Georgia Comprehensive Sickle Cell Center at Grady Health System Morgan L. McLemore, M.D. Associate Director of Division of Hematology for Hemoglobinopathies Medical Director of Georgia Comprehensive Sickle Cell Center at Grady Health System Department of Hematology and Oncology Winship Cancer Institute at Emory University mlmclem@emory.edu

Variant Sickle Cell Syndromes Sb0, SC, Sb+

Pathophysiology of sickle cell disease 6 Glu Val Deoxy Hb S polymer forms with low O2, Causes irreversibly sickled cells Susceptibility to infection Shortened RBC survival Vaso-occlusion NO consumption 5 5

Sickle Cell Anemia Disease: Pathophysiologic features Chronic hemolysis Vaso-occlusion Pulmonary hypertension Priapism Leg ulceration Vaso-occlusive crisis Acute chest syndrome Avascular necrosis Priapism Stroke Shortened Life Expectancy < 50 years of age

Sickle Trait (Hgb AS) ~ 1/12 African Americans Sickle Cell Disease (Hgb SS, Sb0, SC, Sb+) ~1/285 1/400 African Americans ~100,000 with Sickle Cell in the USA Incidence is not Declining

James Eckman, MD

Georgia Comprehensive Sickle Cell Center at Grady Health System ~1,200 Patients Largest Adult Clinic in North America 24-hour acute care for adults age 18 and older Chronic transfusion services Pain management Leg ulcer and Hydrea clinics Transition clinic – to ease the transition from pediatric to adult services

Morgan L. McLemore, MD Fuad A. El Rassi, MD Ross Fasano, MD Michael Amanti, NP Maureen Nijoku, NP Eldrida Randal Chris Terry Carter Nelie Stoyanova

Acute Care 24-hour acute care for adults age 18 and older Established in the 80s First in the world Funded by State Grant Goal is to reduce admissions and reduce complications Goal is prompt evaluation-wait time of less than 30 minutes Current is 40 Keep up to 8 hours before admission/discharge decision

Published Admission rates from ED for Sickle Cell ~40% Our Acute Chest Rates ~ 5 times lower than expected

Estimated additional 1,200 adult patients in the metro area. 1,875 pediatric patients 1,200 Adult patients Estimated additional 1,200 adult patients in the metro area. Average 10 new patients a month 1/3 from out of town

Archives Internal Medicine, 1910;6:517-521

Clinical Trials/Studies Vepoloxamer for acute VOC Phase III Complete Rivipansel for acute VOC Phase III recruiting Riociquat outpatient Phase II opening soon Stride II Allogeneic HSC transplant in adults with Sickle Cell Sickle Cell and Sexual and Reproductive Health Study. M Kotke, MD K Piper, PhD Influenza Vaccine study Surveillance Data CDC, GSU,CHOA

Krishnamurti, L. Et Al Blood, 126(23), 543.

“It is a sad and shameful fact that the causes of this disease have been largely neglected throughout our history. We cannot rewrite this record of neglect; but we can reverse it.” Richard M. Nixon, 1971