What is the treatment for sickle cell crisis?

Management of Sickle Cell Crisis

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

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 being preferred over as-needed dosing 1, 2
• For severe pain, parenteral opioids such as morphine are indicated and should be administered promptly 2, 3
• Long-acting opioid medications should be continued if the patient is already taking them for chronic pain management 1
• Regular pain assessment using validated pain scales is essential, with frequent reassessment to ensure adequate pain control 1, 4

Hydration Therapy

• Aggressive hydration is crucial as patients with sickle cell disease have impaired urinary concentrating ability and dehydrate easily 1, 5
• Oral hydration is preferred when possible, but intravenous fluids should be administered if oral intake is inadequate 1
• 5% dextrose solution or 5% dextrose in 0.25% normal saline is recommended for intravenous hydration rather than normal saline to avoid excessive sodium load due to hyposthenuria 5
• Fluid balance should be carefully monitored to prevent overhydration, with accurate measurement of intake and output 1

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 1
• Oxygen should be used judiciously and reserved for hypoxic patients rather than routinely administered to all patients 5

Infection Management

• Infections are a leading cause of morbidity and mortality in sickle cell disease and can precipitate crises 1, 5
• 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
• Vigorous antibiotic therapy is required for confirmed infections 5

Prevention and Management of Complications

Acute Chest Syndrome

• Acute chest syndrome is a life-threatening complication characterized by a new segmental infiltrate on chest radiograph, lower respiratory tract symptoms, chest pain, and/or hypoxemia 2
• Early recognition and aggressive treatment with oxygen, incentive spirometry, analgesics, and antibiotics are essential 2
• Simple or exchange transfusions may be necessary in severe cases 2, 1
• Incentive spirometry every 2 hours is recommended for prevention, especially in patients with thoracoabdominal pain 1, 6

Stroke

• Any acute neurologic symptom other than transient mild headache requires urgent evaluation 2
• Initial evaluation includes CBC, reticulocyte count, blood type and crossmatch, and neuroimaging 2
• Acute treatment may include partial exchange transfusion or erythrocytapheresis to reduce HbS to <30% and raise hemoglobin to 10 g/dL 2

Splenic Sequestration

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

Priapism

• Priapism is a prolonged painful erection that commonly occurs in children and adolescents with SCD 2, 7
• Treated as a painful event with hydration and analgesia, often at home, unless it lasts more than 4 hours 2
• Immediate notification of the hematology team is recommended when a patient presents with priapism 7

Disease-Modifying Therapies

• Hydroxyurea is the first-line disease-modifying therapy for SCD, increasing fetal hemoglobin and reducing red cell sickling 8, 6
• Hydroxyurea is strongly recommended for adults with 3 or more severe vaso-occlusive crises during any 12-month period, with SCD pain or chronic anemia interfering with daily activities, or with severe or recurrent episodes of acute chest syndrome 6
• Other disease-modifying therapies include L-glutamine, crizanlizumab, and voxelotor, which have been approved as adjunctive or second-line agents 8
• Hematopoietic stem cell transplant is the only curative therapy but is limited by donor availability 8

Transfusion Therapy

• Blood transfusions should be given only when really indicated, not routinely for all crises 5
• In patients with alloantibodies for whom antigen-negative blood is unavailable or with a history of multiple or life-threatening delayed hemolytic transfusion reactions, immunosuppressive therapy may be considered 2
• For those who refuse blood transfusion (e.g., Jehovah's Witnesses), pre-operative erythroid-stimulating agents or hydroxyurea may be appropriate 2

Multidisciplinary Care

• Regular assessment by hematology specialists is recommended for patients with moderate to severe crises 1
• Clinical teams should work in partnership with patients and their families, keeping them informed of clinical decisions 1
• For patients requiring surgery during a crisis, there should be a low threshold for admission to high dependency or intensive care units 1, 2

Common Pitfalls and Caveats

• Avoiding hypoxia is crucial as it can precipitate sickling 1
• Patients should be kept normothermic as hypothermia can lead to shivering and peripheral stasis, increasing sickling 1
• Overhydration can lead to complications such as pulmonary edema and should be avoided 1
• While SCD is characterized by acute and chronic pain, patients are not more likely to develop addiction to pain medications than the general population 8