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
[email protected]
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Variant Sickle Cell Syndromes
Sb0, SC, Sb+
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Pathophysiology of sickle
cell disease
b6 Glu
Val
Deoxy Hb S polymer forms
with low O2,
Causes irreversibly sickled
cells
Susceptibility to infection
Shortened RBC survival
Vaso-occlusion
NO consumption
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Sickle Cell Anemia Disease:
Pathophysiologic features
Chronic hemolysis
Vaso-occlusion
Pulmonary
hypertension
Vaso-occlusive crisis
Priapism
Leg ulceration
Stroke Acute chest syndrome
Avascular necrosis
Priapism
Shortened Life Expectancy < 50 years of age
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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
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James Eckman, MD
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Georgia Comprehensive Sickle Cell
Center at Grady Health System
• ~1,200 Patients
- Largest Adult Clinic in North America
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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
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Morgan L. McLemore, MD
Fuad A. El Rassi, MD
Ross Fasano, MD
Eldrida Randal
Chris Terry Carter
Nelie Stoyanova
Michael Amanti, NP
Maureen Nijoku, NP
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
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Admission Rate From Sickle Cell Acute care
past 78 Months
100%
90%
80%
70%
60%
50%
40%
30%
20%
10%
0%
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3
5
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Admission Rate From Sickle Cell Acute care
past 78 Months
100%
90%
80%
70%
60%
50%
40%
30%
20%
10%
0%
1
3
5
7
9 11 13 15 17 19 21 23 25 27 29 31 33 35 37 39 41 43 45 47 49 51 53 55 57 59 61 63 65 67 69 71 73 75 77
Published Admission rates from ED for Sickle Cell ~40%
Our Acute Chest Rates ~ 5 times lower than expected
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• 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
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Archives Internal Medicine, 1910;6:517-521
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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
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Krishnamurti, L. Et Al
Blood, 126(23), 543.
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“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
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