What is the management plan for a patient experiencing a sickle cell crisis?

Management of Sickle Cell Crisis

Prompt pain control with parenteral opioids, aggressive intravenous hydration, and oxygen therapy to maintain SpO2 above baseline or 96% form the cornerstone of acute sickle cell crisis management, with continuous monitoring for life-threatening complications including acute chest syndrome, stroke, and infection. 1

Immediate Pain Management

Severe pain requires rapid initiation of parenteral opioids such as morphine, administered promptly without delay. 1, 2

• Patient-controlled analgesia (PCA) is the preferred delivery method for moderate to severe pain, with scheduled around-the-clock dosing superior to as-needed dosing. 1
• Continue any long-acting opioid medications the patient is already taking for chronic pain management. 3, 1
• Use validated pain assessment scales with frequent reassessment to ensure adequate control. 3, 1
• Multimodal analgesia should be considered, incorporating regional blocks when appropriate. 3

Critical pitfall: Opioid dependency is rare in sickle cell disease; opioid sensitivity is more common, so avoid under-dosing based on unfounded concerns about addiction. 3

Aggressive Hydration Therapy

Patients with sickle cell disease have impaired urinary concentrating ability and dehydrate easily, making aggressive hydration crucial. 1

• Oral hydration is preferred when the patient can tolerate adequate intake. 3, 1
• Administer intravenous fluids if oral intake is inadequate or impossible. 1
• Use 5% dextrose solution or 5% dextrose in 25% normal saline rather than normal saline alone, as the hyposthenuria in sickle cell disease reduces ability to excrete sodium loads. 4
• Monitor fluid balance carefully with accurate measurement of intake and output to prevent overhydration complications such as pulmonary edema. 3, 1

Oxygen Therapy

Document baseline oxygen saturation and administer supplemental oxygen only to maintain SpO2 above baseline or 96%, whichever is higher. 1

• Do not give continuous oxygen therapy unless necessary—reserve oxygen administration for hypoxic patients. 3, 4
• Continue oxygen monitoring until saturation is maintained at baseline in room air. 1
• Oxygen therapy may be required at night for several nights, particularly following thoracic or abdominal pain. 3

Infection Surveillance and Management

Infections are a leading cause of morbidity and mortality in sickle cell disease and can precipitate crises. 1, 4

• Obtain blood cultures if the patient becomes febrile. 1
• Start antibiotics promptly if temperature reaches ≥38.0°C or if there are signs of sepsis. 1
• Patients are susceptible to bacterial infection due to functional hyposplenism. 1

Prevention and Recognition of Life-Threatening Complications

Acute Chest Syndrome

Acute chest syndrome is characterized by new segmental infiltrate on chest radiograph, lower respiratory tract symptoms, chest pain, and/or hypoxemia—it is life-threatening and requires aggressive treatment. 1

• Implement incentive spirometry every 2 hours for prevention, especially in patients with thoracoabdominal pain. 1
• Treat with oxygen, incentive spirometry, analgesics, and antibiotics. 1
• Simple or exchange transfusions may be necessary in severe cases. 1

Stroke

Any acute neurologic symptom other than transient mild headache requires urgent evaluation for stroke. 1

• Initial evaluation includes CBC, reticulocyte count, blood type and crossmatch, and neuroimaging. 1
• Acute treatment may include partial exchange transfusion or erythrocytapheresis to reduce HbS to <30% and raise hemoglobin to 10 g/dL. 1

Splenic Sequestration

Splenic sequestration presents with rapidly enlarging spleen and hemoglobin decrease of more than 2 g/dL below baseline. 1

• Prompt recognition and careful red blood cell transfusions are crucial. 1
• Critical pitfall: Avoid acute overtransfusion to hemoglobin greater than 10 g/dL. 1

Priapism

Priapism is a prolonged painful erection commonly occurring in children and adolescents with sickle cell disease. 1

• Treat as a painful event with hydration and analgesia, often at home, unless it lasts more than 4 hours. 1
• Immediately notify the hematology team when a patient presents with priapism. 1
• Special consideration: Male patients receiving regional anesthesia may not notice priapism due to altered sensation and require regular examination. 3

Monitoring and Supportive Care

Maintain a low threshold for admission to high dependency or intensive care units for patients with severe crises or significant comorbidities. 5

• Standard continuous monitoring should be used for all patients. 5
• Consider near-infrared spectroscopy to monitor cerebral oxygenation in high-risk patients. 5
• Monitor temperature regularly as fever may be an early sign of sickling or infection. 5
• Thromboprophylaxis is required for all post-pubertal patients due to increased deep vein thrombosis risk. 5

Environmental Management

Keep patients normothermic, as hypothermia can lead to shivering and peripheral stasis, increasing sickling. 1

• Avoid hypoxia, which can precipitate sickling. 1

Role of Hydroxyurea

While hydroxyurea is strongly recommended for adults with 3 or more severe vasoocclusive crises during any 12-month period, it is a disease-modifying therapy for long-term management rather than acute crisis treatment. 2 Hydroxyurea acts as a ribonucleotide reductase inhibitor and requires dose reduction in patients with creatinine clearance <60 mL/min. 6