Sickle cell tests are used to help diagnose sickle cell anemia (also called sickle cell disease) and to identify those who may have sickle cell trait. Sickle cell anemia is an inherited disorder that leads to the production of an abnormal hemoglobin called hemoglobin S (Hb S or Hgb S). Sickle cell tests determine the presence and relative amount of hemoglobin S in a blood sample. Hemoglobin is a protein found in red blood cells (RBCs) that binds to oxygen in the lungs and carries it to tissues throughout the body. Typically, hemoglobin A (Hb A, adult hemoglobin) makes up most of the hemoglobin found in normal RBCs in adults, with small amounts of hemoglobin A2 and hemoglobin F. Before babies are born, they normally produce large amounts of hemoglobin F (Hb F, fetal hemoglobin), which is then replaced by Hb A as the predominant hemoglobin shortly after birth. Abstract source

Dr. Ataga's clinical interest is in classical hematology and hemoglobinopathies. He is the Director of the Center for Sickle Cell Disease at the University of Tennessee Health Science Center (UTHSC) at Memphis. His clinical research is focused on the vasculopathy of sickle cell disease, with an emphasis on pulmonary hypertension and renal complications, and coagulation activation in sickle cell disease. In addition, Dr. Ataga has a strong research interest in the development of new therapies for sickle cell disease. He conducts clinical trials in sickle cell disease funded by the National Institutes of Health and the Pharmaceutical Industry.   Representative Publications: Risk factors for mortality in adult patients with sickle cell disease: a meta-analysis of studies in North America and Europe Crizanlizumab for the Prevention of Pain Crises in Sickle Cell Disease Coagulation abnormalities of sickle cell disease: Relationship with clinical outcomes and the effect of disease modifying therapies Albuminuria Is Associated with Endothelial Dysfunction and Elevated Plasma Endothelin-1 in Sickle Cell Anemia Pharmacologic Therapies for Sickle Cell Disease Online profile

Lanetta Bronté-Hall, MD, MPH, MSPH, is responsible for strategic planning, scientific, and administrative oversight of the Foundation for Sickle Cell Disease Research (FSCDR). She is a leading national and international researcher and population health scientist in the field of sickle cell disease, rare blood disorders, community-based participatory research, and chronic disease management. Dr. Bronté-Hall has extensive experience in developing programs that are closely aligned with the recruitment and retention of underserved and underrepresented populations for treatment of SCD, research and clinical trials. She is currently President and CEO of the Foundation for Sickle Cell Disease Research (FSCDR), and Chief Wellbeing Officer (CWO) of the Sickle Care and Research Network, FSCDR, LLC, an independent full-service outpatient medical treatment and clinical trials center that offers coordinated care for persons suffering from SCD. Dr. Bronté-Hall is an Associate Professor in the Department of Public Health Sciences, Department of Health Services Research and Policy at the University of Miami's Miller School of Medicine, where she has created a sickle cell disease research portfolio and an academic–community partnership to translate research into practice. Online profile

Dr. Knee is a Director of Translational Medicine in the rare disease research unit of Pfizer and is working on sickle cell disease. Online profile

Sylvia Benjamin is Consultant in Haematology and Transfusion Medicine, NHS Blood & Transplant and Clinical Haematology at Oxford University Hospitals NHS Trust. She is honorary senior clinical lecturer at Nuffield Department of Clinical and Laboratory Science, University of Oxford. Online profile

The ocular manifestations of sickle cell disease (SCD) result from vascular occlusion, which may occur in the conjunctiva, iris, retina, and choroid. Because the ocular changes produced by SCD can be seen in other diseases, it is important to rule out other causes of occlusion, including central retinal vein occlusion, Eales disease, and retinopathy secondary to diabetes and other disorders. Other causes of ocular changes that should also be considered include familial exudative vitreoretinopathy, polycythemia vera, talc and cornstarch emboli, and uveitis. Treatment is directed toward preventing vision loss from vitreous hemorrhage, retinal detachment, and epiretinal membranes. Treatment may be medical or surgical. Abstract source

Dr. Olugbenga Olowokure is a hematologist in West Chester, Ohio and is affiliated with University of Cincinnati Medical Center. He received his medical degree from University of Maiduguri College of Medical Sciences. He is one of 8 doctors at University of Cincinnati Medical Center who specialize in Hematology. Research/Clinical Interests: Gastrointestinal oncology, Sickle Cell Disease, Hematology Oncology Online profile

The National Institutes of Health advises that optimal care for patients with sickle cell disease (SCD), including preventive care, is best achieved through treatment in clinics that specialize in the care of SCD. All patients with SCD should have a principal health care provider, who should either be a hematologist or be in frequent consultation with one. When severity of the patient's crisis is assessable, self-treatment at home with bed rest, oral analgesia, and hydration is possible. Individuals with SCD often present to the emergency department (ED) after self-treatment fails. Do not underestimate the patient's pain. Failure to treat acute pain aggressively and promptly may lead to chronic pain syndrome. According to the 2003 BCHS acute painful crisis guidelines, analgesia should be administered with 30 minutes of entering the hospital, with the goal of achieving effective pain control by 60 minutes. Abstract source

George Atweh, MD, is a professor and division director for the hematology/oncology division of the University of Cincinnati (UC) College of Medicine’s internal medicine department. He also serves as director of the adult cancer care program housed at University Hospital and associate director of adult medical oncology for the Cincinnati Cancer Consortium. Prior to joining UC in March 2009, Atweh served in numerous leadership roles at Mt. Sinai Medical Center, including hematology/oncology division chief, interim director of the Tisch Cancer Institute, associate director of the General Clinical Research Center, chair of the appointments and promotions committee and medical director of the cancer clinical trials office. He completed his medical degree, internship and residency at the American University of Beirut and a hematology/oncology fellowship at Duke University Medical Center. He then did a postdoctoral research fellowship at the Yale University School of Medicine. An established clinician-scientist, Atweh holds a U.S. patent for globin gene expression vectors, a discovery that has enabled scientists to pursue novel treatments for blood diseases such as sickle cell. Online profile

Dr. Rucknagel pioneered the use of incentive spirometry to prevent acute chest syndrome, a common complication of sickle cell disease. Dr. Rucknagel has been actively involved in sickle cell anemia research and treatment since he became a medical doctor. He operated a sickle cell clinic at The University of Michigan that is affiliated with a federally-funded sickle cell center in Detroit. His research has been in two basic areas. One is a search for a remedy for sickle cell anemia by manipulating the production of fetal hemoglobin; red blood cells containing fetal hemoglobin resist sickling and, therefore, should help improve the condition of sickle cell sufferers. The other is the development of better and more practical prenatal diagnosis of the sickle cell condition, to make possible more effective counseling of prospective parents. Online profile