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1.
Blood ; 136(Supplement 1):7-8, 2020.
Article in English | PMC | ID: covidwho-1338975

ABSTRACT

Sickle cell disease (SCD) is an inherited hemoglobinopathy that can affect nearly every organ system. Individuals living with SCD are at high risk of developing serious infections which can further trigger disease related complications and attribute additional morbidity and mortality. In light of the evolving pandemic caused by SARS-CoV-2, the causative agent of COVID-19 disease, and the potential for future infectious disease epidemics, it is important to understand the impact that COVID-19 has on hospitalization rates and mortality in this medically vulnerable population. The objective of this study was to describe hospitalization and case fatality rates secondary to COVID-19 among individuals living with SCD in different age groups and compare these to the general population.The Medical College of Wisconsin established the international SECURE-SCD Registry to collect data on pediatric and adult COVID-19 infections in individuals living with SCD. Providers are instructed to report confirmed COVID-19 cases to the registry after sufficient time has passed to observe the disease course through resolution of acute illness and/or death. For each case, providers complete a short form that includes the following data: patient demographics, COVID-19 related hospitalization, COVID-19 severity/management strategies, if the patient died due to COVID, and other information about SCD complications. Data are de-identified and without protected health information to facilitate rapid and increased reporting. We calculated the hospitalization rate and case fatality rate for individuals with SCD by specific age group and contrasted it with the rates publicly available for the general Black population. We utilized data from California Department of Public Health for case fatality rate comparison in Blacks and data from COVID-NET for hospitalization rate comparison. We used indirect age adjustment to calculate standardized mortality ratios using COVID-19 data from California state as the reference population.As of July 17th 2020, 218 cases of COVID-19 in Blacks with SCD in the US were reported to the registry. There was a slight predominance of females (52.8%) and 32.1% of reported cases were patients 18 years and under. There were 15 deaths reported with overall mortality rate of 6.9%. Figure 1 shows the distribution of cases and deaths by age group and gender. Mortality rate in SCD patients was highest in the 50-64 years age group (23.1%) in contrast to mortality rate peaks seen in the general population in patients older than 80 years (Table 1). Young adult SCD patients aged 18-34 years had a case fatality rate of 3.3% and those aged 34-50 years had a rate of 14.9%. California Department of Public Health report case fatality rates for Blacks are less than 1% in both of these comparative age groups. Age-standardized mortality ratio shows that individuals with SCD are 7.7 times more likely to die due to COVID-19 infection compared to the general population. The overall hospitalization rate in individuals with SCD was 72.5% and 18.8% of reported hospitalized cases were children. Among hospitalized adults with SCD, stratification by age showed that 85% were aged 18-49, whereas only 25.7% of people 18-49 years in the general Black population were hospitalized (Table 2).Our findings show that individuals with SCD who have COVID-19 infection have higher rates of death due to COVID-19 than the general Black population. Also, a large proportion of COVID hospitalization for the SCD population occurs among the younger age group. Further analysis is planned to examine effects of underlying comorbidities and prior SCD-associated complications on the severity of COVID-19 in individuals with SCD.

2.
Blood Adv ; 5(13): 2717-2724, 2021 07 13.
Article in English | MEDLINE | ID: covidwho-1291227

ABSTRACT

Patients with sickle cell disease (SCD) are at high risk of developing serious infections, therefore, understanding the impact that severe acute respiratory syndrome coronavirus 2 infection has on this population is important. We sought to identify factors associated with hospitalization and serious COVID-19 illness in children and adults with SCD.We established the international SECURE-SCD Registry to collect data on patients with SCD and COVID-19 illness. We used multivariable logistic models to estimate the independent effects of age, sex, genotype, hydroxyurea, and SCD-related and -nonrelated comorbidities on hospitalization, serious COVID-19 illness, and pain as a presenting symptom during COVID-19 illness. As of 23 March 2021, 750 COVID-19 illness cases in patients with SCD were reported to the registry. We identified history of pain (relative risk [RR], 2.15; P < .0001) and SCD heart/lung comorbidities (RR, 1.61; P = .0001) as risk factors for hospitalization in children. History of pain (RR, 1.78; P = .002) was also a risk factor for hospitalization in adults. Children with history of pain (RR, 3.09; P = .009), SCD heart/lung comorbidities (RR, 1.76; P = .03), and SCD renal comorbidities (RR, 3.67; P < .0001) and adults with history of pain (RR 1.94, P = .02) were at higher risk of developing serious COVID-19 illness. History of pain and SCD renal comorbidities also increased risk of pain during COVID-19 in children; history of pain, SCD heart/lung comorbidities, and female sex increased risk of pain during COVID-19 in adults. Hydroxyurea showed no effect on hospitalization and COVID-19 severity, but it lowered the risk of presenting with pain in adults during COVID-19.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Adult , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Child , Female , Hospitalization , Humans , Risk Factors , SARS-CoV-2
3.
J Med Internet Res ; 23(3): e22219, 2021 03 02.
Article in English | MEDLINE | ID: covidwho-1088863

ABSTRACT

Coincident with the tsunami of COVID-19-related publications, there has been a surge of studies using real-world data, including those obtained from the electronic health record (EHR). Unfortunately, several of these high-profile publications were retracted because of concerns regarding the soundness and quality of the studies and the EHR data they purported to analyze. These retractions highlight that although a small community of EHR informatics experts can readily identify strengths and flaws in EHR-derived studies, many medical editorial teams and otherwise sophisticated medical readers lack the framework to fully critically appraise these studies. In addition, conventional statistical analyses cannot overcome the need for an understanding of the opportunities and limitations of EHR-derived studies. We distill here from the broader informatics literature six key considerations that are crucial for appraising studies utilizing EHR data: data completeness, data collection and handling (eg, transformation), data type (ie, codified, textual), robustness of methods against EHR variability (within and across institutions, countries, and time), transparency of data and analytic code, and the multidisciplinary approach. These considerations will inform researchers, clinicians, and other stakeholders as to the recommended best practices in reviewing manuscripts, grants, and other outputs from EHR-data derived studies, and thereby promote and foster rigor, quality, and reliability of this rapidly growing field.


Subject(s)
COVID-19/epidemiology , Data Collection/methods , Electronic Health Records , Data Collection/standards , Humans , Peer Review, Research/standards , Publishing/standards , Reproducibility of Results , SARS-CoV-2/isolation & purification
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