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COVID-19 outcomes in sickle cell disease and sickle cell trait
Jana Christian1, Sophie Lanzkron1, Rakhi P Naik1
1Division of Hematology, Department of Medicine, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Insights
Coronavirus Disease 2019 (COVID-19) significantly impacts individuals with Sickle Cell Disease (SCD), increasing hospitalizations. Caution is advised with corticosteroids, while hydroxyurea may offer protection against severe COVID-19 outcomes in SCD patients.
Area of Science:
- Hematology
- Infectious Diseases
- Public Health
Background:
- The Coronavirus Disease 2019 (COVID-19) pandemic raised concerns about its impact on individuals with Sickle Cell Disease (SCD) and Sickle Cell Trait (SCT).
- Patients with SCD experience higher rates of emergency department visits and hospitalizations due to COVID-19 compared to the general population.
- Vaso-occlusive crisis and acute chest syndrome are the primary clinical presentations of COVID-19 in SCD patients.
Purpose of the Study:
- To investigate the effects of COVID-19 on individuals with SCD and SCT.
- To identify risk factors associated with severe COVID-19 illness in patients with SCD.
- To evaluate the potential influence of SCT on COVID-19 outcomes.
Main Methods:
- Review of existing literature and clinical data regarding COVID-19 in SCD and SCT populations.
- Analysis of hospitalization rates, clinical presentations, and risk factors for severe disease.
- Assessment of the impact of specific treatments, such as corticosteroids and hydroxyurea.
Main Results:
- SCD patients exhibit significantly higher rates of emergency department utilization and hospitalization for COVID-19.
- Key risk factors for severe COVID-19 in SCD include older age, frequent pain-related acute care visits, hemoglobin SC disease, and pre-existing end-organ damage.
- Corticosteroid use in SCD patients with COVID-19 is linked to severe vaso-occlusive crisis, necessitating cautious use and potential prophylactic transfusions.
- Hydroxyurea may confer a protective effect against severe COVID-19 in this population.
Conclusions:
- COVID-19 poses a significant threat to individuals with SCD, leading to increased severe outcomes.
- Careful consideration of treatment strategies, particularly corticosteroid use, is crucial for managing COVID-19 in SCD patients.
- Hydroxyurea shows promise as a potential protective agent against severe COVID-19 in the context of SCD.
Abstract:
Throughout the Coronavirus Disease 2019 (COVID-19) pandemic, understanding the effects of COVID-19 on persons with Sickle Cell Disease (SCD) and Sickle Cell Trait (SCT) has garnered interest. Patients with SCD diagnosed with COVID-19 utilize the emergency department and are hospitalized at significantly higher rates compared to the general population, with vaso-occlusive crisis and acute chest syndrome as the leading presentations. Whether SCD alone increases the likelihood of severe COVID-19 illness remains uncertain; however, potential risk factors for severe disease among patients with SCD include older age, frequent acute care visits for pain, haemoglobin SC disease, and pre-existing end-organ disease. SCT status may also influence COVID-19 outcomes, particularly among those with pre-existing co-morbidities. Corticosteroids in patients with SCD and COVID-19 should be used with extreme caution given strong associations between corticosteroid exposure and severe vaso-occlusive crisis, with prophylactic transfusion administered if corticosteroids are deemed necessary. Hydroxyurea may be protective in COVID-19.