scholarly journals Academic Performance of Children With Sickle Cell Disease in the United States: A Meta-Analysis

2021 ◽  
Vol 12 ◽  
Author(s):  
Andrew M. Heitzer ◽  
Latacha Hamilton ◽  
Claire Stafford ◽  
Jeffrey Gossett ◽  
Lara Ouellette ◽  
...  
Keyword(s):  
United States ◽  
Academic Performance ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Grade Retention ◽  
The United States ◽  
Cell Disease ◽  
Math Scores ◽  
Reading And Math ◽  
And Mathematics

Background: Students with sickle cell disease are at risk for poor academic performance due to the combined and/or interactive effects of environmental, psychosocial, and disease-specific factors. Poor academic performance has significant social and health consequences.Objective: To study academic achievement and attainment in children with sickle cell disease in the United States.Design: Medline, Embase, SCOPUS, CINAHL, ERIC, and PsycINFO were searched for peer-reviewed articles. Studies of children (ages 5–18) diagnosed with sickle cell disease of any genotype reporting academic achievement (standardized tests of reading, math, and spelling) or attainment (grade retention or special education) outcomes were included. Outcomes were analyzed using a random effects model. Achievement scores were compared to within study controls or normative expectations. Prevalence of grade retention and special education services were compared to national (United States) estimates for Black students. Age at assessment and overall IQ were evaluated separately for association with reading and mathematics scores. Subgroup analyses of reading and math scores were analyzed by cerebral infarct status (no cerebrovascular accident, silent infarct, stroke).Results: There were 44 eligible studies. Students with sickle cell disease scored 0.70, 0.87, and 0.80 (p < 0.001) SD below normative expectations on measures of reading, mathematics, and spelling, respectively. Compared to unaffected sibling and/or healthy controls (k = 8, n = 508), reading and math scores were 0.40 (p = 0.017) and 0.36 (p = 0.033) SD below expectations. Grade retention was approximately 10 times higher in students with sickle cell disease than Black students nationally. Intellectual functioning explained 97.3 and 85.8% of the variance in reading and mathematics performance, respectively (p < 0.001). Subgroup analyses revealed significant differences in reading (p = 0.034) and mathematics (p < 0.001) based on infarct status, with lower performance associated with presence of a silent infarct or stroke.Conclusion: Students with sickle cell disease demonstrate notable academic difficulties and are at high risk for grade retainment. Development of academic interventions and increased access to school support services are needed for this vulnerable population.Systematic Review Registration:https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42020179062.

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.

scholarly journals National Trends in Hospitalizations for Sickle Cell Disease in the United States Following the FDA Approval of Hydroxyurea, 1998–2008

Medical Care ◽  
2014 ◽  
Vol 52 (7) ◽  
pp. 612-618 ◽  
Author(s):  
Maureen M. Okam ◽  
Shimon Shaykevich ◽  
Benjamin L. Ebert ◽  
Alan M. Zaslavsky ◽  
John Z. Ayanian
Keyword(s):  
United States ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The United States ◽  
Cell Disease ◽  
Fda Approval ◽  
National Trends
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