scholarly journals COVID-19 Outcomes in Individuals with Sickle Cell Disease and Sickle Cell Trait Compared to Blacks without Sickle Cell Disease or Trait

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 54-56 ◽  
Author(s):  
Ashima Singh ◽  
Amanda M. Brandow ◽  
Julie Panepinto
Keyword(s):  
United States ◽  
Type 1 Diabetes ◽  
Sickle Cell Disease ◽  
Propensity Score ◽  
Sickle Cell ◽  
Research Funding ◽  
Sickle Cell Trait ◽  
The United States ◽  
Cell Disease

Introduction: By August 1, 2020 in the United States, more than 3 million cases of Coronavirus disease 2019 (COVID-19) had been reported with more than 150,000 deaths due to this disease. Growing evidence suggests that individuals with the pre-existing conditions of hypertension, diabetes, cardiovascular disease and obesity are at a higher risk of more serious COVID-19 illness. However, the impact of COVID-19 on individuals with sickle cell disease and sickle cell trait as compared to those without sickle cell disease or trait is not known. The objective of this study was to determine the rate of hospitalization, disease symptoms and deaths due to COVID-19, in patients with sickle cell disease and sickle cell trait compared to Blacks without sickle cell disease or trait. Methods: We leveraged existing electronic health record (EHR) data from multiple sites that contribute data to a research network, TriNetX. TriNetX query platform was used to identify patients with COVID-19 infection based on ICD diagnoses codes or a positive COVID-19 result from a nucleic acid amplification with probe-based detection test, present any time after January 20, 2020 (this is when the first COVID-19 case was detected in the United States) within the patients' EHR data. We report rates of specific COVID-19 related outcomes among individuals with sickle cell disease and trait, calculated as % of patients in cohort with the particular outcome. Our outcomes of interest included COVD-19 related symptoms, hospitalization, and death, which occurred within 2 weeks of COVID diagnosis. We used propensity score matching (greedy nearest-neighbor matching algorithm with a caliper of 0.1 pooled standard deviations) to create balanced cohorts for comparing outcomes between individuals with sickle cell disease or trait and Blacks without sickle cell disease or trait. Risk ratios and risk differences are reported along with 95% confidence intervals. Given multiple outcomes of interest, we considered a more stringent two-sided alpha of less than <0.01, based on a z-test, to determine statistical significance for differences in outcome rates. Results: As of July 15, 2020, there were 122 COVID-19 patients who had sickle cell disease and 172 COVID-19 patients who had sickle cell trait. Our comparator groups included 15,762 Blacks who were diagnosed with COVID-19 but did not have sickle cell trait/disease. COVID-19 patients with sickle cell disease were significantly younger and a higher proportion had asthma, type 1 diabetes and pre-existing liver conditions compared to Blacks without sickle cell trait/disease (Table 1). COVID-19 patients with sickle cell trait were significantly younger, a higher proportion were females, overweight/obese, and a higher proportion had asthma or type 1 diabetes compared to Blacks without sickle cell trait/disease (Table 1). The rate of respective outcomes for the three groups is shown in Figure 1. Propensity score matching yielded a cohort of patients such that there were no significant differences in demographic and clinical characteristics between patients with sickle cell disease/trait compared to Blacks without sickle cell trait/disease. After matching, COVID patients with sickle cell disease remained at a higher risk of hospitalization, pneumonia and pain compared to Blacks without sickle cell trait/disease (Table 2). The case fatality rates were not significantly different between those with sickle cell disease compared to Blacks. There were no significant differences in COVID outcomes between sickle cell trait and Blacks without sickle cell trait/disease, within the matched cohort. Conclusions: These data provide evidence that sickle cell disease imposes additional risk of severe COVID-19 illness and hospitalization, after balancing for age, gender and other preexisting conditions. The death rate between sickle cell disease and Blacks without sickle cell trait/disease was not significantly different. There are no significant differences in COVID-19 outcomes between sickle cell trait and Blacks without sickle cell trait/disease, after balancing for age, gender and other pre-existing conditions. Disclosures Brandow: NIH / NHLBI: Research Funding; Greater Milwaukee Foundation: Research Funding. Panepinto:HRSA: Research Funding; NINDS: Research Funding; NINDS: Research Funding; NHLBI: Research Funding.

scholarly journals Intravenous Fluid Boluses Are Commonly Administered to Adults with Sickle Cell Disease and Vaso-Occlusive Pain

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4839-4839 ◽  
Author(s):  
Marcus A. Carden ◽  
Paula Tanabe ◽  
Jeffrey A. Glassberg
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Clinical Outcomes ◽  
Sickle Cell ◽  
Research Funding ◽  
The United States ◽  
Adult Patients ◽  
Fluid Type ◽  
Cell Disease ◽  
Oral Hydration

Background: Sickle cell disease (SCD) affects 100,000 adults and children in the United States (USA). Painful, uncomplicated vaso-occlusive episodes (VOE) are the most common complication of SCD and the #1 reason adults with SCD seek medical attention in the ED. The NHLBI guidelines suggest encouraging oral hydration, along with analgesics, during VOE but to give intravenous (IV) fluids only if the patient cannot drink. However, there exists a clinical equipoise regarding the choice of IV fluids to use during the treatment of VOE in the ED, along with the rate or volume given. Despite lack of guidelines or evidence, the use of large IV volumes of crystalloids given over short periods of time (i.e. bolus) in the ED is common practice and we recently showed normal saline (NS) boluses in particular may be associated with inferior pain control and higher admission rates in pediatric patients with VOE (Carden etal., Am J Hem, 2019). Importantly, investigations into the use and impact these different IV fluids, including IV boluses, and how they may impact clinical outcomes among adult patients with SCD and VOE who present to the ED are lacking. Methods: We conducted a cross-sectional survey of emergency providers at the 2011 annual American College of Emergency Physicians Scientific Assembly, where ED providers from across the United States attend, regarding IV fluid practices during VOE. We specifically used a validated instrument to assess self-reported practices toward patients with SCD regarding IV fluid use, including volumes and rates, during uncomplicated VOE. Basic demographic information was obtained and providers were specifically asked: "Please indicate which type of fluids and rate of administration you give to patients with acute sickle cell pain who are not hypotensive and not severely hypovolemic". Providers responded never, rarely, frequently or always to each fluid type and rate. Results: Of 795 respondents to the survey, 722 indicated they took care of patients with SCD, 669 completely responded to the survey, and of those, 244 respondents only took care of adult patients with SCD. Demographic and experience with SCD patients, as well as preferred fluid type and rate of administration is reported in Table 1. IV fluid use during uncomplicated VOE varied among adult providers, but 83% of providers surveyed used IV fluid crystalloid boluses during VOE. Only 45% of providers recommended oral hydration during VOE among adults. Conclusions: Among adults ED providers who care for patients with SCD in the USA, wide variations in practice utilizing IV fluids are common. Despite no guidelines, IV fluid boluses are commonly given, as was seen in pediatric ED studies, and oral hydration is less commonly recommended among adult ED providers. Further investigation is needed to determine if these practices have an impact on clinical outcomes among euvolemic adult patients with SCD and VOE who present to the ED. Disclosures Carden: GBT: Honoraria; NIH: Research Funding. Tanabe:NIH: Research Funding; AHRQ: Research Funding. Glassberg:ACEP: Research Funding; NHLBI: Research Funding; Pfizer: Research Funding.

Red cell storage age policy for patients with sickle cell disease: A survey of transfusion service directors in the United States

2016 ◽  
Vol 54 (1) ◽  
pp. 158-162 ◽  
Author(s):  
Matthew S. Karafin ◽  
Arun K. Singavi ◽  
Mehraboon S. Irani ◽  
Kathleen E. Puca ◽  
Lisa Baumann Kreuziger ◽  
...  
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Red Cell ◽  
Cell Disease ◽  
Cell Storage

scholarly journals The Economic Burden of Sickle Cell Disease in the United States

2018 ◽  
Vol 21 ◽  
pp. S108 ◽  
Author(s):  
J Huo ◽  
H Xiao ◽  
M Garg ◽  
C Shah ◽  
DJ Wilkie ◽  
...  
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Economic Burden ◽  
The United States ◽  
Cell Disease

Childhood Hearing Loss in Patients With Sickle Cell Disease in the United States

2019 ◽  
Vol 41 (2) ◽  
pp. 124-128 ◽  
Author(s):  
Heather K. Schopper ◽  
Christopher F. D’Esposito ◽  
John S. Muus ◽  
Julie Kanter ◽  
Ted A. Meyer
Keyword(s):  
United States ◽  
Hearing Loss ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Cell Disease ◽  
Childhood Hearing Loss

scholarly journals Liver Transplantation in Patients with Sickle Cell Disease in the United States

2020 ◽  
Vol 255 ◽  
pp. 23-32 ◽  
Author(s):  
Rachel Hogen ◽  
Michelle Kim ◽  
Yelim Lee ◽  
Mary Lo ◽  
Navpreet Kaur ◽  
...  
Keyword(s):  
United States ◽  
Liver Transplantation ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Cell Disease

scholarly journals Trends in Sickle Cell Disease–Related Mortality in the United States, 1979 to 2017

2020 ◽  
Vol 76 (3) ◽  
pp. S28-S36
Author(s):  
Amanda B. Payne ◽  
Jason M. Mehal ◽  
Christina Chapman ◽  
Dana L. Haberling ◽  
Lisa C. Richardson ◽  
...  
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Cell Disease ◽  
Related Mortality

scholarly journals Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects

2020 ◽  
Vol 4 (16) ◽  
pp. 3804-3813
Author(s):  
Julie Kanter ◽  
Wally R. Smith ◽  
Payal C. Desai ◽  
Marsha Treadwell ◽  
Biree Andemariam ◽  
...  
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Access To Care ◽  
Sickle Cell ◽  
The United States ◽  
Models Of Care ◽  
System Level ◽  
Cell Disease ◽  
Blood Disorder ◽  
Essential Components

Abstract Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD survive into adulthood. However, access to adult sickle cell care is poor in many parts of the United States, resulting in increased acute care utilization, disjointed care delivery, and early mortality for patients. A dearth of nonmalignant hematology providers, the lack of a national SCD registry, and the absence of a centralized infrastructure to facilitate comparative quality assessment compounds these issues. As part of a workshop designed to train health care professionals in the skills necessary to establish clinical centers focused on the management of adults living with SCD, we defined an SCD center, elucidated required elements of a comprehensive adult SCD center, and discussed different models of care. There are also important economic impacts of these centers at an institutional and health system level. As more clinicians are trained in providing adult-focused SCD care, center designation will enhance the ability to undertake quality improvement and compare outcomes between SCD centers. Activities will include an assessment of the clinical effectiveness of expanded access to care, the implementation of SCD guidelines, and the efficacy of newly approved targeted medications. Details of this effort are provided.

The Number of People with Sickle Cell Disease in the United States: National and Individual State Estimates.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1365-1365
Author(s):  
David C Brousseau ◽  
Julie A. Panepinto ◽  
Mark Nimmer ◽  
Raymond G Hoffmann
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Cell Population ◽  
Sickle Cell ◽  
The United States ◽  
Individual State ◽  
Cell Disease ◽  
Hispanic Children ◽  
Live Births ◽  
Race Ethnicity

Abstract Abstract 1365 Poster Board I-387 Background Although sickle cell disease is a genetic disease diagnosed by state newborn screening programs, it is not a reportable condition. Therefore it is difficult to ascertain the actual number of affected individuals living with sickle cell disease in the United States. One NIH estimate puts the number between 50,000 and 75,000 while the Sickle Cell Disease Association of America estimates the number to be “over 70,000”. Without accurate estimates, clinicians, health services researchers, and policy makers are all working with incomplete information when determining the extent of the cost and health consequences of sickle cell disease. Our objective was to estimate the number of people with sickle cell disease for the United States as a whole and for each individual State, adjusting for the increased mortality of sickle cell disease. Methods Census estimates by age and race/ethnicity were obtained for both the United States as a whole and for each individual State from the US Census website. The prevalence of sickle cell disease for blacks was uniformly applied to the U.S. and individual states using a rate of 289 black children with sickle cell disease per 100,000 live births. Based on previously published prevalence rates for Hispanics, prevalence rates of 89.8 Hispanic children of non-Mexican ancestry with sickle cell disease per 100,000 live births and 3.14 Hispanic children of Mexican ancestry with sickle cell disease per 100,000 live births were calculated. We did not include sickle cell disease for whites or Asians in our estimate. Year 2005 was used as the baseline year for all calculations. Consistent with previous literature, at age 0, 60% of children with sickle cell disease were classified as having HgbSS/SB0 and 40% of children were classified as having HgbSC/SB+. These proportions were altered towards higher proportions of HbSC/SB+ with increasing age based on the increased mortality of the more severe forms of sickle cell disease. To adjust for mortality, we analyzed the data based on age and sickle cell type, and used published mortality data for different sickle cell genotypes to calculate survival of the population to a given age. A population multiplier was then used to adjust population estimates for the difference in population across age groups and differences in population patterns by race/ethnicity. Results Analysis revealed an estimate of 89,079 (95% CI: 88,494 – 89,664) people with sickle cell disease in the United States, of which 80,151 are black and 8,928 Hispanic. The South, with a sickle cell population of 47,354 people, comprised more than 53% of all people with sickle cell disease in the United States. The five states with the highest estimated number of people with sickle cell disease were New York with 8,308; Florida with 7,539; Texas with 6,765; California with 6,474; and Georgia with 5,890. These five states comprised more than 43% of the total sickle cell population for the nation. Finally, the increased mortality for HgbSS/SB0 leads to an alteration in the relative percentages of sickle cell genotypes, with HgbSS/SB0 comprising 60% of people with sickle cell disease at birth, half of the sickle cell population at slightly over 30 years old, and only 25% of the sickle cell population by 60 years old. Conclusion This study reveals that the sickle cell population in the United States is higher than previously reported, with almost 90,000 people with sickle cell disease. In addition, differential mortality increases the percentage of people with HbSC/SB+, such that after 40 years of age they represent the majority of the sickle cell population. The population estimates for the country as well as the individual states provide important information with regard to allocation of resources for this chronic disease which primarily affects lower income, underserved individuals. Disclosures No relevant conflicts of interest to declare.

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