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December 11, 2021
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Largest adult sickle cell center in US reports low rates of COVID-19 infection, mortality

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The diligence and awareness to stay home demonstrated by patients with sickle cell disease during the COVID-19 pandemic proved crucial in reducing morbidity and mortality, according to a study presented at ASH Annual Meeting and Exposition.

The findings from the Georgia Comprehensive Sickle Cell Center at Grady Health System database emphasized the crucial role of virtual health care visits during that time, and investigators recommended further use of such visits in sickle cell care.

Hospitalization among patients with COVID-19 and SCD.
Data derived from El Rassi F, et al. Abstract 3105. Presented at: ASH Annual Meeting and Exposition; Dec. 11-14, 2021; Atlanta.

“We wanted to understand the impact and outcomes of COVID-19 on our sickle cell population,” Fuad El Rassi, MD, director of sickle cell research at Georgia Comprehensive Sickle Cell Center at Grady Health System and associate professor of hematology and medical oncology at Emory University School of Medicine, told Healio.

As the largest adult sickle cell center in the U.S., Grady provided 3,077 sickle cell outpatient visits and 3,695 acute care visits in 2019 but faced a precipitous drop last year because of state regulations for lockdown during the pandemic.

Researchers sought to evaluate all COVID-19 cases at the center in 1 year.

Among 1,343 patients tracked within the clinical database, 55 contracted COVID-19. Among them, 28 were women, 64% had sickle cell anemia genotype, 31% had sickle hemoglobin-C genotype and 5% had sickle beta-thalassemia genotype.

More than a third of patients (35%) were on hydroxyurea for disease modification and 31% had elevated fetal hemoglobin, calculated as fetal hemoglobin above 5% by hemoglobin electrophoresis, researchers reported.

Nearly half of patients (47%) experienced episodes of chronic pain, defined as patient-reported daily pain episodes for more than 4 days a week in the last 3 months. Median daily morphine equivalents was 90 mg (range, 45-225).

Fuad El Rassi, MD
Fuad El Rassi

Results showed the rate of ED visits or hospitalizations among patients with sickle cell disease and COVID-19 was 80%, with 49% of the patient visits related to sickle cell vaso-occlusive events and 27% related to COVID-19 primarily. In addition, 20% of patients with sickle cell disease and COVID-19 were not seen in an emergency setting nor required hospitalization, researchers reported.

Further analysis of the cohort revealed 29% of patients had lung findings on imaging, with 16 patients requiring treatment with remdesivir (n = 9), dexamethasone (n = 8) and/or red cell products (n = 7). The most common signs and symptoms included pain (58%), cough and fever (40%), dyspnea (31%), and pneumonia with chest X-ray evidence (25%).

Among all 55 patients, two developed acute respiratory distress syndrome and were intubated, and two patients died. Both patients who died presented with COVID-19 infections in June and July of 2020.

The June 2020 patient presented with a vaso-occlusive event and was found to have bilateral lung opacities but was asymptomatic and discharged home before returning in less than a week with multiorgan failure (physicians attempted a red cell erythrocytapheresis). The July patient presented with COVID-19 pneumonia, was treated with remdesivir and convalescent plasma and developed multiorgan failure and acute respiratory distress syndrome.

Researchers noted that the two deaths occurred early on during the pandemic, from March to September 2020, when 20 of the 55 cases were diagnosed. In the 35 cases at the center from October 2020 through March 2021, zero deaths occurred as COVID hospitalizations decreased and better treatment became available.

Researchers credited virtual visits as an important step toward protecting patients during that period, which coincided with the second COVID-19 peak during the winter of 2021.

“Despite the fact that staying home has reduced unwanted outcomes, still a significant number of patients had COVID-19 and had to seek care; the bottom line is get vaccinated,” El Rassi said. “Additionally, having access to telemedicine visits for care of sickle cell disease has been crucial to continue the care of our patients.”