What is the treatment for a sickle cell crisis?

Treatment for Sickle Cell Crisis

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

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 1
• 5% dextrose solution or 5% dextrose in 25% normal saline is recommended for intravenous hydration rather than normal saline due to reduced ability to excrete sodium load 3
• 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, 2
• Continuous oxygen monitoring is recommended until saturation is maintained at baseline in room air 1
• Oxygen administration should be reserved for hypoxic patients rather than given routinely 3

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 1
• Vigorous antibiotic therapy is required as infections are the leading cause of morbidity and mortality 3

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 1
• Early recognition and aggressive treatment with oxygen, incentive spirometry, analgesics, and antibiotics are essential 1
• Simple or exchange transfusions may be necessary in severe cases 1
• Incentive spirometry every 2 hours is recommended for prevention, especially in patients with thoracoabdominal pain 1

Stroke

• Any acute neurologic symptom other than transient mild headache requires urgent evaluation for stroke 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

• Priapism is a prolonged painful erection that commonly occurs in children and adolescents with SCD 1
• 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 1, 4

Transfusion Therapy

• Blood transfusion should be given only when clearly indicated, not routinely 3
• 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 1

Disease-Modifying Therapies

• Hydroxyurea increases fetal hemoglobin (HbF) and reduces red blood cell sickling, making it the first-line disease-modifying therapy for most individuals with SCD 5
• Three additional therapies - L-glutamine, crizanlizumab, and voxelotor - have been approved as adjunctive or second-line agents 5
• L-glutamine has been shown to reduce hospitalization rates by 33% and mean length of stay from 11 to 7 days compared with placebo 5
• Crizanlizumab reduced pain crises from 2.98 to 1.63 per year compared with placebo 5
• Voxelotor increased hemoglobin by at least 1 g/dL significantly more than placebo (51% vs 7%) 5

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

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
• Despite the need for opioid analgesics, patients with SCD are not more likely to develop addiction to pain medications than the general population 5
• Delays in pain management are common but should be avoided; acute pain management should be initiated quickly 6, 7