1. Pulse Oximetry Screening for Critical Congenital Heart Disease (CCHD): The Wisconsin Experience Region IV Genetics Meeting September 11, 2012 Sharon Fleischfresser MD, MPH Medical Director, WI CYSHCN Program in partnership with John S. Hokanson MD, University of Wisconsin – Department of Pediatrics 1

2. Presentation Objectives Review the status of pulse oximetry (POX) screening for critical congenital heart disease (CCHD) in Wisconsin as reported in the 2011 and 2012 hospital surveys Provide an overview of the newly funded CCHD demonstration grant Discuss plans for data collection and analysis 2

3. Wisconsin Population: ~ 5.7 million (concentrated in the southeastern portion) Land Area: 54,000 sq mi Approximately 67,000 births per year About 1,400 out of hospital births per year Approximately 100 hospitals routinely deliver newborns 3

4. Scope of the Problem in Wisconsin A study of all babies who were born in Wisconsin between 2002 and 2006 who were discharged from the newborn nursery as normal newborns 1: 24,684 were hospitalized or died due to unrecognized critical congenital heart disease in the first two weeks after birth – 3 per year in Wisconsin 1: 38,397 births died due to unrecognized critical congenital heart disease in the first two weeks after birth – 2 per year in Wisconsin Missed Congenital Heart Disease in Neonates. Benton Ng, MD and John Hokanson, MD. Congenit Heart Dis. 2010 4

5. 2011 Survey of Hospitals Telephone survey of 99 Wisconsin hospitals that routinely deliver newborns (February –March, 2011) Survey directed to nurse manager or administrator of the obstetrics or mother/baby unit Surveys were completed by 88 of the 99 hospitals (88.9%) that routinely deliver newborns Represented 95% of the state’s in-hospital births (62,728 births) Acknowledgement: Survey and results analysis by Daniel J. Beissel, MD (Pediatric Resident), Elizabeth M. Goetz, MD (Pediatric Newborn Hospitalist), John S. Hokanson, MD (Pediatric Cardiologist) at Department of Pediatrics, University of Wisconsin School of Medicine and Public Health 5

6. Limitations of Implementation Number of Surveyed Hospitals Percent of Surveyed Hospital Births Same Day Neonatal Echo is Not Available 55/88 (62.5%)25.6% Prostaglandin E1 is Not Available in Hospital 51/88 (58%)26% Transfer out of State for Higher Level of Neonatal Care 19/88 (21.6%)13.6% Miles Average Distance from Hospitals Without Neonatal Echo to Hospitals with Higher Level of Care 53.15 6

7. Map of Hospitals Screening and Locations of Pediatric Cardiology Practices - 2011 7

8. 2011 Survey Conclusions More than a third of Wisconsin’s newborns were being screened for CCHD with pulse oximetry at the time of the study The majority of Wisconsin’s births occurred in facilities where same-day neonatal echocardiography is available for confirmatory diagnosis of CCHD when necessary 8

9. Timeline September 2011: US Secretary of Health and Human Services recommend that POX screening be added to the core panel of routine newborn screening as recommended by the Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC) December 2011: WI Newborn Screening Umbrella Committee unanimously recommends CCHD screening per SACHDNC and formation of Ad Hoc CCHD Task Force led by Dr. John Hokanson June 2012: Dr. John Hokanson, University of Wisconsin, receives 3-year demonstration grant 9

10. July 2012 Hospital Survey Phone survey of 100 WI hospitals that delivered 12 or more babies in 2011 64 implemented POX screening (estimated at 85% of births based on hospital self-report) 8 planning 14 not screening 14 no data Follow up survey and analysis by Elizabeth M. Goetz, MD (Pediatric Newborn Hospitalist), John S. Hokanson, MD (Pediatric Cardiologist) at Department of Pediatrics, University of Wisconsin School of Medicine and Public Health 10

11. CCHD Demonstration Grant Dr. John Hokanson – Principal Investigator at the UW in partnership with: – Dr. Stu Berger - Medical College of WI (MCW) – State Laboratory of Hygiene (SLH) – Department of Health Services (DHS) – Office of Health Informatics, Wisconsin Birth Defects Registry, Newborn Screening Program, Title V Children and Youth with Special Health Care Needs – Guild of Midwives – Mended Little Hearts – Others Established CCHD Steering Group 11

12. Focus Areas Education for health care providers (hospitals, echocardiographers, primary care providers, midwives) regarding screening and implementation of recommended follow-up protocols Education for families (Mended Little Hearts) Home Births Demonstration (UW, Guild) Data Collection and Evaluation – Surveillance and Analysis (UW,SLH,DHS ) – Family Impact (UW) – Cost Assessment (MCW) 12

13. Next Steps Education for Providers and Families – Update of Pulse Oximetry Screening for CCHD toolkit with protocols and family education materials http://www.pediatrics.wisc.edu/research/research- groups/hokanson/Wisconsin-Pulse-Oximetry-Toolkit.pdfPulse Oximetry Screening for CCHD http://www.pediatrics.wisc.edu/research/research- groups/hokanson/Wisconsin-Pulse-Oximetry-Toolkit.pdf – Release of SHINE website with updated education materials – Establish SHINE project TA team to be available 24/7 to respond to failed POX screening and reporting questions – Research nurses (UW and MCW) and 9 pediatric cardiologists in WI – Ongoing outreach and education by SHINE project team Home Birth Demonstration – Field testing POX equipment WI Guild of Midwives 13

14. Hospitals, Birth Centers, Home Births Hospitals and Cardiology Centers Blood Card Information Failed POX; No blood card with POX screen Echocardiography reports and other diagnostic and clinical information Wisconsin Birth Defects Registry WBDR Wisconsin Office of Health Informatics OHI Birth, Death, Hospitalization Records WE-TRAC/Heart Pulse Oximetry & CCHD Data Staging Table Wisconsin SHINE Project Monthly summaries of blood card & failed POX report data Yearly summaries adding WBDR & OHI data – Matching Blood card, SHINE Data, OHI Data Flow Mechanisms Existing New SHINE CCHD DATA FORM (SHINE Technical Assistance Team) Data Collection and Analysis 14

15. Contact Information Sharon Fleischfresser, MD, MPH Medical Director WI CYSHCN Program Sharon.fleischfresser@wi.gov John Hokanson, MD Principal Investigator, WI SHINE Demonstration grant, University of Wisconsin jhokanson@pediatrics.wisc.edu Liz Oftedahl, CYSHCN Epidemiologist and Wisconsin Birth Defects Registry Elizabeth.oftedahl@wi.gov 15