From the Guidelines
Sickle cell disease crises require prompt medical attention and a comprehensive management approach to reduce morbidity, mortality, and improve quality of life. The management of sickle cell disease crises involves a multifaceted approach, including aggressive pain management, hydration, oxygen supplementation, and prevention of complications such as acute chest syndrome, stroke, and splenic sequestration.
Key Interventions
- For acute pain crises, the mainstay of treatment includes aggressive pain management with opioids such as morphine (0.1-0.15 mg/kg IV every 3-4 hours), hydromorphone (0.015-0.02 mg/kg IV every 3-4 hours), or oral medications like oxycodone (5-10 mg every 4-6 hours) for less severe pain 1.
- Hydration is crucial, typically with IV fluids at 1.5 times maintenance rate.
- Oxygen supplementation should be provided if oxygen saturation falls below 95%.
- For acute chest syndrome, treatment includes antibiotics (ceftriaxone 1-2g IV daily plus azithromycin 500mg daily), incentive spirometry, and possibly blood transfusions 1.
- Stroke requires immediate exchange transfusion to reduce HbS levels below 30%.
- Splenic sequestration may need urgent transfusion and splenectomy consideration.
Prevention of Crises
- Prevention of crises involves hydroxyurea (15-35 mg/kg/day) to increase fetal hemoglobin production, regular hydration, avoiding triggers like extreme temperatures and high altitudes, and prompt treatment of infections 1.
- The use of hydroxyurea, L-glutamine, and crizanlizumab can help prevent recurrent acute pain events and reduce the risk of acute chest syndrome.
- Hematopoietic stem cell transplantation (HSCT) may be considered for patients with severe sickle cell disease, including those with frequent painful events or recurrent acute chest syndrome 1.
From the Research
Sickle Cell Disease Crises
- Sickle cell disease (SCD) is a chronic hematologic disease that affects over 100,000 people in the United States, with many patients presenting to the emergency department seeking treatment for an acute complication 2.
- Vaso-occlusive crisis is the most common recurring complication of SCD, and can be difficult to manage due to the stigma patients face surrounding management of their pain 2.
- Pain management in the emergency department should be well-defined and aggressive, with educational interventions playing a significant role in improving pain management for SCD patients 3.
Treatment Options
- Opioids are commonly used for treating severe pain in SCD, but their use can be associated with liabilities and organ-specific pathological effects 4.
- Morphine is a commonly used opioid for pain management in SCD, but its use should be carefully evaluated due to potential risks 4, 5.
- Ketorolac may be considered as a good alternative to IV morphine in the management of severe vaso-occlusive crisis in SCD, with similar efficacy and fewer side effects 5.
- Hydroxyurea therapy is recommended for patients with sickle cell anemia, as it can decrease the frequency of vaso-occlusive crises and acute chest syndrome with limited adverse effects 6.
Management Guidelines
- Children with sickle cell anemia should receive prophylactic penicillin from birth through at least five years of age, and all persons with SCD require vaccination to prevent invasive pneumococcal disease 6.
- Annual screening with transcranial Doppler ultrasonography is recommended for all children with SCD beginning at two years of age and continuing through adolescence to evaluate the risk of stroke and to initiate transfusion therapy in those at high risk 6.
- Vasoocclusive crises require immediate and adequate analgesia appropriate to the level of patient-reported pain, with antibiotics, hospitalization, and incentive spirometry indicated for those with acute chest syndrome 6.