What are the management options for acute crises in sickle cell anemia?

Management of Acute Crises in Sickle Cell Anemia

The primary management of acute sickle cell crises requires prompt pain control with opioid analgesics, aggressive hydration, oxygen therapy as needed, and prevention of complications through careful monitoring and supportive care. 1, 2

Pain Management

• Patient-controlled analgesia (PCA) techniques are effective for managing moderate to severe pain during vaso-occlusive crisis, with scheduled around-the-clock dosing preferred over as-needed dosing 1
• For severe pain, parenteral opioids such as morphine should be administered promptly, with regular reassessment using validated pain scales 2
• Long-acting opioid medications should be continued if the patient is already taking them for chronic pain management 1
• An oral tier approach can facilitate transition from IV PCA to oral opioids while maintaining adequate pain control 3

Hydration Therapy

• Aggressive hydration is crucial as patients with sickle cell disease have impaired urinary concentrating ability and dehydrate easily 1
• Oral hydration is preferred when possible, but intravenous fluids should be administered if oral intake is inadequate 2
• 5% dextrose solution or 5% dextrose in 25% normal saline is recommended for intravenous hydration rather than normal saline to avoid excessive sodium load due to hyposthenuria 4
• Fluid balance should be carefully monitored to prevent overhydration, with accurate measurement of intake and output 2

Oxygen Therapy

• Baseline oxygen saturation should be documented and monitored, with oxygen therapy administered to maintain SpO2 above baseline or 96% (whichever is higher) 1
• Continuous oxygen monitoring is recommended until saturation is maintained at baseline in room air 2
• Oxygen administration should be reserved for hypoxic patients rather than routinely given to all patients 4

Infection Management

• Infections are a leading cause of morbidity and mortality in sickle cell disease and can precipitate crises 1
• 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 2
• Vigorous antibiotic therapy is required as infections are the leading cause of morbidity and mortality 4

Prevention and Management of Complications

Acute Chest Syndrome

• Early recognition and aggressive treatment with oxygen, incentive spirometry, analgesics, and antibiotics are essential 1
• Incentive spirometry every 2 hours is recommended for prevention, especially in patients with thoracoabdominal pain 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 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

• Characterized by a rapidly enlarging spleen and a decrease in hemoglobin level of more than 2 g/dL below baseline 1
• Prompt recognition and careful administration of red blood cell transfusions are crucial 1
• Care must be taken to avoid acute overtransfusion to a hemoglobin greater than 10 g/dL 1

Priapism

• Treated as a painful event with hydration and analgesia, often at home, unless it lasts more than 4 hours 1
• Immediate notification of the hematology team is recommended when a patient presents with priapism 5
• Men receiving regional anesthesia require special attention as they may not notice priapism due to altered sensation 5

Transfusion Therapy

• Blood transfusion has limited indications such as acute hemolytic, aplastic or sequestration crises 6
• Exchange blood transfusion is used in complications such as acute chest syndrome, priapism, or perioperatively 6
• Transfused blood should be Hb S negative, Rh and Kell antigen matched to reduce risk of alloimmunization 6

Temperature Management

• Patients should be kept normothermic as hypothermia can lead to shivering and peripheral stasis, increasing sickling 2
• Regular temperature monitoring is important as fever may be an early sign of sickling or infection 2

Thromboprophylaxis

• All post-pubertal patients should receive thromboprophylaxis as they have an increased risk of deep vein thrombosis 2
• Additional precautions may be needed for patients with risk factors such as immobility or previous venous thromboembolism 2

Common Pitfalls and Caveats

• Avoiding hypoxia is crucial as it can precipitate sickling 1
• Overhydration can lead to complications such as pulmonary edema and should be avoided 1
• For patients requiring surgery during a crisis, there should be a low threshold for admission to high dependency or intensive care units 7, 2
• Hydroxyurea therapy has been shown to reduce hospital length of stay and opioid requirements during acute painful episodes 8