What is the treatment for sickle cell crisis (vaso-occlusive crisis)?

From the Guidelines

Treating a sickle cell crisis requires prompt medical attention and a comprehensive approach to pain management and hydration, with the most recent guidelines from the American Society of Hematology 2021 recommending hematopoietic stem cell transplantation as a curative intervention for SCD 1.

Key Components of Treatment

• For acute pain, start with NSAIDs like ibuprofen (400-800mg every 6 hours) for mild pain, progressing to opioids such as morphine (0.1-0.15mg/kg IV every 3-4 hours) for severe pain, as recommended by recent guidelines 1.
• Aggressive hydration with IV fluids at 1.5 times maintenance rate is essential to prevent further sickling, and oxygen therapy should be administered if oxygen saturation falls below 95% 1.
• For severe cases, blood transfusions may be necessary to increase the percentage of normal hemoglobin, and antibiotics like ceftriaxone (1-2g IV daily) should be given if infection is suspected, as infections can trigger crises 1.
• Hydroxyurea (15-35mg/kg/day) is a long-term preventive medication that increases fetal hemoglobin production and reduces crisis frequency, as shown in studies 1.

Additional Considerations

• Patients with sickle cell disease have impaired urinary concentrating ability and become dehydrated easily, so dehydration must be avoided, particularly in patients with pre-existing renal dysfunction 1.
• Meticulous peri-operative care and transfusion are crucial in reducing complications, but unnecessary transfusion should be avoided, particularly to reduce the risk of allo-immunisation 1.
• Emerging data suggest that patients with sickle cell disease are at increased risk of COVID-19 infection but may have a relatively mild clinical course, with outcomes determined by pre-existing comorbidities, as for the general population 1.

Long-term Management

• Hematopoietic stem cell transplantation is currently the only curative intervention for SCD, and guidelines recommend its application in clinical practice 1.
• Hydroxyurea is a long-term preventive medication that increases fetal hemoglobin production and reduces crisis frequency, and its use has a positive impact on health-related quality of life in people with HbSS or Sb0-thalassemia 1.
• Patients should continue oral pain medication as needed, maintain hydration by drinking 8-10 glasses of water daily, avoid triggers like extreme temperatures or high altitudes, and follow up with their hematologist to prevent complications and improve quality of life 1.

From the Research

Treatment for Sickle Cell Crisis

The treatment for sickle cell crisis, also known as vaso-occlusive crisis, typically involves the use of analgesics to manage pain.

• Intravenous morphine is often the treatment of choice for severe pain during vaso-occlusive crisis in sickle cell disease (SCD) 2.
• Patient-controlled analgesia (PCA) may be a suitable alternative to continuous infusion (CI) of morphine, as it allows patients to titrate their own analgesia levels, resulting in lower morphine consumption and reduced side effects such as nausea and constipation 2.
• Oral morphine protocols have also been shown to be effective in reducing the number of emergency department visits, total hours spent in the emergency department, and hospital admissions for patients with sickle cell crisis pain 3.
• Regular intravenous narcotic analgesia for the initial 24 hours, supplemented by oral analgesia, may be useful for adult patients with severe acute sickle cell pain crisis when the pain is inadequately controlled by initial emergency room treatment 4.

Alternative Therapies

• Hydroxyurea has been traditionally used to prevent vaso-occlusive crises, including acute chest syndrome, in patients with sickle cell disease (SCD) 5.
• Partial exchange transfusions may be used as an alternative to hydroxyurea, but the interval between transfusions should be carefully managed to prevent episodes of acute chest syndrome 5.
• The use of opioids, such as morphine, should be carefully monitored to ensure efficacy, safety, and patient satisfaction, and alternative routes of administration, such as intranasal or transmucosal, may be considered for rapid onset of analgesia in the emergency department 6.