1. Barriers to Implementation of Evidence-Based Stroke Prevention in Sickle Cell Disease: A Preliminary Study Julie Kanter, M.D. Director, Sickle Cell Research Medical University of South Carolina

2. Stroke Prevention in Sickle Cell Disease Patients with sickle cell disease (SCD) are at increased risk for overt and silent stroke Chronic red blood cell transfusion therapy (CRCT) is a proven method for stroke prevention Patients with abnormal transcranial doppler (TCD; STOP I) Patients with prior stroke CRCT must continue indefinitely (STOP II)

3. Health Care Service Delivery in South Carolina Patient Barriers Large patient population Rural communities Poverty Health Care Barriers Pediatric care in Midlands, Upstate, and Lowcountry Minimal access to adult specialty care No specialty care in the Pee Dee Tri-County

4. Present Study Assess CRCT implementation in patients with SCD at high risk for stroke Hypothesized barriers: Age (pediatric versus adult) Care Provider (primary care vs. specialty care) Proximity to specialty care (Tri-county region) Additional barriers: Indication for CRCT (stroke versus abnormal TCD) Insurance status

5. Methods Retrospective chart review January 1, 2000 – December 31, 2014 Patient identification Pediatric and adult clinic database Previous stroke prevention trial participation Patients grouped based on current CRCT status Patients receiving CRCT Patients receiving care, but not CRCT Patients lost to follow-up in the medical system Data Analysis One-Way ANOVA and Chi-Square or Fisher’s Exact tests Follow-up logistic regression analyses adjusting for age

6. Patient and Health Care Characteristics N = 130 Age (mean)23 Gender (male) 52% Provider type SCD Primary Care Unknown 58% 17% 25% Proximity to care Tri-County Other 67% 23% Reason for Transfusion Prior stroke Abnormal TCD 62% 30% Insurance Medicaid Medicare Private 65% 23% 9%

7. Results Transfusion Status VariableYes (n = 68)No (n = 31)Unknown (n = 31)p-value Age (mean)202528<0.0001 Adult56%81%87%0.002 SCD provider91%45%-<0.0001 Proximity to care Tri-County Other 71% 29% 81% 19% 43% 57% 0.005 Reason Prior stroke Abnormal TCD 62% 32% 48% 45% 77% 10% 0.02 Insurance Medicaid Medicare Private 75% 16% 9% 48% 42% 10% 65% 19% 10% 0.07

8. Results Transfusion Status VariableYes (n = 68)No (n = 31)Unknown (n = 31)p-value Age (mean)202528<0.0001 Adult56%81%87%0.002 SCD provider91%45%-<0.0001 Proximity to care Tri-County Other 71% 29% 81% 19% 43% 57% 0.005 Reason Prior stroke Abnormal TCD 62% 32% 48% 45% 77% 10% 0.02 Insurance Medicaid Medicare Private 75% 16% 9% 48% 42% 10% 65% 19% 10% 0.07

9. Results Transfusion Status VariableYes (n = 68)No (n = 31)Unknown (n = 31)p-value Age (mean)202528<0.0001 Adult56%81%87%0.002 SCD provider91%45%-<0.0001 Proximity to care Tri-County Other 71% 29% 81% 19% 43% 57% 0.005 Reason Prior stroke Abnormal TCD 62% 32% 48% 45% 77% 10% 0.02 Insurance Medicaid Medicare Private 75% 16% 9% 48% 42% 10% 65% 19% 10% 0.07

10. Results Transfusion Status VariableYes (n = 68)No (n = 31)Unknown (n = 31)p-value Age (mean)202528<0.0001 Adult56%81%87%0.002 SCD provider91%45%-<0.0001 Proximity to care Tri-County Other 71% 29% 81% 19% 43% 57% 0.005 Reason Prior stroke Abnormal TCD 62% 32% 48% 45% 77% 10% 0.02 Insurance Medicaid Medicare Private 75% 16% 9% 48% 42% 10% 65% 19% 10% 0.07

11. Results Transfusion Status VariableYes (n = 68)No (n = 31)Unknown (n = 31)p-value Age (mean)202528<0.0001 Adult56%81%87%0.002 SCD provider91%45%-<0.0001 Proximity to care Tri-County Other 71% 29% 81% 19% 43% 57% 0.005 Reason Prior stroke Abnormal TCD 62% 32% 48% 45% 77% 10% 0.02 Insurance Medicaid Medicare Private 75% 16% 9% 48% 42% 10% 65% 19% 10% 0.07

12. Results Transfusion Status VariableYes (n = 68)No (n = 31)Unknown (n = 31)p-value Age (mean)202528<0.0001 Adult56%81%87%0.002 SCD provider91%45%-<0.0001 Proximity to care Tri-County Other 71% 29% 81% 19% 43% 57% 0.005 Reason Prior stroke Abnormal TCD 62% 32% 48% 45% 77% 10% 0.02 Insurance Medicaid Medicare Private 75% 16% 9% 48% 42% 10% 65% 19% 10% 0.07

13. Results Transfusion Status VariableYes (n = 68)No (n = 31)Unknown (n = 31)p-value Age (mean)202528<0.0001 Adult56%81%87%0.002 SCD provider91%45%-<0.0001 Proximity to care Tri-County Other 71% 29% 81% 19% 43% 57% 0.005 Reason Prior stroke Abnormal TCD 62% 32% 48% 45% 77% 10% 0.02 Insurance Medicaid Medicare Private 75% 16% 9% 48% 42% 10% 65% 19% 10% 0.07

14. Results Transfusion Status VariableYes (n = 68)No (n = 31)Unknown (n = 31)p-value Age (mean)202528<0.0001 Adult56%81%87%0.002 SCD provider91%45%-<0.0001 Proximity to care Tri-County Other 71% 29% 81% 19% 43% 57% 0.005 Reason Prior stroke Abnormal TCD 62% 32% 48% 45% 77% 10% 0.02 Insurance Medicaid Medicare Private 75% 16% 9% 48% 42% 10% 65% 19% 10% 0.07

15. Discussion ~50% patients either not receiving CRCT or lost to follow-up Patients less likely to be on CRCT: Adults (over 21 years of age) Patients without a current SCD care provider Abnormal TCD Patients lost to follow-up: Adults Prior stroke Live far from specialty care Limitations Retrospective Generalizability Sub-standard implementation of CRCT Patient- and provider-reported barriers

16. Future Directions Stroke prevention in SCD is ascertainable Patients<16 years of age should receive annual TCD screen Patients with abnormal TCD and/or history of stroke should be starting CRCT The majority of these patients should remain on CRCT indefinitely Research Patient- and provider-reported barriers to TCD screening and CRCT Evaluating different care environments and evaluate different implementation strategies Clinical care Improved training of primary care providers Increase patient access to specialty providers

17. Sickle Cell Disease in South Carolina Lifespan Comprehensive Sickle Cell Center at MUSC PI: Dr. Julie Kanter National Maternal and Child Health Workforce Development Grant › Co-Leads: Dr. Kanter and Jessica Drennan MSW SC 2 pilot project (Duke Endowment) › PI: Dr. Julie Kanter › Project Coordinator: Katherine Williams State Sickle Cell Disease Study Committee › Representative John King, Co-Chairman

18. SC Georgetown Columbia MUSC Florence Upstate Beaufort Case management Sickle Cell in South Carolina (SC 2 )

19. Acknowledgements: Thank you to our dissemination and implementation study team for sickle cell disease: Alyssa Schlenz, Ph.D. Joannie Hayes, M.D. Martina Mueller, Ph.D. Shannon Hudson, Ph.D., Robert Adams, M.D* Cathy Melvin, Ph.D.*