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 ContributionsEpigetic 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, 5- azacytadine 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. Heller, P. and DeSimone, J. (1984). 5-azacytidine and fetal hemoglobin. American Journal of Hematology, 17(4), 439-447.

2. 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. Patil, C.L, Ciribassi, R.M, and King, S. (2014). Centering sickle cell disease: Proof of concept pilot.2. 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. 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.

2. 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. 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.

2. 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

Palestine/ Israel

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

Turkey

Village in northern Greece where 18% have the sickle cell trait. Both parents have the trait and both children

have SS disease.

Greece

Faces and Places

Shonda King, MSW, LSWMedical Social Consultant

Sickle Cell Center Social WorkerUniversity of Illinois Hospital and Health

Sciences System840 S. Wood Street (MC 712)

Chicago, Illinois 60612312-996-1785

sking3@uic.edu