Initial Management of Sickle Cell Disease During Acute Crises
Patients with sickle cell disease in acute crisis require immediate aggressive hydration with IV fluids and oxygen therapy if hypoxic, along with prompt pain management and prevention of complications. 1, 2
Hydration Therapy
- Aggressive hydration is crucial as patients with sickle cell disease have impaired urinary concentrating ability and dehydrate easily 3, 1
- 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 to prevent both dehydration and overhydration 3, 4
- A 5% dextrose solution or 5% dextrose in 25% normal saline may be preferable to normal saline due to reduced ability to excrete sodium loads in sickle cell patients 5
Oxygen Therapy
- Baseline oxygen saturation should be documented and monitored, with supplemental oxygen administered to maintain SpO2 above baseline or 96% (whichever is higher) 6, 2
- Continuous oxygen monitoring is recommended until saturation is maintained at baseline in room air 1, 2
- Avoiding hypoxia is critical as it can precipitate sickling and worsen the crisis 2, 7
Pain Management
- Prompt administration of analgesics is essential, with patient-controlled analgesia (PCA) techniques being effective for moderate to severe pain 6, 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 3, 2
- Regular pain assessment using validated pain scales is essential, with frequent reassessment to ensure adequate pain control 1, 2
Infection Management
- Blood cultures should be obtained if the patient becomes febrile, and antibiotics should be started promptly if temperature reaches ≥38.0°C or if there are signs of sepsis 1, 2
- Antibiotic prophylaxis should be administered according to protocols, as patients with sickle cell disease are more susceptible to infections 6, 2
Prevention of Complications
- Early mobilization should be encouraged when appropriate to prevent complications like deep vein thrombosis 6, 1
- Incentive spirometry every 2 hours is recommended for prevention of acute chest syndrome, especially in patients with thoracoabdominal pain 1, 2
- Patients should be kept normothermic as hypothermia can lead to shivering and peripheral stasis, increasing sickling 6, 2
Monitoring and Multidisciplinary Care
- Regular assessment by hematology specialists is recommended for patients with moderate to severe crises 1, 2
- There should be a low threshold for admission to high dependency or intensive care units for patients with severe complications 6, 2
- Multidisciplinary care involving hematologists, pain specialists, and other relevant specialists is crucial for optimal management 1, 2
Specific Complications Requiring Special Attention
- Acute chest syndrome requires aggressive treatment with oxygen, incentive spirometry, analgesics, and antibiotics 1, 7
- Priapism should be treated as a painful event with hydration and analgesia, with immediate notification of the hematology team 6, 1
- Any acute neurologic symptom other than transient mild headache requires urgent evaluation for stroke 1, 7
Blood Transfusion Considerations
- Blood transfusion should be reserved for specific indications such as acute hemolytic, aplastic or sequestration crises, acute chest syndrome, stroke, or perioperatively 8, 9
- Exchange transfusion may be necessary in severe cases of acute chest syndrome or stroke 1, 8
- For patients who refuse blood transfusion or for whom it is not an option, alternative therapies such as erythroid-stimulating agents or hydroxyurea may be appropriate 1, 9