GRANT NARRATIVE

UNDERSTANDING OF PROBLEM/CURRENT STATUS

Significant hearing loss is one of the most common major abnormalities present at birth and, if undetected, will impede speech, language, and cognitive development. The average age of detection is approximately 14 months. Universal detection of infant hearing loss requires universal screening of all infants. Screening by high-risk ascertainment alone can only identify approximately 50% of newborns with significant congenital hearing loss. Likewise, reliance on physician observation and/or parental recognition has not been successful in detecting significant hearing loss in the first year of life (American Academy of Medicine, February, 1999).

Technological improvements in screening equipment now make it feasible, efficient, and cost-effective for newborn hearing screening to be done by nurses or other trained personnel at birthing facilities prior to discharge. Universal newborn hearing screening has been recommended by the National Institutes of Health, the Joint Committee on Infant Hearing, the American Academy of Pediatrics and other relevant professional organizations.

Screening alone however is not sufficient to ensure that children who fail screening receive appropriate confirmatory testing, follow-up, referral, and services. The purpose of this cooperative agreement is to address the critical need for intervention, tracking, research, and integration with other screening programs. Specifically, the development and implementation of a surveillance and tracking system will help ensure that all infants are afforded screening and those identified with hearing loss are referred to and receive appropriate services. The data collected from this system can additionally be used in various ways including research and outcomes measures, as well as in integration with other newborn screening programs.

The development of a well-coordinated tracking system presents several challenges. The timely identification of children who fail screening is essential. The follow-up components include not only confirmatory testing, but also referral to treatment services and assessment of the success of these services. New Jersey has a strong infrastructure to support newborn hearing services, which includes timely, electronic reporting of children who fail hearing screening, registration into the Special Child Health Services (SCHS) Registry (which includes mandated reporting of birth defects), direct referrals to county-based case management services, and eligibility for the Part C, Early Intervention System (EIS). The development and implementation of a tracking and surveillance program would help to ensure minimal loss to follow-up and that infants receive appropriate services and management.

New Jersey is geographically small and is the most densely populated state with 1,094 persons per square mile. Nearly eight million people make it the ninth largest state, representing three percent of the total national population. The State’s population is projected to grow slowly through the year 2010 to just over 8.5 million. New Jersey is the most urbanized State, but has no single large city. Only three municipalities have more than 100,000 residents.

Compared to the nation as a whole, New Jersey is more racially and ethnically diverse. In 1997, 79.9% of the population was white, 14.5% was black, and 5.3% was Asian or Pacific Islander. In terms of ethnicity, 11.9% of the population was Hispanic. New Jersey’s racial, national and ethnic mix is also evolving, with its white population projected to decline by the year 2025 to about 55% of the total population. The percentage of blacks is expected to increase to about 15% by the year 2025. By the year 2025, Hispanics will become the largest minority group, with an estimated increase to just under 20% of the total population. Asians, which have become New Jersey’s fastest growing racial group, are expected to double their existing percentage of the population to approximately 10% by the year 2025.

The racial and ethnic mix of New Jersey mothers, infants, and children is slightly more diverse than the overall population. In 1997, 17.5% of mothers delivering infants in New Jersey were Hispanics, 73% were white, 18.6% were black, and 6.4% were Asian or Pacific Islanders. The diversity of New Jersey’s maternal and child population raises the importance of addressing disparities in health outcomes and improving services to individuals of diverse backgrounds.

In 1977, New Jersey passed a law and subsequently implemented rules for screening newborns for hearing loss. Prior to May 2000, the regulations required that all newborns be assessed for the presence of one or more specified hearing risk factors. The Department of Health and Senior Services (DHSS) was required to remind the parents of these newborns of the need to have a hearing screening conducted. Within DHSS the responsibility for the Newborn Hearing Program (NHP) was delegated to Special Child, Adult, and Early Intervention Services (SCAEIS). Screening newborns using risk factors identified approximately 100 newborns a week. Letters were mailed to each family, and NHP requested that families have a follow-up report completed and returned. However, of the more than 5,200 letters mailed annually, fewer than 17% of the newborn screening follow-up reports were returned.

Recognizing that this system did not adequately address early identification of children with hearing loss, coupled with the advent of improved screening technology, the DHSS implemented several major initiatives. During 1999, the DHSS revised the health objectives for New Jersey. For the first time, within the Healthy New Jersey 2010: A Health Agenda for the First Decade of the New Millennium, three hearing objectives were included: increase the percentage of newborns screened with state of the art tools for hearing loss prior to discharge from the birthing facility or by one month of age to 90%; increase the percentage of infants receiving diagnostic follow-up after a positive screening for hearing loss by three months of age to 90%; and increase the enrollment of infants diagnosed with hearing loss/deafness in appropriate intervention services by six months of age to 90%. Second, newborn hearing screening has been presented by the Division of Family Health Services (FHS) to the Commissioner DHSS as one of its major goals for the next two years. Third, and perhaps most importantly, the DHSS changed the regulations to mandate universal newborn screening, to be phased in at all New Jersey birth facilities by January 1, 2002.

In May 2000, revised Newborn Hearing Screening rules (N.J.A.C. 8:19) were adopted (Addendum A), which require the DHSS to provide information describing normal auditory development and the Newborn Hearing Screening Program to parents and legal guardians of all infants born in New Jersey. Effective May 15, 2000, all newborns with one or more risk factors associated with hearing loss are required to have an electrophysiological hearing screening performed either prior to discharge or before one month of age. By January 1, 2002, all newborns will be required to be screened for hearing impairment with electrophysiological measures prior to discharge.

DHSS expects that statewide implementation of universal newborn hearing screening will be finalized before January 1, 2002. As of June 2000, of the 71 birthing facilities in New Jersey, 34 use auditory brainstem response (ABR) technology, 22 hospitals use otoacoustic emissions (OAE) technology, and seven facilities use both ABR and OAE. Currently, 37 facilities (52%) have implemented universal screening. In 1999, there were 110,059 live births in New Jersey. Approximately 45,734 (42%) of these babies received electrophysiological hearing screening, 801 (2%) of these failed the initial hearing screen.

Many infants with hearing loss who might otherwise go undetected will be identified through universal newborn hearing screening in New Jersey. In 1998, for instance, of 113,632 births, 62 babies with sickle cell disease, 3 babies with phenylketonuria, 49 babies with congenital hypothyroidism, and 3 babies with galactosemia. It is estimated that 330 would have been identified with hearing impairment (based on American Academy of Pediatrics Task Force on Newborn and Infant Hearing Statement, Feb. 1999).

Since 1997, all birthing facilities and birthing centers use an Electronic Birth Certificate (EBC) system to electronically transmit newborn data to DHSS on almost a real time basis. The EBC has eight modules including the standard birth certificate, perinatal and prenatal histories, information on hearing risk factors, and parental informed consent (Addendum B). The parental informed consent information indicates that the parent(s)/guardian(s) have received materials regarding the NHP. It also indicates that if one or more risk factors associated with hearing loss are found in the medical record, their newborn will receive an electrophysiological hearing screening before discharge or before one month of age. Parent(s)/guardian(s) must sign the consent form, and must indicate on the form whether or not they refuse the hearing screening.

The NHP provides all birthing facilities with the brochure: “Can My Baby Hear?” This brochure details the purpose of the program, the importance of hearing to speech and language development, the risk factors associated with hearing loss, information about ear infections and hearing screening, and a checklist of developmental milestones for hearing from birth through 6 months of age. These brochures are available in English, Spanish, and Haitian French, and are provided to all new parents/guardians at discharge from every birthing facility.

Currently, the NHP extracts data from the EBC to identify those newborns who either have a risk factor or have failed one of the newborn hearing screening tests. Hospitals provide the Newborn Hearing Follow-Up Report (Addendum C) to parents whose infant fails the screening. The hospitals also give families information on audiologic testing and services.

Any newborn/infant identified with congenital or late onset hearing loss is reported to the SCHS Registry (Addendum D) in the Early Identification and Monitoring (EIM) Program, SCAEIS, which is responsible for maintaining mandated birth defects reporting and voluntary registration of children with other health conditions. The SCHS Registry serves as the primary entry point into the local, community-based case management system. A copy of the registration form is given to the case management unit in the county of the child's residence. Compared to other states, this direct link from the surveillance system to the service delivery system is unique. It is both highly effective, cost efficient, and serves as a means of quickly identifying children with special health care needs who may require service intervention. Since nearly 65% of all registered children are reported to the SCHS Registry by three months of age, families receive timely support and information for their children.

SCHS Case Management Units, under the guidance of Family Centered Care Programs (FCCP), SCAIES, are located in each of New Jersey’s 21 counties. Case managers reach out to the families of every registered child. These Units provide care coordination at no expense to families and assist in referring families to financial and social support resources such as Medicaid, KidCare, Charity Care, and the Catastrophic Illness in Children Relief Fund program.

The FCCP also provides assistance to families when hearing aids need to be purchased according to Financial Participation Guidelines. The hearing aids are available from an approved hearing aid vendor and must be prescribed by a New Jersey licensed audiologist. In state fiscal year 1999, approximately $50,000 was provided to families towards the purchase of hearing aids.

The Case Management Units serve as the single point of entry for referral to EIS, which is also funded and managed through SCAEIS. Currently there are 46 Early Intervention Programs (EIP), four of which provide services exclusively for children with hearing loss.  One EIP has a teacher of the deaf, who provides home service to children with hearing loss and their families. The remaining programs provide services for any child with a special need, including hearing loss. Trained EIP personnel provide services to help meet the needs of children between birth and 3 years of age. Services are provided in natural environments, with parents, guardians, or day care workers observing and participating so as to learn the skills necessary to encourage each child’s individual development. EIS also has a toll-free number for Project CHILD FIND, so parents can call to learn more about the early intervention system and the referral process.

NHP has been invited by Karl White, Director of the National Center for Hearing Assessment and Management, to participate in a research study which will focus on issues related to follow-up in early hearing detection and intervention programs and the effects on families of newborns. This proposal examines the need for a state surveillance and tracking system. This system would enable the collection of outcome data and integration with other newborn screening systems, and provide further assurance that newborns in New Jersey are receiving timely screening, identification, and intervention.

GOALS AND OBJECTIVES

The primary goals of this project are to: 1) implement a state wide EHDI surveillance and tracking system to ensure minimal loss to follow-up of infants; 2) identify children with late onset/progressive hearing loss; 3) integrate the EHDI hearing tracking system with existing tracking systems; 4) collaborate with other states; and 5) establish an EHDI Advisory panel to include families and to ensure culturally sensitive services/educational materials are available.

• Implement a statewide EHDI surveillance and tracking system to ensure minimal loss to follow-up of infants.
• DHSS and Maternal and Child Health (MCH) Consortia will provide training/technical assistance to birthing facilities implementing universal newborn hearing screening.
• DHSS will expand the existing data collection system to include tracking information.
• DHSS will monitor hospital compliance with mandated screening and referral to diagnostic testing in collaboration with the MCH Consortia.
• DHSS will monitor hospital compliance with mandated follow-up services, in collaboration with the MCH Consortia.
• DHSS will collaborate with providers to ensure compliance with mandated registration to the SCHS Registry and completion of the Newborn Hearing Follow-Up Report form.
• Identify children with late onset/progressive hearing loss.
• DHSS will monitor the number of children reported to the SCHS Registry with hearing loss diagnosed after 6 months of age and include them in the EHDI system.
• DHSS will work with the advisory panel and providers to encourage reporting of children with late-onset hearing.
• DHSS will compare data from Special Education on the number of children with hearing disorder.
• Integrate the newborn hearing tracking system with other tracking systems.
• Continue to enhance the integration of the EHDI with the SCHS Registry and the Newborn Biochemical Screening Follow-up system.
• The Newborn Biochemical Screening Program will actively track children with potential hearing loss who also have a biochemical disorder.
• Investigate potential connections to lead screening, WIC, or other relevant databases.
• Collaborate with other grant recipients.
• The Research Scientist II (RS II) will collaborate with the other recipients and CDC to develop standard data collection and tracking methods.
• NHP staff will participate in national meetings, conference calls, and outreach to other state newborn hearing programs.
• Collaborate with families and professionals via the EHDI Advisory Panel and focus groups to increase awareness of the importance of early detection of hearing loss and to ensure the availability of culturally sensitive and linguistically appropriate resources.
• Establish an Advisory Panel.
• EHDI services will be organized so that families can participate in decision-making at all levels, and are satisfied with the services they receive as judged by evaluation data collected from a randomly selected sample of families.
• DHSS will work with EHDI Advisory Panel to develop a mechanism to identify and address professional/parental input and concerns about the EHDI-NJ.
• DHSS and EHDI-NJ Advisory Panel members will make presentations and prepare articles about the importance of early identification and intervention.

New Jersey has a strong commitment to including women and people from many different racial and ethnic groups in both staff and services, and has an active program to implement that commitment. The goal of this project is to ensure that all children with hearing impairment are tested and linked with services, regardless of their gender or racial/ethnic background. Partnership with the MCH Consortia, which are representative of the areas they serve, will also provide for diversity. Membership on the project’s Advisory Panel and the focus groups will reflect the diversity of New Jersey, including people with hearing impairment.

DESCRIPTION OF PROGRAM AND METHODOLOGY

The main focus of this proposal is the implementation of a statewide EHDI surveillance and tracking system, through expansion of the existing hearing screening database. Through collaboration with the existing EBC, SCHS Registry, Case Management System, EIS, Newborn Biochemical Follow-up Program, MCH Consortia, and the EHDI Advisory Panel, all components to collect EHDI data for a surveillance and tracking system will be in place.

A RS II will be hired to coordinate and develop the EHDI system. The EHDI system will be populated with data from the EBC for newborns who either have failed a screen or have a risk factor for hearing loss. The new hearing regulations specify the responsibilities of hospitals and providers for assuring confirmatory testing, completion of the hearing report, and when needed, registration with the SCHS Registry. The MCH Consortia, aided by the Advisory Panel, will monitor the hospitals for compliance with the regulations. Reporting to the SCHS Registry provides for direct referral to Case Management Units and the service component of the EIS. By July 2001, EIS will implement a comprehensive management information system and data will be available on provision of services, referrals, and outcomes. These components together will provide all key elements required for the EHDI system.

The EHDI system will include all newborns in the state of New Jersey. In 1999, there occurred 110,059 live births in New Jersey. Of this number, 45,734 babies (42%) received hearing screening by some electrophysiological measure prior to discharge. Of the children receiving the screen, 801 (2%) failed the screening test. Additionally, children diagnosed with late onset hearing loss will also be added to the EHDI system.

As described earlier, SCAEIS and the FHS have extensive experience in developing and using large, confidential data systems.  Staff in SCAEIS are responsible for the development and management of the current Newborn Hearing Screening (NHS) data system. This application was developed in EpiInfo, and has been functional since the implementation of New Jersey’s EBC in 1996. The EBC in New Jersey contains data on the hearing risk factors, whether or not an electrophysiological test was performed, if the child failed the test, and extensive clinical and demographic variables. Every week, each birthing facility electronically submits to the DHSS data on new births and updates to previous births for inclusion in the EBC master data file. NHS staff download the weekly submissions to their computers. The NHS data system then reads the weekly EBC updates into a data file and queries this new file either for children with positive risk factor(s) for hearing loss, or for children failing an electrophysiological screening test. The data records of these children are then merged into the NHS database. Audiologists and other medical personnel submit follow-up reports, detailing results of the child’s formal hearing assessment. The follow-up information is also entered into the NHS database.

The current NHS data system will serve as the foundation for an updated EHDI surveillance and data tracking system. Utilizing the expertise within SCAEIS and FHS, an enhanced system will be created. The RS II will work with program and division personnel, and with CDC, to develop the updated tracking mechanism and to identify the data to be collected. The new system will include all standardized EHDI data, and other demographic/clinical variables of interest. The system will be designed to track a child’s progress from initial screen to confirmatory test to ultimate outcome/intervention. Data sources will include the EBC, birthing facilities, diagnostic centers, SCHS Case Management Units, EIS, and primary care providers. To minimize loss to follow up, the EHDI data system will be programmed to provide reports on the status of the children’s progress though the components of the EHDI process. This system will be developed in standard software, possibly SAS or Access, in collaboration with CDC and the other grant recipients. Current data will be converted into the new system.

New hearing regulations, effective May 2000, delineate the responsibilities of a number of agencies and professionals, in the implementation of universal newborn screening, the establishment of a surveillance and tracking system, and service delivery. Through December 31, 2001, hospitals are required to ensure that newborns with one or more hearing risk factors are screened using electrophysiological methods, either prior to discharge or by one month of age. Beginning January 1, 2002, all newborns must be screened prior to discharge. Screening results are provided to DHSS via the EBC. Hospitals are required to establish guidelines for the provision of follow-up services, such as confirmatory pediatric audiologic assessment and diagnosis of newborns with abnormal or inconclusive test results, submission of the Newborn Hearing Report to SCAEIS, and counseling and educational services for the parents. Each month, the NHP will send maternity hospitals a listing of children born at their facility who were screened for hearing disability. The facilities will determine which children have been missed, and are responsible for ensuring that all children are screened and have confirmatory testing completed. Providers of the diagnostic testing are required to complete the Newborn Hearing Report and, if a child is diagnosed with hearing loss, complete a SCHS registration form.

The hospitals and providers conducting the screening play an essential role to ensure newborns who fail the screen receive a confirmatory test. DHSS will provide support to the seven regional MCH Consortia to work with the hospitals and providers to ensure this occur. The MCH Consortia, coordinated by the Maternal, Child, and Community Health Program, FHS, are private, non-profit organizations. The MCH Consortia promote a coordinated and cooperative prevention-oriented approach to perinatal services. All New Jersey birthing hospitals are required by state regulation to belong to one of the Consortia. The Consortia provide prevention activities, outreach and education, coordination of transport systems, total quality improvement (TQI) management, and development of regional perinatal/pediatric plans.

The Consortia were instrumental in assisting DHSS with the implementation of the EBC. The Consortia directly, and/or through their member hospitals, monitor the quality of EBC data through an ongoing quality assurance process, including the provision of technical assistance and ongoing education. They are responsible for data analysis, consumer and professional education, and infant and pediatric follow-up of issues related to the EBC. The MCH Consortia will monitor hospitals and facilities providing infant and pediatric audiologic testing services to ensure that newborns receive diagnostic testing, newborn hearing follow-up reports are completed, and when necessary, completion of the SCHS registration form.

To complement the role of the MCH Consortia, the DHSS will expand the current NHS Task Force (Addendum E) to form and convene the EHDI Advisory Panel. This Advisory Panel will include representatives from DHSS (Newborn Hearing Program, the Newborn Biochemical Screening Program, Family Centered Care, and Early Intervention), the Departments of Education, Human Services, and Banking and Insurance, audiologists, medical providers, such as a pediatrician, a family practice physician, neonatologist, geneticist and/or genetics counselor, and an otolaryngologist or otologist. Ethnic and racial groups, both sexes, families with a deaf or hearing impaired child, deaf or hearing impaired adults, and a representative from the Commission for the Blind and Visually Impaired will be represented on the Advisory Panel. The work of this Advisory Panel will be ongoing throughout the duration of the EHDI project. This panel will also work with providers and the community to ensure children are tested and reported to the SCHS Registry, if found to have hearing loss.

The Advisory Panel will ensure that the concerns of both parents and professionals are monitored and brought to the attention of DHSS. Surveys and focus groups, comprised of parents and professionals, will be used to document concerns. Families and professionals will be made aware of the importance of early detection of hearing loss and that culturally sensitive and linguistically appropriate services are available. Focus groups will be used for the testing of written material. EHDI services will be organized so that families can use them easily, and participate in decision making at all levels and are satisfied with the services they received as judged by evaluation data collected from a sample of families.

As previously discussed, children with confirmed hearing loss will be reported to the SCHS Registry, which serves as the primary entry point into the community-based case management system. Case Management Units will continue to provide coordinated, community based, family centered care for children with hearing loss. The case manager will work with families/guardians and their physicians to ensure the child receives appropriate community based, culturally sensitive services.

Case Management Units also provide the service coordination for EIS. The timely linkage of the surveillance and service system is particularly beneficial to EIS. Currently, the EIS identifies 10 conditions that constitute “presumptive eligibility,” one of which is hearing impairment. Children presenting with one or more of these conditions are eligible, by virtue of their diagnosis, to receive early intervention services.

Over the past two years, the EIS has been working to develop a coordinated information management network across more than 70 external EIS sites, DHSS, and other state and local agencies. Ideally, the statewide uniform data collection and reporting computerized system will include: client demographics; automated case management/service coordination functions; access/utilization of services; progress/satisfaction of children and families with the received services; real-time electronic scheduling at agencies having openings; and communication with families. Such a system will support the DHSS policy development, program planning, and quality assurance efforts, including the recommendations made by the consumer-driven Early Intervention Stakeholder Task Force.

In the year 2000, the decision was made to use the New Jersey One-Easy-E Link management information system, using FACTORS. This system is currently being used by the Department of Human Services, as well as by Senior Services within DHSS (see Addendum J for detailed description and data dictionary for Senior Services). The FACTORS application will be used for client record keeping for such variables as demographics, family relationships, assessments, service plans, service encounters, referrals, employment history, housing and living arrangements, income sources, insurance, household expenses and budgeting, progress notes, and health history administrative features. The necessary modifications to the databases used by Senior Services will begin in July 2000. EIS staff will meet with a wide range of potential users, including all staff within SCAEIS to ensure that the system contains the information their programs need for evaluation and program planning. The revised system will be functional by June 2001. Future plans for EIS and FACTORS include potential expansion to include non-EIS case management functions, as well as linkage to the educational system.

Once the system is functional, IRB approval will be sought to link the Newborn Hearing data files with the EIS FACTORS. SCAEIS has a long history of linking confidential datasets for statistical, research, and evaluation purposes. One such recent linkage is matching of the AIDS/HIV and SCHS Registry (see Addendum K for copy of the IRB request and approval). The manager of EIS supports such a linkage with the EHDI system. Once this linkage is complete, for each child with hearing loss, there will be complete data on such critical variables as service referrals and utilization, and eventually outcome and performance measures.

The RS II will work with the medical, audiology, MCH, EHDI Advisory Panel, and advocacy communities to develop the mechanism to identify and report infants and children with late onset or progressive hearing loss. All children identified with hearing loss will continue to be reported to the SCHS Registry, and thus referred immediately into the SCHS Case Management system and the EIS. These agencies will submit updated status reports for these children to the NHP for inclusion in the EHDI data system. The electronic birth records for these children will be merged into the database.

When fully functional, the EHDI system will contain a full range of data needed for analysis, needs assessment, description, and reports. The data will be analyzed on an ongoing basis to calculate various statistics. Examples include, but will not be limited to, the following: number of children screened, average time from birth to initial screen, number of children receiving confirmatory testing, average time from screen to confirmatory testing, average age at time of diagnosis, rate of false positives, and the rate of occurrence of hearing problems by region. GIS software will be utilized to identify potential clusters of infants with hearing loss. SAS, or some other standard package, will be used for statistical analysis. Written reports will provide descriptive information about the program as well as serve as the basis for the quality assurance and evaluation of the statewide program. The reports will also be used to document outcome data such as: unexpected clusters of infants with hearing loss in particular regions at particular times; unexpected differences in measure of EHDI screening performance between participating hospitals; false positive rates; and lost to follow-up rates.

Data sources for the analyses will include the EHDI system, EBC, SCHS Registry, and other data sources as needed. EHDI data system will also be used to determine children with missing screens, confirmatory test results, and/or lack of diagnostic and intervention data. The RS II will integrate all systems necessary to gather the outcome data listed above, including: the hospital screening information, follow-up screening information from primary care providers, diagnostic data from audiologists and intervention data from early intervention providers.

As described earlier, children with hearing or metabolic disorders are included in the SCHS Registry. This file is unduplicated, and contains diagnostic information for each registered child. As such, children with either or both conditions are included in a single database. Each year, nearly 9,000 children are newly reported to the SCHS Registry. About half of the children are registered with one or more birth defects while the remaining children have other at-risk or special needs conditions. As part of the registration process, when the child is not expired, a letter is mailed to the family by EIM. The letter, which informs the family of the registration, contains a variety of informational brochures and contacts.

Since 1998, staff from the EIM program have been working to link children reported to the SCHS Registry with birth certificates files. The linking has been completed for birth years 1990-1997. In addition to matching with birth certificates files, a variety of other quality assurance procedures are used to ensure children are accurately reported to the Registry. Quality assurance audits are conducted annually at all maternity hospitals and facilities with pediatric beds. Data conducted from these audits indicate that 85-90% of children with birth defects are reported to the Registry. Other databases, including birth and death files, are reviewed to identify potentially missed children with birth defects.

The Newborn Biochemical Follow-up program actively tracks the nearly 1,600 newborns with abnormal metabolic screening on one or more of the four disorders included in the screening panel: phenylketonuria, congenital hypothyroidism, galactosemia, and hemoglobinopathies, including sickle cell disease. Additionally, approximately 2,800 newborns have sickle cell trait, and while not actively tracked, the families receive information from the Follow-up program. An advisory panel for Newborn Screening was convened by DHSS in April 2000, to investigate adding disorders to mandating screening. Public hearings will be held to receive and hear testimony concerning their recommendations.

Newborns with abnormal results are tracked by the Follow-up program until written case confirmation is received. The follow-up database is maintained by Neometrics, and will be converted in August 2000 to a window-based system. The follow-up staff contact physicians and consultants to ensure that the newborn is rescreened and if confirmed with a disorder, receives treatment. Each month, the Follow-up program issues a “turn-around” tracking report which documents the newborn’s age when the initial presumptive-positive results were received by the program and reported to the physician of record. The program monitors the age at which newborns with confirmed disease are seen by a physician. Laboratory and program staff, and consultants for the disorders, for quality control and management issues use these data.

As the EHDI tracking system is developed, the experience of the biochemical system will be used to guide the process. Additionally, as children who fail hearing screening are identified, their names will be matched to the biochemical database. If a match is found, then the biochemical follow-up staff will remind treating physicians of the failed hearing screening results and the need for confirmatory testing.

The RS II will develop a detailed manual describing the EHDI system, including descriptions of the tracking system, methods for case ascertainment, and data analysis methods. They will also collaborate with other participating sites to prepare and publish the findings of the EHDI system.

EVALUATION PLAN

To be successful, ongoing formative evaluation activities should be thoroughly integrated with implementation activities. The design and implementation of the EHDI data system will allow for the measurement of all major outcomes, time frames, and allow for continuous program monitoring. Formal reports will be prepared at least annually and the information will be provided to all collaborators. The goals will be evaluated in the following ways:

The EHDI data system will include basic information on all infants screened as well as expanded information on diagnostic data, referral status, intervention, and follow-up services. The RS II will analyze the EHDI data to determine the numbers and rates of cases lost at each transition point, e.g., baby not screened, family not contacted for confirmatory testing, family failing to follow-up for confirmatory testing, family not referred for follow-up services, family failing to receive follow-up services, and rates of referral. Where problems are identified, further research will be done to try to identify reasons for losses. This will include examination of rates by county and hospital as well as by preferred language and racial/ethnic group to identify any potential problem areas. In addition, average time to confirmatory testing, diagnosis, and linkage to follow-up services will be calculated and monitored to track improvements over time. MCH Consortia, through their TQI procedures will assist in monitoring birthing facilities, to ensure families receive appropriate services and referrals. DHSS will monitor and evaluate whether TQI procedures have resulted in better reporting to the EHDI system.

The RS II will analyze all data and trends to report program activity and successes or limitations in meeting the screening and follow-up objectives. This report will be presented annually to the EHDI Advisory Panel and the Consortia. Modifications to the EHDI program will be made as necessary. In addition, in collaboration with CDC and other grant recipients, particular outcome data of interest will be collected and analyzed. This will include rates of false positive screenings as well as clusters of hearing loss in particular regions at particular times.

Baseline rates of the numbers of children with late onset/progressive hearing loss will be obtained by identifying children who were registered during the years 1995-1999 with the SCHS Registry with hearing loss diagnosed at greater than 12 months of age. Similar numbers will be calculated during the period of the grant. EHDI Advisory Panel will work to improve locating and reporting children with late onset hearing loss. Efforts to improve identification should result in increases in the numbers of those identified.

Numbers of children receiving special education services for hearing loss will be obtained from Special Education records. This data will be compared with the data from the EHDI data system and the SCHS Registry by age group and county as one estimate of completeness.

Matching among the various tracking systems (e.g., Newborn Biochemical Screening, SCHS Registry) will be performed to determine the numbers of non-matches. Rates of non-matches will be monitored over time. Children not matched will be added to the respective database, where allowed by state regulations. The NBS Program will document and report to the RS II as to the number of children with hearing loss they have actively followed.

Participation in national meetings and telephone conferences will be monitored by DHSS. Outreach efforts to other state newborn screening programs, collaboration with other centers on data analysis and preparation of manuscripts, and development of information-sharing agreements will be documented.

Three methods will be used to document concerns from families and professionals. First, the Advisory Panel will be an important arena for monitoring program progress and raising concerns. These will be documented in the minutes and summarized at least semi-annually. Second, surveys and focus groups of families to be conducted under the auspices of the Advisory Panel will provide an opportunity to assess satisfaction with the EHDI process and the services that have been received. Finally, the MCH Consortia, in their Quality Improvement activities for the hospitals in their region, will gather information not only on concerns raised by professionals, but also on problems they observe with system implementation at the hospital level.

COLLABORATIVE EFFORTS

New Jersey currently has a strong infrastructure to support newborn hearing services, including timely identification of newborns who fail a hearing screening or who need to be screened, new hearing regulations phasing in universal newborn hearing, regulations defining hospital and provider responsibilities, mandated registration into the SCHS Registry, direct referrals to county-based case management services, and eligibility into the EIS. These activities are performed by the Center for Health Statistics, who has responsibility for the EBC, and SCAEIS. These collaborative intra-Department responsibilities will be integrated with other key agencies, including all maternity hospitals, providers of audiologic testing, the MCH Consortia, the Department of Special Education, parents, professionals, and the EHDI Advisory Panel. It is expected that other key collaboration outreach efforts will be made with the CDC and other states’ hearing professionals.

The Advisory Panel will be instrumental for maintaining many of the collaborative relationships, since it is comprised of representatives from all the major stakeholders. Key among the list of Advisory Panel members are parents, representatives of the deaf and hearing-impaired community, involved professionals, inter and intra-Departmental personnel, and medical/health care providers. It is critical that all care for identified children to be coordinated with the child’s primary care provider. Representatives of the primary care providers on the Advisory Panel can assist in developing the plan for communication between the screening project staff, primary care provider, specialists and the family. Family voices, SPAN and other advocacy groups will also be instrumental in both the implementation and successful continuation of this project.

Identifying infants with hearing loss and providing services to them and their families requires participation from many different stakeholders and disciplines. In some cases, these groups serve a broader constituency, of which children with hearing loss are just one part. In other cases, their efforts are focused specifically on children with hearing loss. EHDI-NJ proposes coordinating the efforts of these groups into an effective, statewide system.

Addendum F includes letters of support from interested parties involved in the implementation of universal newborn hearing screening in New Jersey.

STAFFING & MANAGEMENT SYSTEM

Both FHS and SCAEIS have a long and successful history in designing and developing systems of service for children with special needs, including children with hearing loss. They also have vast experience in establishing and maintaining a wide variety of computerized databases. FHS has recently completed a survey of data initiatives within the division (Addendum G). Some examples of the data created and maintained by FHS include the Childhood Lead Poisoning Surveillance System - a registry designed to collect, track, and analyze blood lead results; the New Jersey Cancer Education and Early Detection database – a system which stores data on individuals with breast, cervical, colorectal, or prostate cancer; and the Family Planning database – which is used to collect and analyze demographic and service statistics on family planning clients. Staff has linked the Hospital Discharge database (UB-90) to the EBC. These applications utilize the following software for data processing, storage, and analysis: Oracle, Access, FoxPro, and SAS.

SCAEIS personnel have expertise in the handling of large, complex, and confidential databases. Staff of SCAEIS are well versed in processing and analyzing data, utilizing various techniques such as linkage, editing, follow-up, and analysis. Staff are proficient in using multiple tools for data management, including SAS, Access and Epi Info. SCAEIS is directly responsible for data collection and management of many confidential databases. Examples include the SCHS Registry – a confidential database of over 130,000 children with birth defects and other special needs/conditions; the NHS database – a registry of children at risk for hearing disability; the Sickle Cell Tracking System – a longitudinal system for tracking the status of children having hemoglobinopathies; and the National Birth Defects Prevention Study – a large confidential database containing information on clinical, genetic, environmental and lifestyle factors before and during pregnancy from mothers of children with and without birth defects. The EIM staff have linked the SCHS Registry to the EBC and the electronic death files.

The establishment of the EHDI system will be the responsibility of the RS II (Addendum H). The RS II will assure that the EHDI system will collect basic information on all infants screened as well as diagnostic, referral, intervention, and follow up data. The RS II will be responsible for linking the EHDI system to the EBC, and other existing databases and tracking systems. The RS II will investigate the system used by the metabolic screening program, and integrate relevant components.

Existing managerial, program, and administrative staff in SCAEIS will support the efforts of the RS II. Resumes are in Biographical Sketches, organizational charts are in Addendum I.

Gloria Jones Grant, CSW, Director, SCAEIS: Ms. Grant, who is responsible for all Title V activities related to children with special health care needs, directs all activities of SCAEIS and will ensure that the newborn hearing program (NBH) will continue to be integrated into ongoing activities within SCAEIS, including reporting to the SCHS Registry, case management, and early intervention services.

Marilyn Gorney-Daley, D.O., M.P.H.: Dr. Gorney-Daley, Sr. Public Health Physician for SCAEIS, is responsible for directing several programs in SCAEIS including the Child, Adult and Special Services Program (CASS), which contains the NHP. Dr. Gorney-Daley will ensure continued collaboration between the NHP and other programs both within and outside of SCAEIS in addition to addressing any medical concerns on this project.

Elizabeth B. Congdon, RN, MA: Ms. Congdon is the Program Manager of Child and Adult Special Services, SCAEIS. The NHP is part of CASS. Ms. Congdon will have ultimate responsibility for ensuring that the goals and objectives of the proposed project are met within the specified timeframes. The RS II will report directly to Ms. Congdon.

Carol Hoffman-Sweeten, MS: Ms. Hoffman-Sweeten is the Coordinator, Specialized Pediatric Services, and has direct management responsibilities for the NHP. Ms. Hoffman-Sweeten will coordinate the efforts of the NHP with the RS II to develop the EHDI system.

Jane L. Hye, MS: Ms. Hye is the Public Health Consultant in the NHP. Ms. Hye has primary responsibility for implementation of the new hearing regulations, and will be working to ensure hospitals and providers fulfill their mandated responsibilities. Ms. Hye has extensive background in working with many of the key stakeholders in newborn hearing. All of Ms. Hye’s responsibilities will support the efforts of the implementation of the EHDI system.

Pamela Costa, MA: Ms. Costa is the Program Manager of the EIM program, and is responsible for mandated birth defects reporting and newborn biochemical follow-up screening. She is the Principle Investigator for the New Jersey Center for Birth Defects Research and Prevention. Ms. Costa was responsible for the development of the current SCHS Registry database and will work closely with the implementation of the EHDI system to ensure the integrity of the data system and successful linkages with existing databases.

Leslie M. Beres, MS: Ms. Beres is the Research Scientist I within EIM and has primary responsibility for the SCHS Registry, including database management, data analysis, surveillance, and data dissemination. She has extensive experience in linking and maintaining large, confidential data systems and will provide extensive consultation to the new RS II to ensure the successful development of the EHDI system.

Diane DiDonato, MPH: Ms. DiDonato is the Program Manager of the Family Centered Care Program, SCAEIS, and has responsibility for the SCHS Case Management Units and the Family-Centered HIV Case Management Network. Ms. DiDonato has extensive experience in working with parents of children with special health care needs, development of service networks, and care coordination.

Terry Harrison, Med, Part C Coordinator: Ms. Harrision directs the activities of the EIS, SCAEIS and is responsible for implementation of Part C of the Individuals with Disabilities Education Act. Ms. Harrison is directing the implementation of the EIS electronic management information system and supports the linking of the EHDI and EIS databases, to provide detailed information on service referral, utilization, and outcomes for children with hearing impairment.

ORGANIZATIONAL STRUCTURE & FACILITIES

The EHDI – New Jersey program will be carried out through the DHSS, Division of FHS, headquartered in Trenton, New Jersey, which has sufficient space to house this project. FHS consists of four service units: Maternal, Child and Community Health Services (MCCH), MCH Epidemiology, Women, Infants, and Children (WIC), and SCAEIS. These four service units work to improve the health, safety, and well being of families and communities in New Jersey. SCAEIS is comprised of four programs, all of which ensure the health of populations with special health care needs: Early Identification and Monitoring (EIM), Family Centered Care (FCC), Child and Adult Special Services (CASS), and Early Intervention System (EIS). Organizational charts are contained in Addendum I and show the functional organization of each of the Division’s service units.

EIM is responsible for maintaining the SCHS Registry, including mandated birth defects reporting and voluntary registrations of children with other health conditions. This program also includes Newborn Biochemical Follow-up which is responsible for tracking all newborns with abnormal screens and ensuring that these children are rescreened, and if found to be abnormal, in treatment. The Centers for Birth Defects Research and Prevention is also coordinated through this unit. All children with birth defects, including metabolic disorders and hearing loss, are included within the SCHS Registry. The FCC Program is responsible for the 21 county-based case management services and the HIV Family Centered Care Network. CASS, which includes the NHP, is also responsible for diabetes, renal and cardiovascular diseases, adult cystic fibrosis, Huntington’s Disease, chronic fatigue syndrome, specialized pediatric programs, including cleft palate, PKU follow-up, child evaluation centers, genetic, sickle cell and hemophilia programs and projects. In July 1993, SCAEIS was designated the lead agency for Part C of the Individuals with Disabilities Education Act (IDEA), EIS, when these services were transferred from the Department of Education. The case management units became the single point of entry in each county for Part H, providing service coordination for this system. Within a two year period, with the advice and assistance of the State Interagency Coordinating Council, a system of regional Early Intervention Collaboratives was developed to provide for a decentralized approach to the identification of needs and development of services appropriate to the cultural and socio-economic needs of each region. These collaboratives are now family driven, with 50% of the governing boards’ membership being family members.