Excess deaths among adults with sickle cell disease in 2020 compared to prior years.

In 2020, there was a 20% increase in excess deaths in the USA due to COVID infections but also to changes in the healthcare system due to the pandemic. We hypothesized that people living with sickle cell disease (SCD) may be vulnerable to these changes as SCD can lead to rapid decompensation. We examined all deaths of people with SCD at our center in 2020. Cause of death was determined, clinical variables, and healthcare utilization, and the presence of COVID infection, sepsis, and acute organ failure during the death event was obtained from the electronic medical record. Deaths in 2020 were compared to deaths in 2017-2019. In 2020, deaths increase 244% (22 vs 9), but acute or previous COVID infections were identified in only 36% of 2020 deaths. People who died in 2020 were more likely to have developed acute organ failure during the death event (70.6% vs 21.1%, p = 0.003) compared to prior years. They were also more likely to have a history of stroke and more frequent hematology clinic visits. Deaths in 2020 doubled compared to prior years and COVID infection could not account for all of this excess mortality. People who died in 2020 may have had more severe disease as suggested by having more clinic visits and higher rates of stroke and were more likely to develop organ failure during the death event. This demonstrates that people with SCD may be especially vulnerable to delays in care. Larger multicenter studies should be conducted to examine this further.

COVID19 vaccination in adults with sickle cell disease is not associated with increases in rates of pain crisis.

ABSTRACTPeople with sickle cell disease (SCD) are more vulnerable to hospitalization, pneumonia, and pain following COVID-19 infection. However, given the association between the inflammatory response and vaso-occlusive crises in SCD and a case report of vaso-occlusive crises following administration of the ChAdOx1 nCov-195-7/AstraZeneca vaccine, there is concern that the administration of COVID-19 vaccines in people with SCD might provoke a vaso-occlusive crisis. To address this critical gap in knowledge, we sought to examine acute care usage for vaso-occlusive crisis and frequency and severity of side effects following COVID-19 vaccination among patients at the Montefiore Sickle Cell Center for Adults. As part of regular care, patients were asked if they had received COVID-19 vaccination and any side effects were noted. Electronic medical records were reviewed for the type of vaccine, dates received, episodes of vaso-occlusive crises within seven days of a dose, and side effects noted. The risk of average hospital utilization per week in 2019 was calculated as a baseline. We found that fewer than 1 in 10 patients presented to the hospital within seven days of vaccination and that the risk of hospital utilization was similar to the average risk in a week in 2019. Of patients who reported side effects, one reported a possible case of sensorineural hearing loss otherwise no other rare side effects, including thrombosis or death, were reported.