TRACKING FOR HIGH RISK CONDITIONS

1. TRACKING FOR HIGH RISK CONDITIONS Leslie Beres-Sochka, MS Program Manager Kathy Aveni, RNC, MPH Research Scientist Early Identification and Monitoring Program Special Child, Adult and Early Intervention Services New Jersey Department of Health and Senior Services

2. History of Hearing Screening New Jersey Department of Health began hearing screening of newborns in 1980 Originally risk-based screening Hearing Screening is required by NJ law (P.L. 2001, c.373, NJSA 26:2-101 et seq.) and rules (NJAC 8:19 subchapter 1) New Jersey Department of Health and Senior Services

3. Mandated Screening • Phase-in period had been given to facilities • May 15, 2000 - December 31, 2001: • risk factors for hearing loss; infants with risk must have electrophysiological screen • Law/rules specify confidentiality of reports • Risk-based screening has been replaced by Electrophysiological Universal Hearing Screening New Jersey Department of Health and Senior Services

4. Mandated Screening Effective January 1, 2002 ALL newborns must be screened using an electrophysiological screening device Under NJ rules, the responsibilities of birthing facilities, physicians, and midwives are specified Screening results are reported to DHSS via the Electronic Birth Certificate (EBC) New Jersey Department of Health and Senior Services

5. Mandated Universal Screening • Diagnostic Evaluation - should be completed by 3 months of age • Results must be reported to EHDI Program using the Follow-up form • Children with hearing loss must be registered with the SCHS Registry • link to SCHS county case management • link to EI case management New Jersey Department of Health and Senior Services

6. Special Child Health Services Registry • New Jersey legislation enacted in 1983 (NJSA 26:8-40.21) requires that all children with a birth defect diagnosed by 1 year of age be reported to the state Special Child Health Services Registry • Hearing loss at any age is to be reported to the Registry • Registered cases are referred to the county-based case management system within 10 days of receipt of registration • Registrations are entered into a SAS database • Hearing risk factors that are not evident at birth (syndromes, etc.) may be identified through SCHS registrations New Jersey Department of Health and Senior Services

7. Tracking Legislation C.26:2-103.6 Central registry of newborns at risk of hearing loss. 6. a. The commissioner shall establish a central registry of newborns identified as having or being at risk of developing a hearing loss. The information in the central registry shall be used for the purposes of compiling statistical information and providing follow-up counseling, intervention and educational services to the parents of the newborns listed in the registry. New Jersey Department of Health and Senior Services

8. ECMO (extra corporeal membrane oxygenation) Prolonged mechanical ventilation, 5 days or longer Persistent pulmonary hypertension In utero infection (TORCH) Family history of childhood hearing loss Parent/caregiver concern Head trauma Recurrent or persistent otitis media One minute Apgar 0-4 or 5 minute Apgar 0-6 Birth weight less than 1500 grams Bacterial or viral meningitis Cranio-facial abnormalities Syndromes with known association to hearing loss (Waarenberg, Down syndrome, Klipel-Feil) Hyperbilirubinemia requiring exchange transfusion Ototoxic medication administered for more than 14 days (for example, gentamycin and kanamycin) Newborn Hearing Risk Factors in New Jersey Law New Jersey Department of Health and Senior Services

9. ECMO (extra corporeal membrane oxygenation) Ototoxic medication administered for more than 14 days (for example, gentamycin and kanamycin) Prolonged mechanical ventilation, 5 days or longer Persistent pulmonary hypertension Family history of childhood hearing loss One minute Apgar 0-4 or 5 minute Apgar 0-6 Birth weight less than 1500 g Bacterial or viral meningitis Cranio-facial abnormalities Syndromes with known association to hearing loss (Waarenberg, Down syndrome, Klipel-Feil) Hyperbilirubinemia requiring exchange transfusion In utero infection (TORCH) Risk Factor Information in the Electronic Birth Certificate Data New Jersey Department of Health and Senior Services

10. Risk Factor Information in the Electronic Birth Certificate Data Risk Factor Ascertainment • Nursery Staff review chart for risk factors. • EBC ‘Worksheet’ is filled out reflecting presence or absence of risk factors. • EBC data clerk enters risk factors onto the EBC. • EBC data sent to EHDI program weekly. • EHDI Program populates NJ-EHDI data base with risk factor information. New Jersey Department of Health and Senior Services

11. EHDI Projects • EHDI Registry • CDC-funded cooperative agreement to create data linkages with EBC, SCHS registry and EI database to monitor UNHS implementation and to track diagnosis and treatment of children with a hearing loss or at risk for developing a hearing loss • Follow-up • HRSA-funded grant to locate children who fail to show for follow-up testing and to ensure babies do not “fall through the cracks” New Jersey Department of Health and Senior Services

12. EHDI Registry Data Sources: • Electronic Birth Certificate - Data on type of screening, results, demographics and risk factors • Follow-up forms - Data on outpatient testing results reported on standard forms are entered into EHDI database • Special Child Health Services Registry - Data provides confirmation of diagnosis and age at diagnosis • Early Intervention database - Data on EI enrollment, age at enrollment and services being received New Jersey Department of Health and Senior Services

13. Percent of Newborns Screened Prior to Discharge Source: EBC data New Jersey Department of Health and Senior Services

14. Screening of Babies with Risk Factor for Hearing Loss Source: EBC data New Jersey Department of Health and Senior Services

15. Risk Factor Rates Source: EBC data New Jersey Department of Health and Senior Services

16. Risk Factor Rates New Jersey Department of Health and Senior Services

17. High Risk Tracking Strategies • Routine re-screening of children with risk factors is reported to the NJ EHDI program using the Newborn Hearing Follow-Up Report form • Data from the Follow-Up form is entered into the EHDI database • EHDI program is currently investigating using the EBC information on baby’s provider to send letters inquiring about children known to have missed or failed screening or have risk factors for whom follow-up care has not been reported • In the future, the EHDI program will send reminder letters to parents of children with risk factors New Jersey Department of Health and Senior Services