Severe complications from COVID-19 in U.S. children and adolescents with sickle cell disease
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BACKGROUND: Sickle Cell Disease (SCD) is the most common inherited blood disorder in the United States, causing red blood cells to take on a rigid “sickle” shape, leading to chronic hemolytic anemia and occlusion of blood vessels. SCD results in repeated vascular injury and inflammation, reduced immune functioning, and causes acute complications such as pain crises and Acute Chest Syndrome, as well as progressive damage to every organ system of the body. Given the tendency of hypoxia and other stressors to trigger sickling and SCD complications, it is likely that young individuals with SCD are at increased risk during COVID-19 illness. OBJECTIVES: To characterize complications of SARS-CoV-2 infection in children and adolescents with Sickle Cell Disease, and to determine if children with SCD have worse clinical outcomes than other Black and African American patients without SCD. METHODS: Surveillance for COVID-19 related complications was conducted across the United States from March 15, 2020 to January 31, 2021 as part of the CDC’s Overcoming COVID-19 public health registry. Hospitalized patients < 21 years of age, with positive SARS-CoV-2 test (reverse-transcriptase polymerase chain reaction ([RT-PCR]) and/or antibody test) or recent exposure without an alternate diagnosis were included in the registry. Clinicians reported data on patient demographics, baseline health status, clinical course, and outcomes in a standardized report form. A case series of patients with Sickle Cell Disease was identified in the registry, and illness and outcomes of Black and African American children and adolescents with and without SCD were analyzed in order to characterize disease course, complications, and outcomes in this population. RESULTS: Of 1,996 patients from 62 sites, 585 were Black or African American (54% male, median age 9.6 years), and 47 patients were reported with an underlying diagnosis of Sickle Cell Disease (45% male, median age 14.2 years). 43 (91%) of these patients were Black or African American, which was much higher than expected from the CDC’s estimate that 1 in 365 African American children have SCD. SCD patients came from 22 hospitals in 17 states, and were admitted between March 30 and December 8, 2020. Median hospital stay was 5 days (IQR 2.5-8), whereas for non-SCD Black patients it was 6 days (3-10). Overall, 65% (28/43) of Black SCD patients had at least one additional underlying condition, the most common of which was asthma (n=17, 40%). In comparison, 48% (262/542) of Black patients without SCD reported no underlying conditions, and 45% (n=245) reported two or more, of which obesity was the most common (n=99, 24%). The majority of patients with SCD had acute COVID-19, and only 19% (n=8) of patients with SCD were diagnosed with multisystem inflammatory syndrome in children (MIS-C), which is presumed to be a post-infectious complication, whereas 54% (n=285) of the comparison group had MIS-C. Overall, 35% (n=15) of the SCD patients were admitted to the ICU, compared to 67% (n=362) of patients without SCD. Median length of ICU stay was 4 days (2-7) in both the SCD and non-SCD groups. There were no deaths reported in patients with SCD, although one patient was transferred to a different hospital for a lung transplant, and death was rare in Black patients without SCD (n=9, 1.7%) w. Additionally, 30 patients (64%) presented with an SCD-related complication, including Acute Chest Syndrome (n=15, 32%) and vaso-occlusive pain crises (n=19, 40%). CONCLUSIONS: As a whole, Black children and adolescents with SCD did not display clinical outcomes more severe than Black children without SCD, however they were hospitalized at approximately 27 times the expected rate, suggesting that SCD may be a risk factor for hospitalization with COVID-19. COVID-19 may trigger SCD Complications, and some very severe cases show that SCD may be a risk factor for COVID-19 complications as well.
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