Surviving to Thriving:  Improving Sickle Cell Care across the Lifespan

Patricia Kavanagh, MD, MSBoston University School of MedicineBoston Medical Center

New England Genetics CollaborativeNovember 15, 2011

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

~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

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

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

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.”

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

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

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

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 CareEvidence-Based Primary Care by Trained PCPs

IT Decision Support and Patient TrackingPeer Navigators and VolunteersCommunity-Based Enabling and Support ServicesContinuous Quality ImprovementAge-Appropriate Basic and Formal Genetic Counseling

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

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

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

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

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

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

Wrap-up

Questions?