Centers for Disease Control & Prevention EHDI Grants (2000):
Utah

GRANT ABSTRACT
• Grant Narrative

Title of Grant Program: Cooperative Agreement for Early Hearing Detection and Intervention (EHDI) Tracking, Research, and Integration with Other Newborn Screening Programs
Project Title: Refinement and Expansion of Utah's EHDI Surveillance and Tracking System
Organization: Utah Department of Health
Division of Community and Family Health Services
288 North 1460 West
Salt Lake City, Utah 84116 Phone: 801-538-6161
Project Director: George W. Delavan, M.D.

The goal of this Level II, Option 1 project is to improve the timeliness and appropriateness of Early Hearing Detection and Intervention (EHDI) services to infants and their families by:

1. refining and expanding Utah's existing surveillance and tracking system for EHDI; and
2. integrating the EHDI surveillance and tracking system with other relevant public health information databases and service systems, including, but not limited to, the Electronic Birth Certificate, Heelstick Screening, Birth Defects, Early Intervention, the Utah State Immunization Information System, and Women, Infants and Children.

The resulting system will also be used to assist with research, designed and conducted in conjunction with CDC and other recipients of Level II Cooperative Agreements, to improve EHDI systems. This research will address the costs, causes, benefits, and/or psychosocial effects on families of EHDI systems.

Over the past five years, the Utah Department of Health (UDOH) has made substantial progress in developing a public health information infrastructure. Most local health departments are now linked to the State's Wide Area Network, internet access about key health indicators is available and being expanded, an Electronic Birth Certificate has been developed, a statewide EHDI surveillance and tracking system has been implemented, and the Utah Immunization Information System has been created. What has not happened is the integration of those various components, along with other relevant databases and information systems, into an integrated system which can be used to improve services to children and families, make better public health policy decisions, and advance knowledge about children with special health care needs.

By creating electronic linkages among these various systems and providing procedures for secure internet access and transmission to authorized users, this project will improve the quality and effectiveness of health care services for children. Research to be conducted in collaboration with other recipients will advance our knowledge about issues related to EHDI services.

The project will be conducted by the UDOH Division of Community and Family Health Services, with assistance from the National Center for Hearing Assessment and Management (NCHAM) at Utah State University, the Early Intervention Research Institute (EIRI) at Utah State University, and the Division of Medical Genetics at the University of Utah.

Standard Form 424 i
Budget justification iv
Assurances and Certifications xiv
Narrative 1
STATEMENT OF THE PROBLEM 1
CURRENT STATUS OF EARLY HEARING DETECTION AND INTERVENTION IN UTAH 3
Historical context for Utah's Proposed Project 3
Computer-based EHDI Tracking and Data Management in Utah 5
Infrastructure and Commitment of Utah Department of Health to Support an Integrated EHDI Surveillance and Tracking System 9
Commitment of Utah Department of Health to electronic databases and integration 10
Availability of other resources to support the expansion of an EHDI surveillance and tracking system 12
GOALS AND OBJECTIVES 12
DESCRIPTION OF PROGRAM AND METHODOLOGY 14
Refining and Integrating Utah's EHDI Surveillance and Tracking System 16
Statewide implementation of Windows HI*TRACK 19
Linkages with Birth Defects Registry and Early Intervention 22
Operationalizing an effective and timely link with the Electronic Birth Certificate 23
Implementation of unique identifier for Heelstick, EBC, and EHDI 23
Option 1: Research on Costs, Causes, Benefits, and Psychological and Family Issues 24
Possible research project #1: Economic analyses of EHDI programs 25
Causes of congenital hearing loss 27
Collaborative planning, data collection, analysis, and interpretation 28
Collection of biological samples for children identified with hearing loss 29
EVALUATION PLAN 29
Review by advisory groups 30
Independent evaluations of project activities 31
COLLABORATION EFFORTS 31
STAFFING AND MANAGEMENT SYSTEM 33
ORGANIZATION STRUCTURE AND FACILITIES 34
HUMAN SUBJECTS REVIEW 34
APPENDICES 36
Appendix 1: References Cited in Proposal 36
Appendix 2: Abbreviated Resume for Proposed Staff 41
Appendix 3: Letters of Support 50
Appendix 4: Report of Utah's EHDI Tracking System for 1999 68
Appendix 5: Database Structure for Current HI*TRACK System 80
Appendix 6: Summary of Databases and Service Programs to be Linked with EHDI Surveillance and Tracking System 90
Appendix 7: Description of UDOH Maternal and Child Health Information
Internet-Query Module (MatCHIIM) 94
Appendix 8: Examples of Research Which Could be Included in Level II, Option 1 98
Appendix 9: Agencies Collaborating with NCHAM's National Technical Assistance System for EHDI 107
Appendix 10: Organizational Chart for Utah Department of Health 116

An average of 33 babies with congenital loss are born every day in the United States, making it the nation's most frequent birth defect.¹ Unfortunately, for many of these children, their hearing loss is not identified until they are 2-3 years old.² The relatively late age at which children with hearing loss are identified in the United States has been a concern among researchers, clinicians, and government officials for more than 50 years.³ However, until effective newborn hearing screening equipment and procedures were developed in the late 1980s, efforts to reduce the age of identification had been largely unsuccessful. The potential of those technological developments (i.e., equipment for using otoacoustic emissions [OAE] and automated auditory brainstem response [AABR]) was quickly recognized, and hundreds of hospitals have now implemented universal newborn hearing screening programs.⁴ In fact, hospital-based newborn hearing screening is rapidly becoming the standard of medical care throughout the United States, and many experts predict that within a few years, all babies will be screened for hearing loss before leaving the hospital.⁵

There is substantial research literature showing that hospital-based newborn hearing screening is feasible, relatively inexpensive, has reasonable specificity, and results in the identification of 2-4 babies with hearing loss per thousand births.^6-14 Unfortunately, even though currently available newborn hearing screening equipment can be very effective in identifying hearing loss, many babies with congenital hearing loss are being missed. The Achilles heel of existing early hearing detection and intervention programs is that an alarmingly high number of babies who are referred from hospital-based newborn hearing screening programs never receive diagnostic evaluations. As shown in Table 1, published reports of universal newborn hearing screening programs show that 25%-50% of the babies referred from hospital-based universal newborn hearing screening programs as needing diagnostic audiological evaluations are lost to follow-up before those evaluations are completed. Thus, many of the benefits envisioned for universal newborn hearing screening (UNHS) are not occurring.

*This prevalence is based on those children successfully followed. If all children had been followed, it would probably be higher.

Early Hearing Detection and Intervention (EHDI) programs in Utah mirror what is happening at the national level. Even though all hospitals in Utah are doing universal newborn hearing screening, many babies referred from screening programs are not currently receiving timely and appropriate diagnosis or intervention. The purpose of this Level II project is to build on past accomplishments and the existing infrastructure in Utah to develop a model EHDI surveillance and tracking system which will result in:

1. all newborns being screened for hearing and, where appropriate, receiving timely and appropriate diagnosis and intervention;
2. linkages with other relevant public health information data systems and programs to reduce duplication and ensure efficient, effective, and timely services for children and families; and
3. timely and relevant information for public health activities such as surveillance, research, and quality assurance.
4. Specific objectives of the project will be summarized after describing the current status in Utah with respect to EHDI programs.

Although the technology has been available for a number of years to operationalize an effective EHDI surveillance and tracking system as called for in Program Announcement 00076, the task is much more complex and time-consuming than is often realized. This is evidenced by the hundreds of millions of dollars spent during the last decade to create state-based immunization registries-which are just now beginning to be operational. Thus, for Level II states to be successful, it is critical that a mature statewide newborn hearing screening system already be in place, coupled with an operational computer-based EHDI tracking and data management system. The success of linking these EHDI components to the relevant components of the state's public health information and service system also depends on the degree to which those other components are already operational, the commitment of the Utah Department of Health (UDOH) staff, and the availability of other resources to assist in achieving the overall objectives of this project. This section summarizes the degree to which those components exist in Utah.

Historical Context for Utah's Proposed Project

Utah has been a leader since the early 1970s in developing innovative strategies for early identification of congenital hearing loss. Until 1993, UDOH operated a birth certificate-based high-risk screening program in which the risk factors for hearing loss recommended by the Joint Committee on Infant Hearing (JCIH) were incorporated as a part of the legally required birth certificate. Parents of babies who had any of these risk factors were sent a computer-generated letter and invited to obtain a free audiological diagnosis for their baby. Although often cited as a model program, it was not without its problems. First, it was difficult to get parents to bring their babies back for diagnostic evaluations.¹⁴ Second, research in the 1980s showed that only about half of all children with congenital hearing loss had any of these risk factors.^15-17 Consequently, in the early 1990s, the Utah Department of Health staff began exploring other options for early identifications of hearing loss.

As technological developments related to the measurement of otoacoustic emissions (OAE) and automated auditory brainstem response (AABR) made hospital-based universal newborn hearing screening feasible, researchers and public health officials in Utah were again among the nation's leaders in evaluating and implementing this new technology.^18-20 The first universal newborn hearing screening (UNHS) program in Utah using OAE was implemented in June of 1993 shortly following the National Institutes of Health (NIH) Consensus Development Conference, which recommended that all babies be screened for hearing loss before leaving the hospital. By the end of 1996, approximately 70% of all Utah babies were born in hospitals with UNHS programs. In 1998, the state legislature passed a law requiring all birthing facilities to have a UNHS program in operation by July 1, 1999,²¹ and hospitals reported that 98% of babies were screened from July 1, 1999 to December 31, 1999 (see Appendix 4).

It is important to recognize, however, that hospital-based screening is only the first step in a process to identify babies with hearing loss and provide them and their families with timely and appropriate services. Unfortunately, many hospitals in Utah have struggled making these connections. For the benefits of universal newborn hearing screening to be available to babies and their families, screening must be appropriately coordinated and linked with diagnostic and early intervention services in a way that is family-centered and culturally-competent. Throughout this proposal, the term "Early Hearing Detection and Intervention Program" will be used to refer to all components from hospital-based hearing screening to follow-up and tracking, diagnosis, and provision of medical, audiological, and educational services. It also includes the provision of culturally-competent family support services and providing for all children to be in a medical home. ⁽¹⁾

When the law requiring all hospitals to do newborn hearing screening was passed in 1998, a State Advisory Group, including representatives from hospitals, parents, professional and advocacy groups, and relevant state programs, was appointed by the Governor. In January 2000, this Advisory Group completed a needs assessment of the state's EHDI program in preparation for a funding proposal submitted to and subsequently funded by the Maternal and Child Health Bureau (MCHB). Table 2 summarizes the results of that needs assessment.

Computer-based EHDI Tracking and Data Management in Utah

Since the mid-1990s, hospitals with newborn hearing screening programs in Utah have been using a computerized tracking and data management system developed by the National Center for Hearing Assessment and Management (NCHAM) at Utah State University. This system, known as HI*TRACK, is a DOS-based computer program designed with suggestions from managers of dozens of hospital-based newborn hearing screening programs. Although the DOS-based system has worked well, it should be converted to a Windows-based system to make it easier to use and to expand its capabilities. This section describes the current DOS-based system and summarizes work currently underway to convert HI*TRACK to a Windows-based system.

The DOS-based HI*TRACK enables hospital staff to track each infant's status relative to hearing screening, diagnostic assessment, referral to follow-up services, and enrollment in intervention programs. Currently, HI*TRACK has fields for over 200 variables related to demographic, medical, and contact information about the baby and mother, results of screening and diagnostic measures, and status relative to diagnosis and intervention (see Appendix 5). All hospitals are required to report to UDOH the medical ID number, last name, date of birth, and screening results for every newborn. Additionally, the newborn hearing screening program coordinator at each hospital decides what other information is relevant to that hospital and establishes the required and optional fields. Data can be typed into HI*TRACK or can be transferred via machine-readable language from any of the newborn hearing screening devices produced by various manufacturers (e.g., Bio-logic, Grason-Stadler, Intelligent Hearing Systems, Madsen, Natus, Otodynamics, SonaMed, Starkey). The HI*TRACK software uses the data that has been entered to create a relational database. The HI*TRACK system enables people at the hospital to quickly:

• customize the software through user-defined codes and definitions to reflect the specific audiologists, screeners, physicians, and hospitals which are contributing data to that particular database;
• collect basic demographic information for all newborns screened and link that information to data about diagnostic and referral status for infants who need assessment or follow-up services;
• produce program management tools such as tickler lists of infants who need further screening or diagnostic evaluation, and descriptive summaries of individual screener's performance;
• produce standard and customized letters that communicate the results of screening and diagnostic procedures to parents and/or physicians;
• generate reports for any specified time period on the status of the early hearing detection and intervention program, including numbers and percentages of infants screened, missed, or lost during inpatient screening, referred for further audiological assessment, and found to have confirmed hearing lost; and
• create reports on user-defined variables of interest, such as infant birth weight, length of stay in intensive care units, etc.

Although the existing HI*TRACK system has been very useful for hospital-based newborn hearing screening programs, the DOS operating system limits future enhancements and prevents the system from achieving its full potential. In addition, user feedback collected over the past several years has identified new features that would make the system more responsive to the goals of Utah's EHDI program and allow data to be linked more efficiently with other relevant databases. Some of these new features include:

• ability to store information about all hearing screening and diagnostic tests for a particular baby, instead of just "the most conclusive results" for each test on each baby.
• a more powerful database that can support the secure exchange of newborn hearing screening data with other hospitals and other public health databases.
• electronic connections to other public health databases (e.g., Electronic Birth Certificates [EBC], Newborn Screening, WIC, etc.) so that health-care providers and program managers can have immediate and integrated access to these additional sources of information.
• capability of electronic communication of EHDI results with other service providers, such as Early Intervention (EI), Birth Defects Registry (BDR), Immunization.
• enhanced information-retrieval options and reporting capability.
• access to non-confidential or aggregate data by researchers.
• more flexibility in letter-writing capabilities.

To address these needs, the current DOS-based version of HI*TRACK is being converted to the Windows operating system. A beta version of the new Windows-based system will be implemented in ten hospitals beginning August 1, 2000 as a part of a small project funded by NIH. That project will also test the feasibility of using the new Windows-based HI*TRACK software at the state level. The state-oriented version of the HI*TRACK software (referred to here as the state EHDI database) includes all the functionality available to the hospitals, plus a variety of administrative features necessary for managing the exchange of information with the hospital and for assisting in the tracking and follow-up activities that have been so problematic in previous EHDI programs (see Table 1).

The NIH-funded beta test of the new Windows-based HI*TRACK system will serve as operational prototype to systematically evaluate the feasibility of a full-scale statewide EHDI system to be implemented as a part of the proposed project.

Infrastructure and Commitment of Utah Department of Health to Support an Integrated EHDI Surveillance and Tracking System

Utah is geographically large, and it is not uncommon to find small rural communities 30 or more miles apart. Of its 29 counties, 4 are urban and 25 have less than 100 persons per square mile. Public health in Utah is administered by the Utah Department of Health (UDOH) located in Salt Lake City and by 12 independent, autonomous local health departments located throughout the state. Among these 12 districts, there are over 50 offices and clinic sites, including at least one in every county.

Over the past five years, the Utah Public Health Information Network, which consists of a variety of databases and information systems that these local health departments both contribute to and access information from, has been emerging. Although not all components of the Utah Public Health Information Network are fully operational, the hardware/software infrastructure is in place and enough components are functioning to provide an excellent foundation for the activities proposed in this Cooperative Agreement. Each of the local health departments is electronically connected to UDOH through the state's Wide Area Network or via modem. The Wide Area Network uses Frame Relay and TLS. It supports IPS, Appletalk, and TCP/IP. T1 lines are installed to each site containing a file server and a Timeplex router is installed using ISPF routing.

Commitment of Utah Department of Health to electronic databases and integration. As the potential for public health information systems expands with improved technology, there is an urgent need to integrate the various sources of information and to ensure that such systems are appropriately governed by uniform data standards, communication networks, and policy level agreements regarding confidentiality, data access, sharing, and reduction of the burden of collecting data. Because of past work, UDOH is in an excellent position to address these issues and create an effective EHDI surveillance and tracking system which is a part of such a broad-based integrated public health information system. In 1998, the statutory authority for the screening of metabolic diseases (hereafter referred to as Heelstick Screening) was expanded to include hearing screening of all Utah newborns. Both the Heelstick Screening and EHDI programs fall within the administrative structure of the Bureau of Children with Special Health Care Needs. Both screening programs have a long history of cooperation and data sharing with the Office of Vital Records and Statistics. This cooperation is evidenced by the fact that in 1978, Utah was the first state in the nation to include a question concerning a family history of hearing loss on its birth certificate. Under a secure protocol established by Executive Order of the Governor in 1982, Utah birth certificates are linked across generations. For many years, the newborn population estimates used by these two newborn screening programs in Utah's Maternal and Child Health Block Grant Applications and Annual Reports have been based on birth certificate records.

In 1995, UDOH successfully created the first public health data information Internet query system in the nation (now referred to as MatCHIIM, or the Maternal and Child Health Information Internet-Query Module-see Appendix 7). The U.S. Public Health Service Data Policy Coordinating Committee recommended the UDOH Internet query system as an innovative example of how states were using new information technology to distribute data.

In 1998, UDOH adopted and implemented a core of data element standards to facilitate electronic data interchange used within UDOH by data analysts, program managers, and service providers. These standards were identified and developed by the Department Information Systems Standards and Integration Committee chaired by the Director of Vital Records and Statistics. Standards are based on the ASC X12 national standards of the American National Standards Institute (ANSI). In addition to the X12 standards, UDOH has also utilized the ANSI HL7 national standard in developing the Utah Statewide Immunization Information System (USIIS) architectural definitions for connectivity. On July 1, 1999, a new administrative rule, Submission of Data through an Electronic Data Interchange, became effective. This rule provides legal authority for the submission of information to UDOH through an electronic data interchange.

UDOH has also established a set of business principles, one of which calls for UDOH "to strive to provide an integrated view of its services to all its customers" and "to strive to provide an integrated view of its customers to all its employees." To conform with these principles, UDOH is working to integrate existing information systems that will allow customers and staff to obtain the information they need, regardless of where it is collected. UDOH has already started the process with the Utah Statewide Immunization Information System (USIIS), which includes a central registry database to exchange current demographic and immunization information with local public and private databases and registries. The database is presently being populated with a combination of birth certificate data and Women, Infants and Children (WIC) records. When the statewide system becomes fully operational (projected by October 1, 2003), USIIS will provide controlled confidential web-based Internet access for qualified users to individual immunization information in a central registry linked to local public and private databases and registries.

Availability of other resources to support the expansion of an EHDI surveillance and tracking system. The proposed project will be assisted by work funded by several other federally-funded projects which are focusing on closely related areas. Resources requested for this project will not be used to supplant those funds or any existing state resources. Work funded by other projects which are relevant to the proposed project is summarized in Table 3.

This Level II, Option 1 project has goals in two areas:

1. Improve the timeliness and appropriateness of EHDI services to infants and toddlers and their families by:

1. refining and expanding Utah's state-based surveillance and tracking system for EHDI;
2. integrating the EHDI surveillance and tracking system with other relevant public health information databases and service systems, including, but not limited to, Vital Statistics (Electronic Birth Certificate), Newborn Screening for Inborn Errors of Metabolism (referred to hereafter as Heelstick Screening), Birth Defects, Early Intervention, the Utah State Immunization Information System (USIIS), and Women, Infants, and Children (WIC).

2. Using the resulting system to assist with research, designed and conducted in conjunction with CDC and other recipients of Level II Cooperative Agreements, to improve EHDI systems. This research will address the costs, causes, benefits, and/or psychosocial effects on families of EHDI systems.

Specific objectives for the first goal consistent with those outlined in Program Announcement 00076 are shown in Table 4. Specific objectives for possible research projects related to goal #2 are discussed in the Methodology section.

This project will be implemented in Utah where there are presently 43 birthing hospitals and one non-birthing tertiary care center for infants who are required by state law to screen all babies for hearing prior to discharge. By state law, hospitals must also report the results of screening to UDOH, and 99% of the births in 1999 were at hospitals which use the HI*TRACK newborn hearing screening tracking and data management software. UDOH uses the same software and electronically merges data from each hospital into a state EHDI database. In 1999, hospitals reported to UDOH that 98.0% of 44,047 births were screened.

In 1999, 85% of births were Caucasian, 10% were Hispanic, 2.9% were Asian or Pacific Islander, 1.5% were American Indiana, and .6% were African-American. The target population for this project is all babies born in Utah and their families. A particular focus of the project will be those infants and families for whom the current EHDI system is not working well. These are primarily families living in the rural/remote areas of the state and ethnic minorities, particularly those where language is a problem. The research components of the project will do sampling to ensure adequate representation from the rural/remote areas of the state, equal numbers of males and females, and as many babies as possible from ethnic minorities.

1. Collaborate with other state programs, private service programs, and advocacy groups to build a coordinated EHDI infrastructure which can be used to refine existing EHDI surveillance and data tracking system to achieve the following standards:

1. 99% of all newborns in Utah will be screened for hearing loss before being discharged from the hospital.
2. Infants who do not pass the screening test will receive appropriate audiological evaluation by 3 months of age.
3. Infants identified with a hearing loss will be enrolled in an appropriate intervention and receive appropriate specialty health care (including genetics counseling where appropriate) before 6 months of age.
4. The EHDI statewide data management system will be electronically linked to other relevant public health databases such as Vital Statistics, Birth Defects, Early Intervention, Immunizations, and WIC, to better coordinate services, reduce the number of children lost to follow-up, and provide better data for public health purposes.
5. Less than 10% of infants who fail the screening process will be lost to follow-up before they complete the diagnostic procedures.
6. All infants screened for hearing loss will have a medical home.
7. Families of all newborns will be provided with culturally-competent support as part of the screening, referral, diagnosis, and early intervention enrollment process.
8. EHDI services will be organized so that families can use them easily, can participate in decision making at all levels, and are satisfied with the services they receive.

2. Develop electronic or web-based standardized data collection and tracking methods, forms, data analysis plans, and procedures for security, confidentiality, and informed consent where necessary.

3. Refine the existing mechanisms to collect standardized EHDI data (including the type of hearing loss and type of intervention services) from appropriate sources, such as birthing hospitals, diagnostic centers, and/or intervention programs.

4. Collaborate with State programs such as Early Intervention, the Parent Infant Program, and the Utah Center to ensure infants and young children identified with hearing loss receive timely, appropriate, and family-friendly services.

5. Develop mechanisms to identify and collect standardized data on infants/children with late onset or progressive hearing loss.

6. Use the EHDI Surveillance and Tracking System to obtain 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 birthing hospitals, false positive rates, and loss to follow-up rates.

7. Integrate and enable two-way interaction between the EHDI system and other screening programs (e.g., Newborn Screening, Lead Screening, Birth Defects Registry, EPSDT, and WIC) and service programs (e.g., early intervention, immunizations, neonatal follow-up).

8. Implement an evaluation plan to monitor progress on activities, document concerns from parents and professionals about the EHDI process, and assess the timeliness, completeness, and success of the project.

This section will first discuss the activities for refining and integrating ⁽²⁾ Utah's EHDI surveillance and tracking system and will then discuss our proposed approach to the collaborative research activities under Option 1.

Refining and Integrating Utah's EHDI Surveillance and Tracking System

Refining and expanding the current EHDI surveillance and tracking system and integrating it with other public health data and service systems will require the active participation of many state agencies, private service providers, and advocacy groups. Groups which are committed to working on the goals of this project are shown in Figure 1. Appendix 3 contains letters of support, and Appendix 6 contains a brief description of each of these groups with a summary of how they will contribute to the expanded and integrated system.

The number of different groups in Figure 1 who provide services relevant to EHDI emphasizes the importance of coordinating activities, reducing duplication, and making information available to authorized people who need it. For example, work done previously by Project Bridge to link the USIIS and WIC data systems will be expanded to include linkages with EHDI and BDR. In addition, the recently developed Electronic Birth Certificate (EBC) is already being used to "populate" the Heelstick Screening database, and this project will expand this to the USIIS and EHDI databases. Use of the EBC will not only reduce the burden of data collection, but will provide more points of contact, provide parents and physicians with more timely and complete information, and assist in location and follow-up of "lost" children. Timely availability and reporting of data will also lead to improved services.

During the first half of the five-year project, the focus will be on linking and coordinating programs shown in the inner circle of Figure 1 (EHDI, USIIS, WIC, BDR, Heelstick, EI, EBC) and linking those with UDOH and the local health departments. During the second half of the project period, these linkages will be expanded to other databases and service providers, examples of which are shown around the outside of the inner circle in Figure 1 and described more fully in Appendix 6.

Specific examples of the benefits of integrating and linking EHDI, USIIS, WIC, BDR, Heelstick, EI, and EBC are given below:

In Utah, approximately 10% of the infants do not pass the initial screen administered in the hospital and have to come back for an outpatient screen within the first few weeks of life. Babies who do not pass the outpatient (about 1% of the total population) need ongoing diagnostic assessments. The biggest challenge in an EHDI program is getting children to come back for these subsequent appointments. Having EHDI linked to the Immunization and WIC databases would mean that these children could be flagged, and parents could be reminded when they come in for immunizations or WIC appointments.

The electronic birth certificate contains substantial information about health status variables that are thought to be linked to congenital hearing loss (APGAR scores, very low birth weight, congenital infections, etc.). Linking the electronic birth certificate to the EHDI database would allow better analyses of those variables than has previously been possible.

Congenital hearing loss is a birth defect which is not currently included in the Birth Defects Register. By linking EHDI with BDR, this information could be routinely added.

Health status variables included on the electronic birth certificate are also relevant to epidemiological studies concerning birth defects. Linking the EBC with the BDR would allow such studies to be done.

Although the timing and procedures for follow-up in Heelstick Screening are much different than those for EHDI, the Heelstick system has been operating successfully for many years, and there is much EHDI can learn from it. For example, the EBC is now being used to populate the Heelstick database-similar procedures will be used for EHDI. By linking Heelstick and EHDI to USIIS, authorized primary care providers will have access to results for individual children as needed.

Linkages with EBC would also allow geographic mapping of various conditions to be done. Such mapping can be used for epidemiological work, as well as health care delivery analyses.

By linking EHDI to the Early Intervention database, the chances that children diagnosed with hearing loss will "fall through the cracks" are reduced substantially. Because the linkage will be two-way, EI staff can also update records so that the EHDI Registry of Children with Hearing Loss will be more current and accurate.

Almost half of the babies born in Utah are eligible for the WIC program, and even parents who are unlikely to return for follow-ups to hearing screening are likely to come in for their WIC appointments. Therefore, WIC provides an excellent contact point to remind parents about the need for follow-up to the hearing screening.

By linking Heelstick, EHDI, the Electronic Birth Certificate, Early Intervention, Lead Screening, and Neonatal Follow-up, a large number of health outcome indicators and risk factors will be brought together for the entire population of children born in Utah. This information can then be linked with other information about early identification of children with genetic conditions and special health needs. This combined profile will be used to create an electronic community-based child health profile which will be available to authorized health care providers for evaluating and improving an individual child's personal health care.

Obviously, there are substantial benefits to linking hospitals to a statewide EHDI tracking and data management system that is capable of linking to other public health information systems. Not only will babies and their families receive better and more timely services, but public health agencies will have information for timely monitoring, quality assurance, and program improvement. In addition, the integrated database can substantially expand what we know about the etiology and epidemiology of hearing loss.

Table 5 lists the activities to be conducted during each year of the project. The first year will focus on the statewide implementation of the new Windows HI*TRACK program, the addition of hearing loss to the BDR, developing a timely link between EHDI and EBC, and beginning the implementation of a unique identifier system for all newborns.

Statewide implementation of Windows HI*TRACK. The implementation of the Windows-based HI*TRACK program is a good example of how this project will "dovetail" with and benefit from other funded projects described earlier in Table 3. By the time this project is started, the beta test of Windows HI*TRACK funded by the NIH project will be almost completed. Similarly, the "data integration assessment" work conducted by the Genetic Services and Data Integration (GSDI) and NIH projects will have produced design specifications to address the following issues:

*Includes conversion to better back-end database, better security, authentication, encryption, revisions based on user groups.

Back-end database. The prototype system being beta-tested now as a part of the NIH project uses Microsoft's ACCESS product for both the front-end and the back-end databases. The reason ACCESS is used is that it is completely adequate for a database with up to 100,000 active records, and there are no additional license fees for the hospital to use the ACCESS database. The ACCESS database is also sufficient as a back-end database for limited statewide use. As more records are added to the system, a more sophisticated back-end database will be required. We anticipate moving to a back-end database like Oracle during the latter part of Year #1. Because Oracle is completely compatible with ACCESS, hospitals will be able to continue to use the cheaper, but completely adequate, ACCESS database for their needs, and the hospital data will be merged into the more powerful back-end Oracle database at the state level. Because the Utah Department of Health already has an Oracle license, this approach allows complete testing of the concept without wasting resources.

Security and confidentiality protocols. Hospitals in Utah currently transfer information to UDOH without any special encryption or security. The Department of Health and Human Services provides guidelines for encryption of medical data. The design document developed by the NIH project by January 2001 will be used to implement procedures for user authentication, encryption of data as it is transferred, and other appropriate safeguards. A secure Internet site for later use in the project will also be implemented so health professionals can access and submit information.

Data transmission protocols and information access/update features. Currently, hospitals send HI*TRACK data to UDOH using disks or email. The UDOH version of HI*TRACK has procedures for determining whether information on those disks is updated information for an existing child in the database or information for a new child. During the first year, additional work will be done to determine the requirements for transferring information using the Internet. Obviously, such transmission will require more work on security and confidentiality, but other issues involving data transmission need to be evaluated. For example, infants who do not pass the hospital-based screening should be referred to community-based audiologists for diagnostic assessments. The results of those diagnostic assessments should also be maintained in the EHDI tracking and data management system. With appropriate security measures, it would be possible for audiologists to have direct access to the database to submit updated diagnostic information. Similarly, physicians who are responsible for those children may have information to be submitted to the database. Expecting audiologists or physicians to have software similar to what is maintained by the hospitals is unrealistic. However, an Internet-based system in which physicians and audiologists could submit information to the system is technically possible. It would also be beneficial for physicians and audiologists to query the database regarding a particular child to determine which screening and diagnostic tests have been done, whether a child is overdue for certain tests, and what action should be taken next. The design specifications for creating such an Internet-based system will be developed as a part of the NIH project, and implementation activities will begin during Year #1.

Enhancements to the Windows-based software. On a continuing basis, the project team will gather and document ideas for enhancements to the Window-based software. The first such information will come from the ten hospitals doing beta-testing as a part of the NIH project. Suggested enhancements will be prioritized and integrated into a system for regular upgrades. It is expected that work on these aspects will be more intense during the first three years of the project. Design specifications will be generated from a user group consisting of representatives from hospital test-sites, audiologists, primary-care physicians, hospital administrators, and public health officials. The group will include hospitals who use different newborn hearing screening protocols and equipment, serve different populations, and have different kinds of facilities.

Linkages with Birth Defects Registry and Early Intervention. As noted earlier, even though hearing loss is the most frequent of all birth defects, it is not currently included in the Utah Birth Defects Registry (BDR). With partial support from this project, the Director of BDR has agreed to add congenital hearing loss as one of the conditions. This will result in several benefits. First, very little is known about the cause of many birth defects, but it is clear that they are often correlated, and the ability to cross reference information about congenital hearing loss with information already in BDR will contribute much to our understanding of the etiology of hearing loss. Certain birth defects may also be predictive of late-onset hearing loss,²² and the linkage of EHDI and BDR will help ensure that children with late-onset hearing loss are identified as soon as possible. By connecting BDR and EHDI, it will also be possible to provide authorized caregivers with more information once the system becomes Web-enabled.

Adding EHDI information to BDR and linking the two systems will require hiring and training a part-time abstracter for the birth defects system and revising the BDR database, which is now programmed in a rudimentary "file maker" DOS-based system. A new ACCESS database will be designed, including the procedures necessary to automatically convert the existing data into the new system. It is expected that this work can be completed and the two systems can be linked by January of 2001.

Operationalizing an effective and timely link with the Electronic Birth Certificate (EBC). Although Vital Statistics is already sharing EBC information with the EHDI data and tracking system, this information is extracted from the EBC database and sent as a paper report to the manager of the EHDI database. Although this report has been useful, it would be much better to have a two-way electronic connection between the two systems. If that were done, the EBC could be used to populate the EHDI database so that newborns missed in the hospital could be quickly contacted. In addition, the names of babies born at home could be shared in a more timely manner with the EHDI data management system so that follow-up screening could be done for these babies. Finally, the EBC contains valuable information about maternal and neonatal health risk factors which could be used in following up with high risk babies and conducting research to improve health care delivery and epidemiological studies.

Work done by GSDI and the MCHB Universal Newborn Hearing Screening project has already determined that the data structures of the two systems are compatible. Programmers for the two systems will collaborate with each other in developing a two-way transfer protocol, pilot test it off-line, and then implement a prototype of the system. Once this is done, the user interface will be developed for the program managers in each system, and procedures for security and authentication will be developed. It is expected that this work can be completed by June 1, 2001.

Implementation of unique identifier for Heelstick, EBC, and EHDI. One of the biggest problems in maintaining and integrating public health data systems is identifying duplicate records because of different names being used, incorrectly entered information, or babies seen by different providers. Having a unique numerical identifier for every baby in the state would assist in solving this problem, although it is not a complete solution since numerical identifiers can also be mistyped. However, a unique numerical identifier for each newborn would increase the "hit rate" for probabilistic matching systems which are currently used in the various databases.

As a part of the State Systems Development Initiative (SSDI) project described in Table 3, a pilot test is currently underway to use the numerical code on the Heelstick Screening filter paper as a identifying number for newborn hearing screening and the Electronic Birth Certificate. This pilot test will be completed by October 31, 2000. The results of that pilot test will be used by this project to make refinements to the system and begin implementing it statewide. This implementation will happen in an iterative process with adjustments and improvements being made at each stage of the process. Because of the nature of the task, each iteration will require at least six months to determine whether the unique numerical identifier is working as anticipated. Depending on the success of the pilot project, we anticipate being able to implement the more populated areas of the state (accounting for approximately 80% of all births) during the first year of the project, and then expanding the system to the remainder of the state during the second year.

Option 1: Research on Costs, Causes, Benefits, and Psychological and Family Issues

Program Announcement 00076 specifies that recipients of Level II, Option 1 awards are to:

"collaborate with other Level II recipients and with other federal and national agencies to . . . develop a set of core research questions and analytic guidelines for [research] related to the costs of EHDI programs, the causes of congenital hearing loss, the benefits of early identification of hearing loss, and/or psychological and family issues associated with EHDI."

Because the research to be conducted will not be specified until this collaborative planning occurs, we have summarized in Table 6 the strengths and experience of the team proposed for this project in each of the four areas. The remainder of this section briefly summarizes two possible research projects which could be conducted as a part of this collaborative effort and addresses other related issues. A more complete description of each of these projects is in Appendix 8.

Possible research project #1: Economic analyses of EHDI programs. Interestingly, there have been very few systematic cost analyses done of EHDI programs, and those that have been done have been incomplete, have suffered from serious methodological issues, have been based primarily on hypothetical data, or have evaluated protocols and equipment that are no longer used.^23-31 This project will use state-of-the-art economic analysis techniques to conduct a series of complete economic analyses of the screening, follow-up, and diagnostic components of eight hospital-based EHDI programs in Utah. Using data from interviews, hospital records, time diaries of staff, and observations, a complete economic analysis will be done for all aspects of each program. Based on interviews with parents, audiologists, and physicians for a sample of children identified in each of the sites, estimates will also be made of the costs associated with diagnostic services. The sample of parents will include those whose child: a) failed the inpatient screen, but passed the outpatient screen; b) failed both the inpatient and outpatient screens, but passed the diagnostic evaluation; and c) failed the screenings and the diagnostic evaluation (i.e., has a hearing loss). In addition to cost data, information will be collected during these interviews with family members about the effects of screening and diagnostic procedures on parents' concerns about relevant psychological and family issues (e.g., anxiety, bonding, family functioning).

Analyses will incorporate state-of-the-art economic analysis techniques,^32-34 such as sensitivity analysis, discounting, shadow pricing, and cost-distribution procedures. The resulting information will provide cost-effectiveness data of EHDI programs which will be comparable across programs, since the same data gathering techniques and assumptions will have been used in each. Such information will enable program administrators and policymakers to consider cost issues in making decisions regarding the implementation and improvement of EHDI programs.

Causes of congenital hearing loss. The etiology of moderate to profound sensorineural hearing loss has historically been estimated through studies of children with hearing impairments attending school settings.³² The conclusions from such analyses are confounded by the effects of undetermined late-onset loss due to factors such as meningitis, use of ototoxic drugs, or cytomegalovirus. Fortunately, now that newborn hearing screening is becoming the standard of care, we are in a much better position to determine etiology.

The goal of this proposed research project is to determine the etiology categories of hearing loss in infants by using genetic diagnostic and counseling consultation for all families of infants with congenital hearing loss identified through Utah's EHDI programs. Each family will be offered a consultation with a clinical geneticist and genetic counselor at no charge. Standard clinical data, including perinatal history, detailed three-generation pedigree, and physical examination will be used to identify any syndromes (expected to be less than 10%). Family tree analysis (which is much easier to do and much more conclusive in Utah because the average family size is much larger and many families have extensive genealogical data) will most likely identify that 25-30% of the families have a close relative with similar hearing loss identifying a specific genetic transmission (autosomal dominant, autosomal recessive, x-linked, or mitochondrial). In addition, routine urinalysis will be done to test for conditions such as cytomegalovirus. Using these procedures, children who have well-established environmental or genetic causes will be identified. All families will be offered a Connexin 26 analysis^33,34 at no charge based on a small sample of blood drawn from the baby.

Given the infrastructure and resources which already exist in Utah, such a study can be conducted for a relatively small amount of money. Over the course of this five-year project, it is projected that at least 300 children will be included in the analysis, making it substantially larger than other studies which have reported on the causes of congenital hearing loss.³²

Collaborative planning, data collection, analysis, and interpretation. The partners proposed for this project (UDOH, NCHAM, EIRI, and the Division of Medical Genetics at the University of Utah) will work collaboratively with other agencies and recipients to develop multi-site research questions and analytic guidelines. Although two specific research projects are proposed as examples of what could be done, the research portion of this project will be modified however necessary to accommodate multi-site projects designed by the collaborators. For budgeting purposes, about one-third of the total budget has been allocated for collaborative research activities and two-thirds for refining and expanding the EHDI surveillance and tracking system. Of course, the proposed research is only possible because of data that will be available from the expanded EHDI surveillance and tracking system. Thus, the proportion allocated to research could be viewed as being substantially higher.

Another strength of this proposal is that the co-director for the project (Dr. Karl White) is also the principal investigator of a four-year grant from MCHB for a National Technical Assistance System for EHDI. This TA System is already collaborating extensively with national organizations involved with EHDI issues, such as the American Academy of Pediatrics, the American Speech-Language-Hearing Association, the Directors of Speech and Hearing Programs in State Health and Welfare Agencies, the Alexander Graham Bell Association, Family Voices, and the Joint Committee on Infant Hearing. Eighteen such national organizations have committed to work collaboratively with the National TA System to assist in the expansion and refinement of EHDI programs in the United States. The existing relationship NCHAM has with these organizations will be a valuable benefit in obtaining input from these groups in developing "core research questions and analytic guidelines." A description of how those collaborating agencies are working with NCHAM is included as Appendix 9.

Collection of biological samples for children identified with hearing loss. Depending on the decisions made about the specific collaborative research to be conducted, the project is planning to collect a sample of blood from each infant identified with a congenital hearing loss (assuming parents provide written informed consent). Procedures for doing this will be decided in concert with CDC and other recipients. Using stored blood from Heelstick Screening is probably not a possibility because Utah, like many other states, only stores these samples for three months, and a significant number of children with congenital hearing loss will not complete the diagnostic procedures by three months of age.

Because this project is addressing issues in a rapidly evolving area of technology, a comprehensive evaluation component will be needed to guide mid-course corrections as technology improves and the needs of participating groups change.

Accomplishment of stated objectives. For purposes of the evaluation, the objectives contained in Table 4 will be specified in more detail at the beginning of each year and included in the annual plan which must be submitted to the Division Director for all projects within the Community and Family Health Services Division of UDOH. The management team for the project (Dr. Delavan, Dr. White, Dr. Mahoney, and Dr. Clyde) will be responsible for developing this plan and monitoring whether activities are being implemented in a timely manner and the objectives are being accomplished.

Specific objectives for activities in Year #1 are included in Table 7, indicating the start and completion dates and the tracking evaluation methods to be used for each one. Evaluation data summarized in this table will be collected by the information analyst at UDOH and the graduate research assistant at Utah State University, who will both work under the direction of Dr. White. Results of these evaluation activities will be reported each month at the management team meetings. Obstacles which are interfering with the successful accomplishment of activities will be identified, possible solutions discussed, and plans and timelines adjusted as necessary.

Review by advisory groups. The following two advisory groups, which are already functioning, will also play a key role in monitoring and evaluating the activities of this project:

As a result of other funded projects, UDOH has already established a Child Health Advanced Record Management (CHARM) team. This working group currently includes representatives from Heelstick Screening, EHDI, and EBC. As a part of this project, CHARM will be expanded to include representatives from USIIS, EI, and BDR. The CHARM team will meet at least quarterly to review progress being made by this project and the other projects listed in Table 3, coordinate activities, resolve problems, and identify resources necessary to move forward.

The Governor has appointed a State Newborn Hearing Screening Advisory Committee consisting of 12 members who represent audiology, early intervention, family practice physicians, pediatricians, neonatology, health insurance companies, public health, and parents. The Committee Chair has agreed to include the evaluation of this project as a part of the Committee's agenda at each of their quarterly meetings (see letters of support). At the beginning of the project, a copy of the proposal will be sent to all Committee members, and a member of the management team will make a report at each Committee meeting on the progress made on each of the objectives. The Committee will submit a brief annual report on the status of the project to the CDC project officer.

Independent evaluations of project activities. As a part of the Universal Newborn Hearing Screening Initiative funded by MCHB, an independent evaluation of Utah's EHDI program will be conducted each year by the Research and Evaluation Methodology Program at Utah State University. This evaluation will include onsite visits to hospitals, questionnaires and interviews with parents, hospital staff, and other stakeholders, and examination of archival records and databases. Activities funded by this project will be included as a part of those evaluation activities. Funds have been budgeted to continue to contract with this evaluation team to do the evaluation for one additional year after the MCHB project is completed.

In order to develop effective EHDI surveillance and tracking system which is integrated with other relevant public health databases and service systems, it is necessary to collaborate with a large group of public health and private groups. The first half of this project will focus primarily on refining the existing EHDI surveillance and tracking system and developing linkages with EBC, USIIS, Heelstick Screening, BDR, EI, and WIC. The second half of the project will expand these linkages to other relevant groups using the USIIS system as the backbone of that integrated system. The various groups who have agreed to participate in this effort were depicted earlier in Figure 1 and are described more fully in Appendix 6. The goal is to have a system in which all authorized service providers, including hospitals, diagnostic centers, early intervention programs, and primary care physicians have access to the information they need to provide fast, friendly, and effective services to children and their families.

In conducting the research efforts associated with Option 1, the project team is also committed to collaborating with other Level II recipients and other federal and national agencies in developing a set of core research questions and analytic guidelines. Other work of the offerer in developing collaborative relationships with national organizations which are concerned with EHDI is summarized in Appendix 9.

The project will be under the direction of Dr. George Delavan, who directs the Division of Community and Family Health Services within the Utah Department of Health. As shown in the organizational chart in Appendix 10, most of the public health programs relevant to this project are in Dr. Delavan's Division. The day-to-day operations of the project will be coordinated by a management team consisting of Dr. Delavan, Dr. Karl White at Utah State University, Dr. Thomas Mahoney who is the Manager for Hearing Speech and Vision Services in Dr. Delavan's Division, and Dr. Stephen Clyde, Associate Professor of Computer Science at Utah State University. The management team will meet on a monthly basis to review the progress of the project, make necessary adjustments, and develop strategies to accomplish the goals of the project.

Dr. White will oversee those aspects of the project related to research and evaluation and the programmatic aspects of EHDI surveillance and tracking. Dr. Mahoney will oversee those aspects of the project related to provision of EHDI services to families. Dr. Clyde will oversee those aspects of the project related to computer programming, database design, and database integration, which is his area of expertise on the faculty at Utah State University. Curriculum vita for each of these people is included in Appendix 2 and demonstrates the specific experiences each member of the management team has had in the areas for which they will be responsible.

Additional members of the team have been selected because of their expertise in relevant areas or will be hired specifically for this project. Depending on which research projects are eventually selected for the collaborative research projects in Option 1, Dr. Goetze and Dr. Carey have extensive expertise and experience as demonstrated by their vita in Appendix 2. Other project staff (i.e., computer programmers, information analysts, abstracters, graduate research assistants, and clerical help) will be hired using established procedures at the Department of Health or Utah State University.

The project will be located within the Division of Community and Family Health Services directed by Dr. Delavan. As shown in the organizational chart in Appendix 10, most of the public health programs that will be collaborating with this project are also located in Dr. Delavan's Division, which will facilitate communication and cooperative work. Past accomplishments of UDOH and NCHAM in establishing an effective EHDI system in Utah demonstrates the adequacy of the facilities and resources necessary for this kind of a project. Administrators at UDOH and Utah State University have committed appropriate space and equipment to support the functions of the project.

As soon as funding is approved, the proposed work will be submitted to Institutional Review Boards at the Utah Department of Health and Utah State University. It is believed that the work related to the EHDI surveillance and tracking system will be exempt from Protection of Human Subject regulations [45 CFR 46.101(b)] according to Exemption #5 because the work will be refining surveillance and tracking procedures used to improve existing public health services in the state. The work will be conducted subject to the approval of department and agency heads, and hospitals are only being asked to submit data to the state which is already covered by state law or regulation. In later phases of the project as linkages are made to allow private providers to have access to the system, submissions will be made to the Institutional Review Boards to assure appropriate protection of human subjects.

Depending on the specific research projects selected in collaboration with other Level II recipients and federal agencies, it is anticipated that Institutional Review Board approval of the work will be required. Until the specific research questions are selected, however, it is not possible to have this work reviewed.

1. White, K. R. (1996). Universal newborn hearing screening using transient evoked otoacoustic emissions: Past, present, and future. Seminars in Hearing, 17(2), 171-183.

2. U.S. Department of Health and Human Services (HHS). (1990). Healthy People 2000: National Health Promotion and Disease Prevention Objectives. Washington, DC: Public Health Service.

3. Mauk, G. W., & Behrens, T. R. (1993). Historical, political, and technological context associated with early identification of hearing loss. Seminars in Hearing, 14(1), 1-17.

4. National Center for Hearing Assessment and Management (2000). Universal newborn hearing screening programs in the United States. World Wide Web, https://www.infanthearing.org/status/programs_in_us.html.

5. White, K. R., Mauk, G. W., Culpepper, N. B., & Weirather, Y. (1998). Newborn hearing screening in the United States: Is it becoming the standard of care? In L. Spivak (Ed.), Universal newborn hearing screening (pp. 225-255). New York: Thieme.

6. Vohr, B. R., Carty, L. M., Moore, P. E., & Letourneau, K. (1998). The Rhode Island Hearing Assessment Program: Experience with statewide hearing screening (1993-1996). The Journal of Pediatrics, 133(3), 118-119 and 353-357.

7. Mehl, A. L., & Thomson, V. (1998). Newborn hearing screening: The great omission. Pediatrics, 101(1), http://www.pediatrics.org/cgi/content/full/101/1/e4, pp. 1-6.

8. Barsky-Firkser, L., & Sun, S. (1997). Universal newborn hearing screenings: A three-year experience. Pediatrics, 99(6), http://www.pediatrics.org/cgi/content/full/99/6/e4, pp. 1-5.

9. Finitzo, T., Albright, K., & O'Neal, J. (1998). The newborn with hearing loss: Detection in the nursery. Pediatrics, 102(6), 1452-1460.

10. Mason, J. A., & Herrmann, K. R. (1998). Universal infant hearing screening by automated auditory brainstem response measurement. Pediatrics, 101(2), 221-228.

11. Johnson, J. L., Kuntz, N. L., Sia, C. C. J., & White, K. R. (1997). Newborn Hearing Screening in Hawaii. Hawaii Medical Journal, 56, 352-355.

12. Prieve, B. A., Stevens, F. (2000). The New York State universal newborn hearing screening demonstration project: Introduction and Overview. Ear & Hearing, 21(2), 85-91.

13. White, K. R., & Behrens, T. R. (Eds.). (1993). The Rhode Island Hearing Assessment Project: Implications for universal newborn hearing screening. Seminars in Hearing, 14(1).

14. American Academy of Pediatrics Task Force on Newborn and Infant Hearing. (1999). Newborn and infant hearing loss: Detection and intervention. Pediatrics, 103(2), 527-530.

15. Mahoney, T.M., & Eichwald, J. (May 1987). The ups and downs of high-risk hearing screening. Seminars in Hearing; Hearing in Infants, 8(2), 155-163.

16. Elssmann, S. F., Matkin, N. D., & Sabo, M. P. (1987). Early identification of congenital sensorineural hearing impairment. The Hearing Journal, 40, 13-17.

17. Mauk, G., White, K. R., Mortensen, L., & Behrens, T. R. (1991). The effectiveness of screening programs based on high-risk characteristics in early identification of hearing impairment. Ear and Hearing, 12, 312-319.

18. Weirather, Y. P., Korth, N., White, K. R., Downs, D., Woods-Kershner, N. (1997). Cost analysis of TEOAE-based universal newborn hearing screening. Journal of Communication Disorders, 30(6), 477-493.

19. White, K. R. (1996). Universal newborn hearing screening using transient evoked otoacoustic emissions: Past, present, and future. Seminars in Hearing, 17(2), 171-183.

20. White, K. R., & Maxon, A. B. (1995). Universal screening for infant hearing impairment: Simple, beneficial, and presently justified. International Journal of Pediatric Otorhinolaryngology, 32, 201-211.

21. National Center for Hearing Assessment and Management (2000). Legislative activities. World Wide Web, https://www.infanthearing.org/legislative/index.html.

22. American Academy of Pediatrics (1995). Joint Committee on Infant Hearing 1994 Position Statement. Pediatrics 95(1), 152-156.

23. Mahoney, T.M. (1986). Large-scale high-risk neonatal hearing screening. In E.T. Swigart (Ed.), Neonatal hearing screening (pp. 123-141). San Diego, CA: College-Hill Press.

24. Prager, D.A., Stone, D.A., & Rose, D.N. (1987). Hearing loss screening in the neonatal intensive care unit: Auditory brain stem response versus crib-o-gram; a cost-effectiveness analysis. Ear and Hearing, 8(4), 213-216.

25. Markowitz, R.K. (1990). Cost-effectiveness comparisons of hearing screening in the neonatal intensive care unit. Seminars in Hearing, 11(2), 161-166.

26. Turner, R.G. (1991). Modeling the cost and performance of early identification protocols. Journal of the American Academy of Audiology, 2(4), 195-205.

27. Turner, R.G. (1992). Comparison of four hearing screening protocols. Journal of the American Academy of Audiology, 3(3), 200-207.

28. Turner, R.G. (1992). Factors that determine the cost and performance of early identification protocols. Journal of the American Academy of Audiology, 3, 233-241.

29. Kenworthy, O.T. (1987). Identification of hearing loss in infancy and early childhood. In J. Alpiner & P. McCarthy (Eds.). Rehabilitative audiology: Children and adults. Baltimore: Williams & Wilkins.

30. Maxon, A. B., White, K. R., Behrens, T. R., & Vohr, B. R. (1995) Referral rates and cost efficiency in a universal newborn hearing screening program using transient evoked otoacoustic emissions (TEOAE), Journal of the American Academy of Audiology, 6, 271-277.

31. Kemper, A. R., & Downs, S. M. (2000). A cost-effectiveness analysis of newborn hearing screening strategies. Arch Pediatr Adolesc Med, 154, 484-488.

32. Kalatzis, V., & Petit, C. (1998). The fundamental and medical impacts of recent progress in research on hereditary hearing loss. Human Molecular Genetics, 7(10), 1589-1597.

33. Green, G. E., Scott, D. A., McDonald, J. M., Woodworth, Sheffield, V. C., & Smith, R. J. H. (1999). Carrier rates in the Midwestern United States for GJB2 mutations causing inherited deafness. JAMA, 281(23), 2211-2216.

34. Cohn, E. S., Kelley, P. M., Fowler, T. W., Gorga, M. P., Lefkowitz, D. M., Kuehn, H. J., Schaefer, G. B., Gobar, L. S., Hahn, F. J., Harris, D. J., & Kimberling, W. J. (1999). Clinical studies of families with hearing loss attributable to mutations in the Connexin 26 gene (GJB2/DFNB1). Pediatrics, 103(3), 546-674.

35. Yoshinago-Itano, C., Sedey, A. L., Coulter, D. K., & Mehl, A. L. (1998). Language of early- and later-identified children with hearing loss. Pediatrics, 102(5), 1161-1171.

36. Yoshinago-Itano, C., & Apuzzo, M. L. (1998). Identification of hearing loss after age 18 months is not early enough. American Annals of the Deaf, 143(5), 380-387.

37. Moeller, M. P. (In press, 2000). Early intervention and language development in children who are deaf and hard of hearing. Pediatrics.

38. Bess, F. H., & Paradise, J. L. (1994). Universal screening for infant hearing impairment: Not simple, not risk-free, not necessarily beneficial, and not presently justified. Pediatrics, 93(2), 330-334.

39. Paradise, J. L. (1999). Universal newborn hearing screening: Should we leap before we look? Pediatrics, 103(3), 670-672.

40. U.S. Preventive Services Task Force. (1996). Screening for hearing impairment. Guide to clinical preventive services, p. 393-405. Baltimore: Williams & Wilkins.

41. Watkin, P. M., Baldwin, M., Dixon, R., & Beckman, A. (1998). Maternal anxiety and attitudes to universal neonatal hearing screening. British Journal of Audiology, 32(1), 27-37.

42. Barringer, D. G., & Mauk, G. W. (1997). Survey of parents perceptions regarding hospital-based newborn hearing screening. Audiology Today, 9, 18-19.

43. Feldman, W. (1990). How serious are the adverse effects of screening? Journal of General Internal Medicine, 5 (Supplement), S50-S53.

44. Osman, K., Pawlas, K., Schutz, A., Gazdzik, M., Sokal, J. A., & Vahter, M. (1999). Lead exposure and hearing effects in children in Katowice, Poland. Environmental Research, 80(1), 1-8.

45. Gorlin, R. J., Toriello, H. V., & Cohen, M. M. (1995). Hereditary hearing loss and its syndromes. NY: Oxford University Press.

46. Fraser, G. R. (1976). The causes of profound deafness in childhood. Baltimore: JHU Press.

The goal of this project is to determine the actual costs of screening, follow-up, and diagnosis of EHDI programs in Utah. Using the ingredients model of economic analysis, coupled with standard economic analysis techniques, such as sensitivity analysis, discounting, and distribution of costs, the following two objectives will be addressed:

Objective 1: To identify the resources and costs of screening and tracking newborns in Utah for hearing loss.

Objective 2: To identify the resources and costs of diagnosing infants in Utah for hearing loss.

Background

The first step in a cost study is to identify and estimate the amounts and itemized dollar values of all resources used in the program. The second step is to sum the dollar value of those resources to obtain total program costs. While many studies describe themselves as cost studies, most are not done using the resource or ingredients approach that is proposed here. Previous work does not incorporate the concept of opportunity cost or estimate the contributions of families to the screening process. Most studies do not base costs on real data about the resources actually used to screen and diagnose members of families and infants. Instead, most studies make assumptions about the costs from various sources. One study, that of White, 1993, took cost analysis a step closer to the ingredients method by incorporating the actual time personnel used to screen infants, using an activity diary method to estimate the quantity of resources used. However, that study did not include the costs of diagnostics or parent contributions, and was limited to the cost of screening in one hospital. The most recent study, that by Kemper, 2000, illustrates many of the limitations of existing literature on newborn hearing screening. Kemper admits using costs from reports in the literature, discussions with experts, and the hypothetical experience of a cohort of newborns in the U.S. Given the large number of newborns and growth in numbers of newborns who actually undergo newborn screening each year, there is no reason why cost analysis should continue to be based on hypothetical samples, resource, and cost information.

Method

The proposed cost study is significant because it applies the best cost methodology available in a state which already has a comprehensive EHDI program. It also builds on the expertise of economists who have extensive training and experience in conducting national and state cost studies of services for Children with Special Health Care Needs using the most respected cost method, the ingredients method.

Ingredients method/opportunity cost. An accurate, complete cost analysis involves compiling a complete description of universal newborn hearing screening services for each newborn infant and family in the study. The largest, and often most challenging, component to accurately track is the time of personnel such as nurses, audiologists, physicians, parents, and other health care professionals. Typically, the major resources used in a program can be categorized as personnel, materials, equipment, and facilities. Resources, such as parent time, while they do not entail an actual dollar expenditure, do represent costs because they could be used for other valued alternatives. The dollar value of such resources must be estimated based on "opportunity cost," the value of a resource in its best alternative use. For example, parent time has a value-if the parent is employed, that value may be estimated by the parent's wage rate. When the parent is not employed in the workforce, the opportunity cost is decreased productivity at home or in other activities and may be estimated using a prevailing wage rate, such as the median wage rate for women in their geographic region.

Thus, the cost of a newborn hearing screening program is defined as the value of all of the resources that it uses had they been assigned to their most valuable alternative use. In this sense, all costs represent the sacrifice of an opportunity that has been forgone. It is this notion of opportunity cost that lies at the base of cost analysis in the proposed study. By using resources in one way, we are giving up the ability to use them in another way, so a cost has been incurred.

The type of detailed accounting to be used in this study can be thought of as constructing an "ingredients model" (Levin, 1983), wherein all ingredients that are required for any particular program or treatment are specified and a value is placed on each of them. The dollar values of all ingredients are then summed to establish the total cost of the treatment. The ingredients approach means that to obtain a more complete picture of costs, researchers must contact the participants to obtain a complete picture of services. Thus, parents will be surveyed about the costs to them of screening and diagnostic activities, including time, wages (if applicable), mileage to appointments, and other contributed resources.

The ingredients method also requires intensive time tracking of personnel involved in screening and, when applicable, parent time. This will be done using a combined mail-telephone survey to those who are involved in screening at eight hospitals throughout the state of Utah and in diagnostics for the sample described below. Time-tracking forms listing the activities related to newborn screening will be developed, piloted, and mailed to all personnel involved in newborn hearing screening activities. A different survey will be developed and administered for personnel involved in diagnostics. Hospital personnel will be asked to complete the screen cost instruments and return them, by fax or mail, after completing their screening activities for the week that is specified on the form. Telephone calls will be made to participants to remind them to complete the time diary. Personnel will also be given the option of completing the survey by phone. The one-week activity diary method will be repeated for each hospital every quarter over the three years of cost data collection. Personnel involved in diagnostics for the 50 infants and their families involved in the cost study each year will be identified from the EHDI database and from interviews with families. The survey will be administered with these personnel and their employers to identify the resources and costs associated with diagnostics for the proposed sample.

Distribution of costs. Another important piece of the cost puzzle is the distribution of costs, whether paid by parents, medical insurance (public or private), or other private or public entities. Parents will be asked to report out-of-pocket costs and to describe their insurance coverage (e.g., self-insured, Medicaid, CHIP, employer-based insurance) and the amount of their co-pays and deductibles. This parent survey will be mailed in advance and followed with telephone calls to give families time to review their medical and insurance records. Information on the distribution of costs will be obtained through the parent survey and when collecting cost information from hospitals and providers.

Estimating uncertain costs: The use of sensitivity analysis. Sometimes it is necessary to estimate costs. However, there may be no reliable standard on which to base a cost estimate, or there may be a range of cost estimates for a particular ingredient. For example, the value of parent time may be estimated using the median wage rate for the nation, state, or locality. There are also debates over how much of hospital's fixed costs to include, such as lights, janitorial service, etc., in the cost of newborn screening. Sensitivity analysis allows more than one cost estimate, varying the costs to reflect those different estimates.

Adjustments made to account for changes in dollar value over time. In conducting a longitudinal study, it is essential to account for the effect of time on the value of money. Two adjustments may be required. First, to compare dollar figures from different years-for example, the cost of a screen in 2001 to that in 2003-all dollars must be adjusted to have the same purchasing power. This is often done using a price index such as the Gross National Product deflator to convert dollar figures to their value in a single year, thereby removing the effect of annual inflation. The second impact of time on cost is that of discounting. Discounting may be necessary because even after dollar figures from different years have been adjusted for the effects of inflation, they are still not equivalent in value. A dollar today is better than a dollar next year because of the opportunity cost of lost investment over time. The annual opportunity cost is the rate at which people willingly trade a dollar today for a dollar next year. This is called the discount rate. Discounting converts all dollars to their "present value which is, typically, their value in dollars when the project began. Such economic principles are understood by the applicant and will be applied to the longitudinal cost analysis where appropriate.

Sampling. The 43 birthing hospitals in Utah will be stratified based on size, and eight hospitals will be randomly selected for inclusion in the study. The four categories for size include annual birthrates of: a) more than 2000 live births, b) 800-1999 live births, c) 300-799 live births, and d) fewer than 300 live births per year. Two hospitals will be randomly selected from each cell. Administrators and staff at the hospital who are involved in hearing screening and diagnostics will be contacted, requirements for participation will be described, and their participation sought. If they decline to participate, another hospital will be randomly selected from that cell and substituted. This will result in a representative sample for the state, and thus generate accurate estimates of those costs for the state.

Data will be collected for a total of 360 families and infants over a three-year period. The specific families to be included in the sample during each of these three years are described below. First, because inpatient screening requires no extra time from the family, parent interviews will begin during the first year with a random sample of 35 families from the state's tracking database who failed the inpatient screen and passed the outpatient screen. In addition, 35 families who failed both inpatient and outpatient screens and passed the diagnostic assessment will be randomly selected from the tracking database and interviewed regarding their screening costs, both out of pocket and contributed, as well as other sources of medical reimbursement they may have accessed during the screening process. This family interview provides a unique opportunity to obtain information from families about their reaction to the false positive inpatient screen and outpatient screen results, an issue which has caused much controversy in discussions of EHDI programs. Thus, some questions will be incorporated into the cost survey to capture the impact of those false positive results on their perceptions of their child's health. Finally, a random sample of 50 families who fail both inpatient and outpatient screens and the diagnostic assessment (i.e., children identified with a congenital hearing loss) will be included in the cost study and surveyed related to their costs, who paid those costs, and issues related to private and public health insurance coverage.

Project Activities

Year 1: Identify the sample
Develop and/or adapt Instruments for providers, parents, and administrators
Train office assistants in data collection
Pilot instruments with providers, parents, and administrators
Begin round one data collection, mail-call routine, from providers, families, and administrators

Year 2: Continue data collection from providers, families and administrators
Identify newborn/family sample for Year 2
Begin round 2 of cost data collection with providers, parents and administrators

Year 3: Continue data collection from providers, families and administrators
Identify newborn/family sample for Year 3
Begin round 3 of cost data collection with providers, parents and administrators

Year 4: Continue data collection from providers, families and administrators
Preliminary cost data analysis

Year 5: Estimate the cost of screening, tracking, diagnosis-per child, by hospital, statewide
Analyze cost for patterns by key variables
Estimate the distribution of costs by funding source
Final analysis and interpretation of results
Final report, publications, and presentations

Capability of the Applicant

The principal investigator for the study, Dr. Goetze, has conducted numerous national, state, and local cost studies using the ingredients method proposed for this project. She also has extensive knowledge about the finance sources used to pay for health and intervention services which is relevant to the distribution study that is proposed. Dr Goetze has extensive experience designing and implementing the methods proposed for this study, including methods in estimating the costs to families associated with early intervention services. She is the author of numerous publications and reports in the areas of cost and finance related to early intervention, health, and education.

The project staff includes Dr. Catherine B. Broadhead, who has a Master's degree in Psychology and Human Resources from the Université Catholique de l'Ouest (France) and recently obtained a PhD. in Economics from Utah State University. Dr. Broadhead is currently a Research Associate at the Early Intervention Research Institute and is actively engaged in ongoing research. She specializes in cost-benefit analysis and statistical analysis employed in educational research. Her extensive experience in survey design and instrumentation is of particular value for the proposed project. She is currently working on the Longitudinal Study of Early Intervention Costs, Effects, and Benefits for Children with Disabilities and their families (LEEF) and a project which is evaluating two Early Intervention finance models in Ohio and Indiana.

The etiology of moderate to profound sensorineural hearing loss has historically been estimated through studies of children with hearing impairments attending school settings. Fortunately, now that newborn screening is becoming the standard of care for infants in the United States, we are in a much better position to determine etiology.

The goal of this project is to determine the etiological categories of hearing loss in infants identified in Utah's statewide EHDI program. This will be accomplished by providing an etiologic evaluation at no charge for all children identified by the state's EHDI database with congenital hearing loss.

Objective #1: Determine the frequency of genetic hearing loss due to mutations of a gene encoding Connexin 26.

Objective #2: Integrate as a routine part of follow-up care, genetic consultation and services for the families of infants determined to have congenital hearing loss.

Objective #3: Include information on the etiology of deafness as a part of the EHDI database which is linked with other relevant public health information systems (e.g., Birth Defects, Early Intervention).

Background

Congenital hearing loss occurs in approximately 3 infants per 1000 in Western nations. Estimates of the proportion of congenital hearing loss due to genetic causes vary from 20-75%, with most reviews indicating about 60%. The bulk of the cases of genetic origin are nonsyndromic (with syndromic causes accounting for about 10% of the total), and about 80% of the losses attributable to genetics have autosomal recessive etiology.⁴⁵ This means that about 40% of all prelingual hearing impairment is due to autosomal recessive genes.

Recent studies indicate that mutations in the Connexin 26 gene (Cx 26) are responsible for about 50% of the nonsyndromal recessive cases.^31-34 This would mean that about 20% of all prelingual sensorineural hearing loss is due to mutations of this important gene. Of note, there is evidence that one mutation (35 del G) is responsible for the majority of pathologic Cx 26 mutations.

Most previous studies of the etiology of hearing impairment have been done with older children, including unspecified numbers with late-onset losses caused by conditions like meningitis or high doses of ototoxic drugs. This suggests that the frequency of genetic causes of hearing loss, including Connexin 26, might even be higher in newborns and infants.

A recent study in Iowa³³ indicated that about 40% of individuals referred to an ENT clinic with congenital hearing loss had mutations of Connexin 26. If the familial cases were left out of the analysis, 28% of cases in this group had Connexin 26 mutations. This suggests that Connexin 26 accounts for almost 30% of all children with cryptogenic deafness, i.e., hearing loss of no known cause. This fits quite well into the proposals made almost thirty years ago by Fraser⁴⁶ that a significant proportion of this unidentified cryptogenic group would be the first case in their family with autosomal recessive hearing loss. In addition, this detection rate would warrant routine genetic evaluation of all children with congenital hearing loss of unknown etiology.

Several factors make Utah an ideal place to study the etiology of hearing loss. First, Utah has a comprehensive EHDI program. Second, there is only one major referral center for children, i.e., Primary Children's Medical Center, one University Medical Center (which is structurally attached to the Primary Children's Medical Center and the pediatric faculty are the same), and the bulk of the population of the state resides in a 70-mile strip between Ogden and Provo. Third, there is an integrated program for care of children with disabilities through the Health Department's Children with Special Health Care Needs program. This program includes traveling clinics that go to all of the major towns and cities in the state of Utah that are not within that 40-mile drive to Salt Lake City. Fourth, the Center for Human Genetics, the state's large genetic research center and one of the most advanced and complete in the country, is located at the University of Utah, which is the state's primary genetic service provider and receives funding from UDOH for a substantial portion of its genetic service programs. This group includes five board-certified medical geneticists, two cytogeneticists, and four genetic counselors. Fifth, there is an organized infrastructure for clinical genetics research through the International Clinical Genetics Research and Consultation Program (ICGRCP). This program operates through the Clinical Research Center at the University of Utah and employs a study coordinator and two full-time genetic counselors devoted to research projects. Finally, Utah has the largest genealogy library in the world, which facilitates human genetic research. Families in Utah are also willing participants in genetic research. The rate of refusal to participate is less than 5%, and the lost to follow-up rate is less than 10% for most clinical genetic research projects. Utah also has the highest birth rate in the nation, making the family size in the state larger than for any other. Under the highly secure protocols of the Resource for Genetic and Epidemiological Research (RGE), which was established by Executive Order of the Governor in 1982, Utah birth certificates are continually linked across sibships and generations. Such genealogical information enhances researchers' ability to study the genetics of many disorders affecting newborns.

The research project will be conducted by the Division of Medical Genetics at the University of Utah/Primary Children's Medical Center. This Division is well connected to the Utah Department of Health through clinical contracts for the Outreach Clinics, as well as the Metabolic Program. The Division has been working with the Utah Department of Health for 22 years in these various clinical service projects. Thus, the Division is well positioned to conduct a clinical research project on the etiology of hearing loss in Utah children.

The University of Utah DNA Diagnostic Laboratory is a comprehensive service laboratory providing many DNA tests for Utah and the Intermountain Region. The laboratory does DNA testing for various conditions such as Fragile X, myotonic dystrophy, cystic fibrosis, colon cancer, paternity testing, and many others. The director, Dr. Ward, is board certified in Clinical Genetics and in Clinical Molecular Genetics by the American Board of Medical Genetics. The laboratory is currently developing the DNA testing for Connexin 26 and is now in the process of piloting it. Offering it for clinical service will occur in the next two months.

The Division of Medical Genetics is also connected with the state's Heelstick Screening Laboratory. The Division provides the medical input to the statewide screening laboratory and orchestrates the follow-up clinic for children identified with PKU and galactosemia at the Metabolic Clinic at Primary Children's Medical Center. Thus, infrastructure for using the heelstick screening blood spots to test for Connexin 26 is easy to establish.

Design

Our aim is to determine the etiological categories of hearing loss in infants by using genetic diagnostic and counseling consultation for all families of infants with congenital hearing loss identified through Utah's EHDI program. The etiologic and diagnostic evaluation will occur either at the clinics in the Clinical Research Center at the University of Utah or during the Outreach Clinics described above. The families will be seen by a board-certified clinical geneticist (who are all pediatricians) and by a genetic counselor. The usual clinical data including perinatal history, detailed 3-generation pedigree, and physical examination to identify a syndrome will be the cornerstone of the diagnostic evaluation. This strategy will identify a small percentage of children with syndromes (certainly less than the 10% of those identified as syndromes by adolescents). Family trees will most likely identify that 25-30% of families have a close relative with a similar hearing loss identifying a specific genetic transmission (autosomal dominant, autosomal recessive, x-linked, or mitochondrial). In addition, routine urinalysis (to test for cytomegalovirus among other things) will be recommended to be done by the primary care physicians. In this way, some children will be identified to have a well-established environmental or (by pedigree analysis) genetic cause. All families will be offered a Connexin 26 analysis at no charge. This will be accomplished easily by extracting DNA from a drop of blood similar to what was collected earlier for the Heelstick Screening. ⁽³⁾ Prior to participating in the Connexin 26 analysis, families will provide written informed consent. Results will be conveyed to the families with a followup genetic consultation offered.

After results of the initial consultation and the laboratory testing, a session with a genetic counselor will be offered. This, along with the initial diagnostic evaluation and the Connexin 26 analysis, will be of no charge to the family. Other routine diagnostic studies which are clinically indicated in the diagnosis and management of an infant with congenital hearing loss will be part of routine care and billed appropriately (e.g., cytomegalovirus, ophthalmologic examinations, etc.).

Based on the literature, we estimate that about 30% of families will have a straight-forward mendarian pattern based on pedigree, about 5% will have a syndromic cause, about 10% will be a well-established environmental cause, and that over 50% will have unidentified causes (cryptogenic). As mentioned above from the Iowa study, almost 30% of this latter group may be due to Connexin 26 mutations. In addition, about 50% of the families identified as being autosomal recessive by pedigree will have Connexin 26 mutations.

We expect that this approach will also lead to the identification of families with autosomal dominant, x-linked, or perhaps maternal inheritance (mitochrondrial). These families will be studied for linkage and positional cloning by investigators at the University of Utah. Moreover, these data will lay a foundation for integrating genetic services into EHDI programs in general.

Summary

Utah is in a unique position to participate in a study of the causes of congenital hearing loss. First, Utah already has a well-established Newborn Screening Program. Second, the Division of Medical Genetics is integrated into children's services at the Utah Department of Health, the Traveling Clinics, and the Metabolic Newborn Screening Program. Faculty members of the Division of Medical Genetics have been active in clinical genetics research and have established an infrastructure (ICGCP) and have also been involved in many positional cloning projects that have been successful at the Eccles Institute of Human Genetics. Third, Utah is a unique state in and of itself since specialty services are limited and occur in very few medical centers. The Utah Birth Defects Network based at the Utah Department of Health is a component of the Genetics Division and represents a close connection to the Medical Genetics Division and the Utah Department of Health. Finally, there is the presence of the GRE and the cadre of world-class investigators at the Eccles Institute of Human Genetics.

1. A "medical home" is an approach to providing health care services, which provides a place where child and family can receive the medical care they need from someone they trust.

2. It is important to note that integration can occur at various levels. For example, one form of integration would be for Vital Statistics to generate a report of home births at the end of the year and send it to the EHDI database to determine how many of those home births were screened. That type of "after the fact" integration is not what this project will do. Instead, our goal is to achieve integration which is timely enough to improve services to children and families. For example, Vital Statistics should electronically transmit reports of home births to EHDI as they arrive, so EHDI can check and immediately notify the parents of the availability of screening.

3. Note: An obvious question is why we don't use the original blood spot from the Heelstick Screening. First, Utah, like many other states, only stores these specimens for 3 months. Because a significant number of these children may not be diagnosed in time to come to the clinic before 3 months of age, the original blood spot may not be available. Second, it is always better to have fresh blood instead of stored blood when the option is available.