admission for rapidly progressive disease despite initial interventions 1
• Consider ICU admission for consideration of exchange transfusion 1
• Thirteen percent of ACS patients require mechanical ventilation, though 81% of those requiring mechanical ventilation recover with aggressive treatment 6

Ongoing Monitoring

• Perform regular monitoring of oxygen saturation 1
• Regular pain assessment using validated pain scales 1
• Monitor for neurologic events, which occur in 11% of ACS patients, with 46% of those developing respiratory failure 6

Additional Supportive Measures

Thromboprophylaxis

• Implement thromboprophylaxis for post-pubertal patients due to increased risk of deep vein thrombosis 1

Mobilization

• Encourage early mobilization when appropriate 1
• Provide chest physiotherapy if the patient is unable to mobilize 1

Multidisciplinary Coordination

• Involve hematology specialists immediately for daily assessment and transfusion decisions 1
• Work in partnership with patients and their families, keeping them informed of clinical decisions 1
• Patients with sickle cell disease and their caregivers know their disease best, and all actions should result from shared decision-making 2

Common Pitfalls to Avoid

• Do not undertreat pain due to concerns about opioid use—inadequate pain control worsens respiratory mechanics and precipitates ACS 2
• Do not wait for chest radiograph infiltrates to appear before treating suspected ACS, as infiltrates may not be visible on initial presentation 2, 1
• Do not overlook the possibility that chest pain initially attributed to vaso-occlusive crisis may evolve into ACS, as nearly half of ACS patients were initially admitted for another reason, mainly pain 6
• Do not use normal saline for hydration due to impaired sodium excretion in sickle cell patients 5