Sickle cell disease (SCD) is a genetic disorder of hemoglobin and afflicts ~110,000 African-Americans in the US. Because of its complex pathophysiology through chronic hemolytic anemia, microvascularocclusion, and a chronic inflammatory state, it affects multiple organ systems and leads to significant morbidity and organ damage as well as leads to frequent hospitalizations and health care encounters. During the past 35 years, primarily through research and patient care conducted by the NIH funded Comprehensive Sickle Cell Centers and some pivotal clinical trials, the life expectancy of patients with SCD has increased from the teens to mid- to late forties. This is still considerably shorter than that of African-Americans who do not have sickle cell disease and can be viewed as a major disparity even in this underserved minority population. While significant advances have been made in the understanding of the disease pathophysiology and in novel therapies through basic and translational research, these advances have been slow to be taken to clinical practice. The Southeastern Exploratory Sickle Cell Center of Excellence seeks to improve the care and quality of life of the SCD patient population by i) investigating the basic mechanism of action of a highly successful and effective hemoglobin F inducing drug, hydroxyurea, ii) identifying genetic variations underlying the frequency of pain, response to narcotics, and thus addressing the important issue of biologic/genetic bases of pain and its under treatment leading to the stigmatization of many SCD patients and its resulting disparity, iii) investigating the medical, social, and economic reasons for underutilization of hydroxyurea in SCD, iv) training primary care physicians with evidence based medicine in the care of patients with SCD, given the sobering reality that there will not be enough specialists in non-malignant hematology to meet the needs of the growing adult SCD population, and v) implementing innovative methods and concepts for the care of SCD patients in the ED and for transitioning from pediatric to adult care. Relieving the health disparity of SCD patients is the primary goal of this application.

Public Health Relevance

Sickle cell disease in the United States primarily affects African-Americans and is considered an orphan disease. Because of the lack of knowledge of health care providers in the management and treatment of painful episodes and other complications of this disease, patients are most often undertreated or not treated at all, which has created an enormous health disparity for these patients.

Agency
National Institute of Health (NIH)
Institute
National Institute on Minority Health and Health Disparities (NIMHD)
Type
Exploratory Grants (P20)
Project #
1P20MD003383-01
Application #
7668810
Study Section
Special Emphasis Panel (ZRG1-EMNR-L (52))
Program Officer
Tabor, Derrick C
Project Start
2009-05-28
Project End
2013-12-31
Budget Start
2009-05-28
Budget End
2009-12-31
Support Year
1
Fiscal Year
2009
Total Cost
$1,418,512
Indirect Cost
Name
Georgia Regents University
Department
Internal Medicine/Medicine
Type
Schools of Medicine
DUNS #
966668691
City
Augusta
State
GA
Country
United States
Zip Code
30912
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Anea, Ciprian B; Lyon, Matthew; Lee, Itia A et al. (2016) Pulmonary platelet thrombi and vascular pathology in acute chest syndrome in patients with sickle cell disease. Am J Hematol 91:173-8
Ikuta, Tohru; Sellak, Hassan; Odo, Nadine et al. (2016) Nitric Oxide-cGMP Signaling Stimulates Erythropoiesis through Multiple Lineage-Specific Transcription Factors: Clinical Implications and a Novel Target for Erythropoiesis. PLoS One 11:e0144561
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Ghoshal, Pushpankur; Rajendran, Mythilypriya; Odo, Nadine et al. (2014) Glycosylation inhibitors efficiently inhibit P-selectin-mediated cell adhesion to endothelial cells. PLoS One 9:e99363
Ikuta, Tohru; Kuroyanagi, Yuichi; Odo, Nadine et al. (2013) A common signaling pathway is activated in erythroid cells expressing high levels of fetal hemoglobin: a potential role for cAMP-elevating agents in ?-globin disorders. J Blood Med 4:149-59

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