What is the management approach for a sickle cell crisis?

Management of Sickle Cell Priapism in Crisis

The management of sickle cell priapism requires immediate intervention with hydration, analgesia, and monitoring for complications, with men receiving regional anesthesia needing regular examination to ensure priapism has not occurred. 1

Initial Management Approach

• Immediate notification of the hematology team when a patient with sickle cell disease presents with priapism, as this represents an emergency that requires prompt intervention 1, 2
• Aggressive hydration is crucial, as patients with sickle cell disease have impaired urinary concentrating ability and dehydrate easily; oral hydration is preferred when possible, but intravenous fluids should be administered if oral intake is inadequate 1, 2
• Meticulous fluid management with accurate measurement and replacement of fluid losses is essential, with monitoring of fluid balance to prevent overhydration 1, 2

Pain Management

• Patient-controlled analgesia (PCA) techniques are recommended for managing moderate to severe pain during priapism, showing superior outcomes with lower overall morphine consumption compared to continuous infusion 2, 3
• Baseline analgesic use should be documented, and long-acting opioid medication should be continued if the patient is already taking it for chronic pain management 1, 2
• Regular pain assessment using validated pain scales is essential, with encouragement for patients to report pain, particularly pain similar to their usual sickle pain 1, 2

Oxygen Therapy and Temperature Management

• Baseline oxygen saturation should be documented and monitored, with oxygen therapy administered to keep SpO2 above baseline or 96% (whichever is higher) 1, 2
• Continuous oxygen monitoring is recommended until saturation is maintained at baseline in room air 1, 2
• Patients should be kept normothermic as hypothermia can lead to shivering and peripheral stasis, which increases sickling; active warming measures should be employed if needed 1, 2

Special Considerations for Priapism

• Men receiving regional anesthesia require special attention as they may not notice the presence of priapism due to altered sensation; they need regular examination to ensure priapism has not occurred 1
• For patients with priapism, there should be a low threshold for admission to high dependency or intensive care units, especially if the priapism is prolonged or recurrent 1, 2

Infection Prevention and Thromboprophylaxis

• Antibiotic prophylaxis should be administered according to protocols, as patients with sickle cell disease are more susceptible to infections, which can worsen priapism 1, 2
• All post-pubertal patients should receive thromboprophylaxis as they have an increased risk of deep vein thrombosis, with additional precautions for patients with risk factors such as immobility 1, 2

Hydroxyurea Therapy

• Hydroxyurea may be considered as part of the long-term management strategy to reduce the frequency of vaso-occlusive crises, including priapism episodes 4, 5
• Patients on hydroxyurea require monitoring for potential side effects, including cytopenia and skin changes 4

Mobilization and Monitoring

• Early mobilization should be encouraged when appropriate to prevent complications like deep vein thrombosis 1, 2
• Regular assessment by hematology specialists is recommended for patients with priapism, with multidisciplinary care involving urologists for specific management of the priapism itself 1, 2

Patient Education and Follow-up

• Clear instructions about post-crisis care, including pain management and hydration, should be provided to patients 2, 6
• Patients should be informed about the risk of recurrent priapism and educated on when to seek immediate medical attention 2, 6