Transcript Sickle cell disease

Chicago Experience, Progress, Outcomes,
and Expectations for the Future
Shonda King, MSW, LSW
Medical Social Consultant
University of Illinois Medical Center
Comprehensive Sickle Cell Center
Presenter Disclosures
Shonda King
“No relationships to disclose”
Purpose
The purpose of my presentation is to provide an overview
of the panel presentations and briefly discuss the
significance that Chicago, specifically the University of
Illinois at Chicago, has played in the advancement of care
for those affected by Sickle Cell Disease.
Presentation Outline
Experience: Chicago Connection to Sickle Cell Disease
Progress and Contributions
Outcomes: Comprehensive Care at All Life Stages
Future: Transitioning, Group Education, and Cure
Experience
Disease was “discovered” here at Rush University Medical
Center in 1910
University of Illinois at Chicago became home to one of
the NIH’s first ten nationwide elite Comprehensive Sickle
Cell Centers in 1972.
The national organization for Sickle Cell Support was
founded here in 1971 under the name the Midwest
Association for Sickle Cell Anemia (MASCA), it is now
called the Sickle Cell Disease Association of America.
U of I Physicians and Researchers have contributed
significantly to the advancement of care for individuals
with sickle cell disease since the 70’s
Progress and Contributions
Epigetic Gene Therapy (Dr. Joseph DeSimone, Dr. Rivers)
Prophylactic Penicillin [PROPS Study]
Hydroxyurea (Dr. Mabel Koshy)
Sickle Eye Disease (Dr. Maurace Rabb)
Pregnancy and Sickle Disease (Dr. Mabel Koshy)
Baby HUGS [Pediatric Hydroxyurea Study]
Kidney Disease (Dr. Santosh Saraf)
Psychosocial Needs (Dr. Anita Hurtig)
Metabolism of Opiates (Dr. Robert Molokie, Dr. S. Shord)
Acute Care Treatment Center (Dr. Michel Gowhari)
Stem Cell Transplantation (Dr. Santosh Saraf)
Hemoglobin F Inducers (Dr. Rivers, Dr. Lavelle)
Outcomes: Dr. Joseph DeSimone and Epigenetics
Thanks to U of I Geneticist Dr. Joseph DeSimone et. al, 5azacytadine was the first epigenetic study in the United
States and sickle cell disease was used as the first model
for treatment in diseases.1,2
The drug has epigenetic effects on RNA and DNA and was
used to increase fetal hemoglobin to reduce the incidence
of pain crisis and the duration of painful crisis.
Cancer therapies are designed better because of this study .
1.
2.
Heller, P. and DeSimone, J. (1984). 5-azacytidine and fetal hemoglobin. American Journal of Hematology,
17(4), 439-447.
Saunthararajah, Y. et al (2003). Effects of 5-aza-2′-deoxycytidine on fetal hemoglobin levels, red cell
adhesion, and hematopoietic differentiation in patients with sickle cell disease. Blood, 102(12).
Outcomes: Dr. Mabel Koshey and Hydroxyurea1
UIC enrolled the largest number of patients to participate
in the Double Blinded study.
Results showed that Hydroxyurea was effective in
decreasing painful crisis and hospitalizations
No direct correlations of QOL with the study, but personal
patient testimonies document the research participants
satisfaction.
Hydroxyurea approved by the FDA in 1991 as an effective
drug to manage SCD.
1.
Charache, S. (1995). Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia.
Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. New England Journal of
Medicine, 332(20): 1317-22.
Outcomes: Eye Disease and Pregnancy
Protocols on Sickle Eye Disease [Retinal Detachments,
Strokes in the Eye, and Floaters] came from the work of
Ophthalmologist Dr. Maurace Rabb who was a Professor
and Clinician in the UIC Ear and Eye Infirmary.1
Recommendations on the care of pregnant females with
sickle cell disease came from former UIC Director Dr.
Mable Koshy.2,3
1. Apple, D.J. and Rabb, M., 1978. Clinicopathologic correlation of ocular disease: a text and stereoscopic atlas.
C.V. Mosby: St. Louis.
2. Koshy, M and Byrd, L. (1991). Management of pregnancy and sickle cell syndromes. Hematology/Oncology
Clinics of North America, 5(3), 585-596.
3. Rogers, D. and Molokie, R.E. (2011). Sickle Cell Disease in Pregnancy. Obstetrics and Gynecology Clinic in
North America. 37(2), 223-37.
Outcomes: Psychosocial Progress
UIC Pediatric Psychologist Dr. Anita Hurtig1 looked at the QOL of
pediatric patients with SCD and recommended that those patients
receive NP assessments to determine their cognitive level of
functioning and made recommendations on now to structure services to
increase this level of functioning.
In 2004, Shonda King, MSW, LSW developed the concept categories
of comprehensive transitional care for young adults with sickle cell
disease.
1.
Hurtig, A.L. and Viera, C.T., (1986). Sickle cell disease : psychological and psychosocial issues. University
o Illinois Press: Urbana, Illinois.
Outcomes: Metabolism of Opiates
Research conducted by Hematologist/Oncologist Dr. Robert
Molokie and Pharmacist Dr. Stacy Shord revealed that because
of genetic differences, some groups metabolize Codeine to
Morphine differently than others.
Individuals with sickle cell disease of West African descent
were found to not have the enzyme to metabolize Codeine to
Morphine. They metabolize the drug at 50% of its clinical
effectiveness compared to other ethnic groups.1
This is significant because those patients who were found with
this enzyme deficiency had more frequent ER visits due to their
need for higher doses of medication to control their pain and
were not drug seeking.
1.
Shord, S. and Molokie, R.E. (2009). The pharmacokinetics of codeine and its metabolites in Blacks with
sickle cell disease. European journal of clinical pharmacology, 65(7), 651-658
Future: Transitioning
Transitioning is the process where a pediatric patient transfers
from pediatric care to adult centered care.
Teenage patients are seen in the Adult Center to initiate the
transition.
Five areas of Transitioning were identified.
Medical
Social and Psychosocial
Resource
Education
Vocation and Career
Future: Group Health Education
Centering Sickle Disease: Proof of Concept Pilot1
Exclusive to the University of Illinois Hospital and
Health Sciences System – Comprehensive Sickle Cell
Center
Have A Heart for Sickle Cell Anemia Foundation2
Unique pediatric and parent support group held at the
University of Illinois Hospital and Health Sciences
System.
1.
2.
Patil, C.L, Ciribassi, R.M, and King, S. (2014). Centering sickle cell disease: Proof of concept pilot.
www.hhsca.org
Future: Epigenetics
Dr. Angela Rivers1 and Dr. Donald Lavelle1,2 of the
University of Illinois Comprehensive Sickle Cell Center
are expanding on the work of Dr. Joseph DeSimone and
are looking at other ways of stopping the switching of
Hemoglobin F to Hemoglobin S in individuals with sickle
cell disease which occurs at about 6 months of age in
infants.
1.
2.
Rivers et al. (2015). RN-1, a potent and selective lysine-specific demethylase 1 inhibitor, increases γ-globin expression, F
reticulocytes, and F cells in a sickle cell disease mouse model. Experimental Hematology, 43(7):546-5.
Jessie Brown Veterans Administration, Chicago, Illinois
Future: Stem Cell Transplants
“Physicians
at the University of Illinois
Hospital & Health Sciences System have
cured 12 adult patients of sickle cell disease
using a unique procedure for stem cell
transplantation from healthy, tissue-matched
siblings.”1, 2
1.
2.
Saraf, S. et al (2015). Nonmyeloablative Stem Cell Transplantation with Alemtuzumab/Low-Dose
Irradiation to Cure and Improve the Quality of Life of Adults with Sickle Cell Disease. Biology of Blood
and Marrow Transplantation.
http://www.sciencedaily.com/releases/2012/06/120618194714.htm. Chicago woman cured of sickle cell
disease.
Conclusion
Since the first description of the disease in 1910, Chicago has
been a leader in research in the care of patient with sickle cell
disease with the University of Illinois playing a significant
role in the advancement of care. As new therapies and
models of care are being developed, the University of Illinois
Hospital and Health Sciences Systems will continue to lead in
providing the best in comprehensive care for pediatric and
adult patients living with sickle cell disease.
Faces of Sickle Cell Disease
Greece
Turkey
Palestine/ Israel
Village in northern Greece where 18% have the sickle
cell trait. Both parents have the trait and both children
have SS disease.
Three sisters from a Bedouin family from
Northern Israel. The lady (left) and the 3
children in front all have SCD
A family from Adana in south
east Turkey. Both parents and
daughter on the left has SCD
Faces and Places
Shonda King, MSW, LSW
Medical Social Consultant
Sickle Cell Center Social Worker
University of Illinois Hospital and Health
Sciences System
840 S. Wood Street (MC 712)
Chicago, Illinois 60612
312-996-1785
[email protected]