1. Surviving to Thriving: Improving Sickle Cell Care across the Lifespan Patricia Kavanagh, MD, MS Boston University School of Medicine Boston Medical Center New England Genetics Collaborative November 15, 2011

2. Sickle Cell Disease  In US, ~100,000 people with SCD 2,000 babies born each year  Universal newborn screening & prophylactic antibiotics has led to improved survival ~95% children live to 18 years  Recent advances have decreased morbidity and mortality Hydroxyurea associated with improved survival TCD screening detects children at risk of stroke

3.  ~1,100 people with SCD in the Boston area; half are children. BMC serves two-thirds of patients with SCD  Immigrants comprise 28% of Boston’s population and 10% of Boston’s children Two of the largest populations are from Haiti (8.5%) and Dominican Republic (7.9%) – SCD common in both populations SCD in Greater Boston

4. Challenges in SCD Care  SCD care is complicated, requiring care from multiple providers, multiple settings Clinical settings (Hospital, ED, PCP, Hematology) Home Daycare/School Work  Lack of funding from federal programs and private foundations $9:$1 funding ratio for cystic fibrosis to SCD SCD population 3 times larger than CF

5. Identified Barriers in Boston area  No systematic coordination of SCD care between PCPs and specialists  Lack of primary care, especially adults, with SCD  Need for high quality transition to adult SCD care  Limited information & support provided at the time of newborn screening SCD and sickle cell trait

6. Parents’ Voices  Lack of PCP: “You know, [child] hasn’t been to his primary care doctor…because…[pediatric hematology] just give him everything over here.”  Transition Difficulties: “How can kids grasp the whole idea that they have to take this [medication] for the rest of their lives? And so, they’ll skip a day or two.’”  SCD diagnosis: “The worst experience for me… was when I found out. She was like, ‘You’re sure you want to go through with this? It’s going to be hard…’ I didn’t like…that they told me the results over the phone.”

7. HRSA SCD and Newborn Screening Program  Six sites: IL, MA, NY, OH, PA, TN  Patients: Children and adults  Focus: Sickle cell disease and trait Genetic counseling, especially for trait Education on SCD and trait (CBOs) Care coordination Mental health services Transition to adult services

8. Network Created for SCDNSP  BMC – Comprehensive sickle cell care and primary care  FQHC – Primary care (6 centers)  Greater Boston Sickle Cell Disease Association  New England Newborn Screening Program

9. Strengths of BMC + Network  Strong center for SCD research Basic science – Center of Excellence for SCD Health services research – Quality of SCD care  Long-standing relationships with FQHCs  Advanced HIT systems Data warehouse  Medical-Legal Partnership

10. Goals of SCDNBS Program Comprehensive Medical and Psychosocial Care and Community Support across the Lifespan Birth School Age Adolescence Transition Adulthood Community Outreach and Education Co-Managed Pediatric and Adult Primary and Specialty Care Evidence-Based Primary Care by Trained PCPs IT Decision Support and Patient Tracking Peer Navigators and Volunteers Community-Based Enabling and Support Services Continuous Quality Improvement Age-Appropriate Basic and Formal Genetic Counseling

11. Goal 1: Care Coordination for SCD  Improve coordination between: PCPs Specialists School nurses  How? Delineate responsibilities between specialty and primary care Delivery of evidence-based care by trained PCPs (Sickle Cell Champions) Improved IT decision support

12. Goal 2: Transition to Adult Care  Begin transition in early adolescence Utilize or develop materials appropriate for preteens and teens Track progress in transition process  Educational  Vocational  Social  Psychological

13. Goal 3: HIT and Care Coordination  Optimize electronic health record BMC and FQHC use same EHR  Massachusetts Health Disparities Repository Identify patients in need of care/services  Electronic referrals from PCPs in Network to specialists

14. Goal 4: Increase community services  Education and outreach  Peer Navigators  Genetic counseling for sickle cell trait  Peer-to-Peer Network  Annual conferences/gatherings for SCD community

15. Goal 5: NBS follow-up  Create a system in which PCPs: Provide initial information on SCD Counsel on sickle cell trait  Parents of newborns  Adolescents  Adults

16. Goal 6: Collaborate and Disseminate  New England Pediatric Sickle Cell Consortium  Hemoglobinopathy Learning Collaborative QI project on ED pain management for SCD  Newborn Screening Coordination and Evaluation Center (HRSA-funded)  Conferences  Publications

17. Wrap-up  Questions?