Coronavirus Disease Among Persons With Sickle Cell Disease, United States, March 20–May 21, 2020

Julie A. Panepinto; Amanda Brandow; Lana Mucalo; Fouza Yusuf; Ashima Singh; Bradley Taylor; Katherine Woods; Amanda B. Payne; Georgina Peacock; Laura A. Schieve

Disclosures

Emerging Infectious Diseases. 2020;26(10):2473-2476. 

In This Article

The Study

The Medical College of Wisconsin established the SECURE-SCD Registry to collect data on COVID-19 cases occurring globally in persons living with SCD. A link to the registry (https://covidsicklecell.org) was distributed to healthcare providers caring for patients with SCD by medical professional and patient advocacy networks and was made available on the Centers for Disease Control and Prevention website. Providers were asked to report all confirmed COVID-19 cases among patients with SCD to this registry; they were specifically asked to report only confirmed COVID-19 cases and to report cases after resolution of acute illness or death. Persons who had the sickle cell trait were not included in this registry, nor were persons with SCD who had suspected but not confirmed COVID-19. Providers were asked to report if the patient died of COVID-19 or complications of COVID-19. All data were deidentified without protected health information.

For each case, providers were asked to complete a short form with questions on demographics; SCD genotype; SCD-related health history; and COVID-19 clinical course, severity, and interventions. In addition to data on COVID-19 clinical severity indicators, such as hospitalization, ICU admission, and death, COVID-19 severity level based on patient manifestations were collected by using established criteria for asymptomatic, mild, moderate, severe, and critical[10] (Table).

This analysis was limited to cases among persons with SCD living in the United States reported during March 20–May 21, 2020. We describe the reported cases and deaths caused by COVID-19 and provide 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 HbSβ0-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. 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.

A total of 13 (7%) patients died (Table). Their mean age was 38.5 years, and >90% were adults. Nearly 40% of deaths were among persons who had genotypes generally associated with milder SCD (types HbSC or HbSβ+-thalassemia). Patients who died had high proportions of frequent pain episodes in the previous 3 years (85%), pulmonary hypertension (39%), decreased renal function (23%), SCD nephropathy (15%), and overt stroke (31%). Of the 13 deaths, 8 were among persons who had severe or critical COVID-19; 5 deaths were observed in persons who had mild or moderate COVID-19.

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