Management of Sickle Cell Disease During Acute Crises
Patients with sickle cell disease require aggressive hydration and oxygen therapy (if indicated) during acute crises to prevent complications and reduce morbidity and mortality.
Initial Management Approach
- Aggressive hydration is crucial as patients with sickle cell disease have impaired urinary concentrating ability and dehydrate easily 1, 2
- Oral hydration is preferred when possible, but intravenous fluids should be administered if oral intake is inadequate 1, 3
- Meticulous fluid management with accurate measurement and replacement of fluid losses is essential, with monitoring to prevent overhydration 3, 4
- Oxygen therapy should be 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
Pain Management
- Patient-controlled analgesia (PCA) techniques are effective for managing moderate to severe pain during vaso-occlusive crisis 5, 1
- 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 3, 1
- Regular pain assessment using validated pain scales is essential, with encouragement for patients to report pain, particularly pain similar to their usual sickle pain 3, 2
Prevention and Management of Complications
- Patients should be kept normothermic as hypothermia can lead to shivering and peripheral stasis, which increases sickling 5, 2
- Early mobilization should be encouraged when appropriate to prevent complications like deep vein thrombosis 5, 1
- Incentive spirometry every 2 hours is recommended for prevention of acute chest syndrome, especially in patients with thoracoabdominal pain 1
- Regular assessment by hematology specialists is recommended for patients with moderate to severe crises 1, 2
Special Considerations
- Blood transfusion should be reserved for specific indications such as acute hemolytic, aplastic or sequestration crises, and not routinely used for uncomplicated painful crises 6, 7
- For patients with priapism, there should be a low threshold for admission to high dependency or intensive care units 5, 2
- Men receiving regional anesthesia require special attention as they may not notice the presence of priapism due to altered sensation 3, 5
Common Pitfalls and Caveats
- Avoid using normal saline as the sole IV fluid as it may be associated with adverse outcomes such as poor pain control and volume overload; consider 5% dextrose or 5% dextrose in 25% normal saline 7, 4
- Overhydration can lead to complications such as pulmonary edema, acute chest syndrome, and new oxygen requirements 8, 4
- Blood transfusions should not be given routinely for uncomplicated painful crises but reserved for specific indications 6, 7
- Oxygen should not be administered unnecessarily to non-hypoxic patients as it provides no benefit and may mask early signs of acute chest syndrome 1, 2