From the Guidelines
Managing sickle cell crisis requires prompt intervention with pain control, hydration, and oxygen therapy, as outlined in the most recent guidelines from the Association of Anaesthetists in 2021 1. The primary goals of management are to reduce morbidity, mortality, and improve quality of life.
Key Management Strategies
- Pain management: start with NSAIDs like ibuprofen (400-800mg every 6 hours) for mild pain, then progress to opioids such as morphine (0.1-0.15mg/kg IV every 3-4 hours) or hydromorphone (0.015-0.02mg/kg IV every 3-4 hours) for moderate to severe pain, as recommended by expert consensus opinion 1.
- Hydration: maintain adequate hydration with IV fluids at 1.5 times maintenance rate, typically using 0.9% normal saline or 0.45% saline with dextrose, to prevent dehydration and reduce the risk of sickle-related complications 1.
- Oxygen therapy: provide supplemental oxygen to maintain saturation above 95% to reduce the risk of tissue ischemia and sickling 1.
Monitoring and Prevention
- Monitor vital signs, pain scores, and oxygen saturation every 2-4 hours to promptly identify any complications or worsening of the patient's condition 1.
- Incentive spirometry (10 breaths every 2 hours while awake) helps prevent acute chest syndrome, a common complication of sickle cell crisis 1.
- Consider exchange transfusion for severe complications like stroke, acute chest syndrome, or multi-organ failure, as recommended by the American Society of Hematology guidelines 1.
Prevention Strategies
- Hydroxyurea therapy can increase fetal hemoglobin production and reduce crisis frequency, and should be considered as part of a comprehensive management plan 1.
- Patients should be educated about avoiding triggers such as dehydration, extreme temperatures, high altitudes, and strenuous exercise to reduce the risk of sickle cell crisis 1.
From the Research
Guidelines for Managing Sickle Cell Disease Crisis
- The management of sickle cell disease crisis requires prompt medical intervention to provide relief and comfort to the patient 2.
- Effective analgesia is crucial in treating the painful crisis of sickle cell disease, with oral morphine protocol being a useful tool for providing consistent oral analgesia to patients with sickle crisis pain 3.
- Intravenous hydration is frequently used in emergency and inpatient settings, but it can lead to adverse outcomes like fluid overload, pulmonary edema, and increased length of stay 4.
- A 5% dextrose solution or 5% dextrose in 25% normal saline is recommended for intravenous hydration, as the haemoglobinopathy may cause hyposthenuria with reduced ability to excrete the sodium load in normal saline 2.
- Infections require vigorous antibiotic therapy, and oxygen administration should be reserved for hypoxic patients, with blood transfusion given only when really indicated 2.
Acute Complications of Sickle Cell Disease
- Acute complications are the most frequent causes of hospitalization and morbidity in patients with sickle cell disease, with vaso-occlusive crisis being responsible for more than 90% of hospitalization 5.
- Evaluation of acute complications includes the understanding of chronic complications, particularities related to patient's age, the search for a triggering factor, and a differential diagnosis 5.
- Acute chest syndrome, cerebrovascular accidents, and priapism are life-threatening complications of sickle cell disease that deserve urgent attention 2.