From the Guidelines
Sepsis in sickle cell disease requires immediate recognition and aggressive management with broad-spectrum antibiotics within one hour of presentation, even before culture results are available. Patients with sickle cell disease who present with fever (≥38°C) should be treated as medical emergencies, as they are at high risk for septicemia and meningitis with Streptococcus pneumoniae and other encapsulated bacteria 1. The recommended empiric antibiotic regimen includes ceftriaxone, which is preferred due to its long half-life, allowing for outpatient management with close follow-up when necessary. Key considerations in management include:
- Prompt administration of broad-spectrum antibiotics, such as ceftriaxone sodium, within one hour of presentation
- Urgent CBC and reticulocyte counts, blood culture, and other testing as indicated, such as for infection with influenza or severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)
- Close monitoring for signs of clinical deterioration, including hypotension, increased respiratory rate, altered mental status, or decreasing oxygen saturation
- Fluid resuscitation with isotonic crystalloids (20-30 mL/kg) for hypotension or signs of poor perfusion, with careful monitoring to avoid excessive fluids
- Supplemental oxygen to maintain saturation above 95%
- Consideration of exchange transfusion in severe cases with multiorgan dysfunction. It is crucial to recognize that sickle cell patients with sepsis are at higher risk for acute chest syndrome, multiorgan failure, and death due to their functional asplenia and the vaso-occlusive nature of sickle cell disease, which can be exacerbated by infection-induced inflammation 1.
From the Research
Sepsis in Sickle Cell Disease
- Sepsis is a major concern in patients with sickle cell disease, as they are at increased risk of infection due to their compromised immune system 2.
- Infections in sickle cell disease can be bacterial, fungal, or viral, and treatment decisions are often based on consensus guidelines, clinical experience, or adapting treatment applied in other diseases, rather than randomized controlled clinical trials 2.
- Prevention of infection is crucial, and targeted prophylaxis and vaccination are key strategies in managing sickle cell disease 2.
- Patients with sickle cell disease may experience recurrent pain, fatigue, anemia, and increased infection susceptibility, and treatment options focus on managing symptoms and preventing complications 3.
- Hydroxyurea, a medication that increases the production of fetal hemoglobin, is commonly used to reduce the frequency and severity of pain crises, but its role in preventing sepsis is not well established 4, 3.
- Blood transfusions may be used to improve oxygen delivery and reduce the risk of complications, including sepsis, but the evidence for their effectiveness in this context is limited 5.
- Emergency department management of patients with sickle cell disease requires special consideration, as they may present with acute complications such as pain, infection, respiratory distress, stroke, or priapism, which can be difficult to manage 6.