Coronavirus Disease among Persons with Sickle Cell Disease, United States, March 20–May 21, 2020

Sickle cell disease (SCD) disproportionately affects Black or African American persons in the United States and can cause multisystem organ damage and reduced lifespan. Among 178 persons with SCD in the United States who were reported to an SCD–coronavirus disease case registry, 122 (69%) were hospitalized and 13 (7%) died.

case-fatality rates. Given uncertainty in the sample representativeness and ongoing data reporting to the SECURE-SCD Registry, we view these data as a hypothesis-generating case series. Thus, we did not use statistical tests to assess the significance of differences in mortality rates by subgroup.
As of May 21, 2020, a total of 178 COVID-19 cases were reported to the SECURE-SCD Registry among persons living in 22 states. The mean age of these case-patients was 28.6 years; 57% were female, and 80% were Black (Table). A total of 76% of case-patients had sickle cell types HbSS or HbSβ 0 -thalassemia, which is consistent with estimates of SCD genotype distribution in the United States (11). Recent (within the past 3 years) adverse events indicative of vasoocclusive crises were common among case-patients; 54% reported >3 pain episodes requiring hospitalization and 32% reported >1 acute chest syndrome episodes. ‡SCD genotypes are HbSS (homozygous for hemoglobin S, a severe phenotype associated with shortest survival); HbS 0 -thalassemia (compound heterozygous for hemoglobin S and  0 -thalassemia, clinically indistinguishable from HbSS); HbSC (heterozygous for hemoglobin S and hemoglobin C, usually moderate clinical severity); HbS+-thalassemia (heterozygous for hemoglobin S and reduced amounts of -globin, usually milder severity).
§Acute chest syndrome is a multicausal pneumonia-like illness. ¶COVID-19 severity level classified as asymptomatic, no clinical signs or symptoms during the positive COVID-19 period; mild, symptoms of acute upper respiratory tract infection, including fever, fatigue, myalgia, cough, sore throat, runny nose, and sneezing or gastrointestinal symptoms or digestive symptoms, such as nausea, vomiting, abdominal pain and diarrhea; moderate, pneumonia, with or without clinical symptoms, no hypoxia; severe, early respiratory symptoms or gastrointestinal symptoms followed by dyspnea and hypoxia (O2 saturations <92%); critical, acute respiratory distress syndrome, respiratory failure, encephalopathy, shock, coagulopathy, and multiorgan impairment (lung, heart, kidney, brain) that might be life threatening.
Prevalence of pulmonary hypertension, previous stroke, renal disease, and use of chronic transfusion therapy were all >10%.
A total of 6% of COVID-19 case-patients were asymptomatic, 54% had mild disease severity, 18% had moderate disease severity, 17% had severe disease severity, and 5% had critical disease severity. Nearly 90% of case-patients accessed care through an emergency department, 69% were hospitalized, 11% were admitted to an ICU, 6% required a ventilator, 38% required a transfusion, and 2% required dialysis.

Conclusions
Our findings suggest that persons who have SCD and become infected with SARS-CoV-2 have a high risk for a severe disease course and a high case-fatality rate. Among confirmed COVID-19 case-patients reported to the registry, the 69% hospitalization rate, 11% ICU admission rate, and 7% mortality rate are alarming, given that the mean patient age was <40 years. Comparison to a previous report of COVID-19 in the general US population indicates that hospitalization, ICU admission, and case-fatality rates for persons with SCD could be much higher than persons of similar ages in the US population-at-large (12). For example, COVID-19 case-fatality rates were reported as <1% for persons 20-44 years of age and for persons 45-54 years of age in the population-at-large (12).
These data should be considered in the context that these cases may not be representative of all COVID-19 cases among persons with SCD. There may be bias toward more severe cases in this registry; however, providers were asked to report all COVID-19 cases among their SCD patients. Also, whereas providers were specifically instructed to report only confirmed COVID-19 cases, further guidance was not provided on case confirmation, nor were laboratory testing results included in the registry; thus, we cannot rule out the possibility that a small number of suspected cases were erroneously reported.
Our findings are consistent with expectations based on SCD pathophysiology. SCD can cause multisystem organ damage, life-long disability, and reduced lifespan. Nonetheless, in this case series, COVID-19 deaths occurred in persons who had severe and mild-to-moderate SCD genotypes. Also, deaths occurred in COVID-19 case-patients classified as having mild-to-moderate disease severity. We did not have data to assess whether this finding was caused in part by an impact of SARS-CoV-2 exacerbating preexisting cardiac or SCD concurrent conditions; further study is needed.
Persons with SCD face socioeconomic and healthcare access disparities that might compound their already high risk for severe COVID-19 because of their underlying disease and concurrent conditions. SCD complications might negatively impact educational achievement and employment (13,14). Accessing appropriate healthcare is difficult given the lack of providers with SCD expertise. SCD patients might delay seeking care, and emergency department visits are high among this population that is placed at increased risk for poor health (14).
Our findings underscore the need to consider the unique circumstances faced by high-risk subgroups. SCD is one of many possible explanations for higher rates of illness and mortality from COVID-19 among Black populations in the United States. As with all high-risk groups, staying home, social distancing, and hand hygiene are necessary for persons with SCD (15), along with prompt care-seeking if COVID-19 is suspected or SCD complications arise. In addition, specific socioeconomic and healthcare access challenges that many persons with SCD face (i.e., social determinants of health) need to be considered in implementing prevention measures.