1. 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

4. Variant Sickle Cell Syndromes
Sb0, SC, Sb+

5. 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

6. 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

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

8. James Eckman, MD

10. 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

11. 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

12. 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

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

16. 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

17. Archives Internal Medicine, 1910;6:517-521

20. 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

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

23. “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