We need to increase awareness about the importance of testing for congenital cytomegalovirus so this leading cause of non-genetic deafness in newborns can be diagnosed and treated.

By Frances C. Esteve

Congenital cytomegalovirus (cCMV) is the leading cause of non-genetic deafness in newborns, and even though it is more prevalent than many other congenital infections, testing for congenital CMV has not been widely implemented across the United States.   

Growing Awareness

While there has been a significant growth in awareness and education as well as testing adoption in recent years, such advancement highlights the need for additional progress. In just over 10 years we have seen states with legislation enacted go from one to 23 with an additional seven states with proposed legislation. By the same token, it has taken over 10 years to get less than half of the states to come on board. Even with the progress that has been made through the work and support from groups like the National CMV Foundation (NCMVF), the Early Hearing Detection and Intervention (EHDI) Program, and The American Academy of Otolaryngology, to name a few, the focus remains on the advancement of congenital CMV testing adoption as the primary step toward management of congenital CMV infection. 

Images Source: CMV Legislation | National CMV Foundation | National CMV Foundation

Building Momentum

The state of Colorado does not have legislation in place requiring testing for congenital CMV, yet the Colorado Chapter of the American Academy of Pediatrics (AAP-CO) has been leading a Work Group on cCMV infection since 2018. A major focus for the group has been improving the diagnosis of cCMV and improving support for families raising children with cCMV. 

In their view, when cCMV cases are missed, those children get sub-optimal medical management and suffer worse health outcomes. Twenty Colorado hospitals are developing policies to institute targeted screening for congenital CMV. AAP-CO has received grants from AAP and EHDI to support the establishment of the Colorado cCMV Family Network. AAP-CO has demonstrated progress toward cCMV diagnosis, but at the same time, it has shown the need for additional work to fully implement a complete support system1. 

In 2019, the CDC, in collaboration with state, local, and territorial health departments, implemented a population-based mother-infant-linked longitudinal surveillance, the Surveillance for Emerging Threats to Mothers and Babies Network (SET-NET), which collects data on infants’ disease threats, including congenital syphilis, hepatitis C, and COVID-19 (National Center on Birth Defects and Developmental Disabilities, 2022). During 2022-2023, the CDC funded five Jurisdictions (Iowa, Minnesota, New Jersey, New York, and Utah) to pilot a congenital CMV surveillance through SET-NET. Of the 21 locations working mainly with the Department of Health (DOH), four focus on cCMV surveillance (Utah, Iowa, Minnesota, and New Jersey)2. 

SET-NET also aims to track birth defects, developmental problems, and other disabilities as these children age, for up to three years. CDC research on cCMV includes3:

  • Evaluating the various laboratory tests for newborn screening for congenital CMV
  • Determining the most effective screening approaches to identify babies with congenital CMV
  • Characterizing the impact of the disease in various populations, and
  • Assessing the long-term outcomes of children with congenital CMV.

The first step toward universal screening happened in 2022 when the state of Minnesota became the first state to enact legislation requiring universal screening. It was then followed by Connecticut, which is scheduled to go live in 2025. Earlier this year the American Academy of Otolaryngology-Head and Neck Surgery endorsed the need for universal screening. 

When asked what advancement in the last several years has had the largest impact in increasing awareness and education and testing adoption related to congenital CMV, Cedric Pritchett, MD, a pediatric otolaryngologist at Nemours Children’s Hospital, observed: “While partnerships with the State Departments of Health and State Early Hearing Detection and Intervention Agencies have had a measurable impact on the visibility and awareness of congenital CMV infection, State legislative directives have had the largest scope and scale of impact. The positions of professional organizations and societies have lent essential credibility to these legislative and regulatory entities.” 

Parents Key to Congenital CMV Screening

Parents’ and/or mothers’-to-be knowledge of congenital CMV has made progress from a reported 7% in 20164 to almost 15% in 20235. We take the advancement in awareness and education, but in the grand scheme of things, there is still room for improvement. Progress is needed in educating mothers-to-be as they can implement simple tips to prevent infection, given that almost 90% of parents surveyed had engaged in behavior that increased the risk of cCMV infection during pregnancy5.  A qualitative assessment shows that clinical practice guidelines (CPG) and healthcare practice patterns related to congenital CMV vary. It was noted that current trends in legislation supporting newborn screening for cCMV has grown the awareness among patients and healthcare providers. In turn, increased awareness encourages parent inquiries and drives more providers to perform cCMV screening despite absent or limited CPG. Participants identified that clinical practice is predominantly informed by disease-specific guidelines. CPGs that comprehensively address guidelines management will be key in improving care for expectant mothers and newborns6.

As part of a study evaluating parental perspective on healthcare providers following a newborn diagnosis of  congenital CMV infection, the parents valued access to the information, follow-up visits for early detection of hearing loss and other developmental delays, and support from other parents. Parents wished to obtain this information from their pediatrician, but experts provided more up-to-date knowledge about prognosis, monitoring, and treatment information. With more U.S. states implementing cCMV screening strategies, which should lead to more infants being diagnosed, progress is needed for providers to meet parents’ expectations and communication needs7. Continued growth in awareness from parents as they work with advocate groups, and from healthcare providers as new legislation becomes available, are needed and necessary to improve the care of children with cCMV infection. 

Congenital CMV Testing Implementation Shows Increase in Detection

Data review of 445 pediatric patients from the University of Minnesota Lions Clinic identified 44 children with cCMV, of which 33 (75%) had sensorineural hearing loss (SNHL). Seventeen (39%) passed their newborn hearing screen (NHS) and would have been missed if not for universal screening. Of these, six (35%) demonstrated delayed-onset SNHL, which was detected at a mean age of 20 months. Eleven (25%) remained normal at 19 months. Babies under this program benefited from early detection and intervention. Critical steps to success are the interface with state EHDI programs and improved intervention strategies with CMV-infected newborns8. 

A retrospective review of targeted screening implementation revealed that the rate of cCMV testing in children who did not pass their NHS increased from 14% to 88% after full implementation of targeted screening, together with an increase in earlier referral to infectious disease. A main factor that led to the successful implementation of this program was establishing a working group with representation from the divisions of ID, audiology, otolaryngology, and neonatology. The working group facilitated items like a specific workflow to determine who would order testing, receive results, and how the rationale for testing would be explained to families. Initial discussions with nursing prior to implementation and intermittent contact were critical to the success of the protocol. The approach used can be similarly applied and relevant to a universal screening program9. 

In 2018 under a Quality Improvement (QI) initiative for early identification of cCMV, the aim was to increase congenital CMV screening by 25% from a base rate of 22%. The initiative looked to study and screen symptomatic newborns with suggestive findings of cCMV, who failed the newborn hearing screen, who had not had a hearing screen performed by 10 days of age, or who were infants of HIV-positive mothers. 

This effort included a multidisciplinary approach; 903 infants were eligible for screening with small gestational age (46%) being the most common indication for screening, followed by failed NHS (32%): 

  • 67% of eligible infants were in NICU and 33% were in the newborn nursery
  • 444 of the infants received cCMV screening, resulting in an increase from a baseline of 22% in 2016 to 74% during the one-year study period. 
  • 4 Infants were congenital CMV positive and received appropriate treatment because of these interventions. 

The study showed that it is feasible to improve cCMV screening by implementing a standardized extended screening protocol using QI methodologies10. 

In recent years, testing for congenital CMV has become simpler, easier, and more accessible. The availability of FDA-cleared tests for congenital CMV has paved the way to bringing molecular testing closer to the patient. They allow institutions to run the test on-site instead of sending out, which usually takes 2-4 days to get results. 

Being able to run the test on-site not only can be more cost effective, but it can provide same-day results that can avoid delays in discharge. Regardless of the testing approach, a key factor remains: There is no way to tell with 100% certainty that transmission occurred unless the newborn is tested within 21 days of birth. 

With a rapid molecular test like Alethia CMV from Meridian Bioscience, which utilizes a simple-to-collect saliva sample11, physicians and parents can have actionable results before the baby goes home. Equally important is what needs to happen after a positive result. Under ideal circumstances, the result is obtained from the laboratory before the baby is discharged from the hospital, providing the opportunity, if necessary, for prompt treatment, additional testing, and/or referral to other areas. 

These studies all have several key points in common, the most prevalent being the implementation of testing for cCMV which helped increase the identification of congenital CMV-infected babies that otherwise would have been missed and not given the opportunity to be treated appropriately in a timely manner. It also highlighted the importance of collaborating with other departments/disciplines within the institution and/or the state programs to maximize the positive outcome of a child and provide support for the parents.

Congenital CMV Screening: What is Lacking and/or Missing?

A report on congenital CMV surveillance in the US indicated that the number of cases reported by health departments (HD) ranged from 3 to 47 cases per year in seven states, which was much lower than the expected number of cCMV cases. 

Two major challenges identified that impact surveillance and follow-up activities are personnel constraints and limited fundings. Additional reported challenges included a lack of a standardized case definition, complex and siloed data systems, incomplete reporting, and delays in receiving case data. According to the report, only 10 of 50 states systematically collect cCMV case data2.

A study using a large U.S. EHR database found that only one in 10 infants with lab-confirmed diagnosis of cCMV received a diagnosis code for congenital CMV infection. Currently, cCMV surveillance relies primarily on passive reporting from hospitals, providers, and labs. Moving forward, identifying ways to ensure integration of newborn screening with surveillance and follow-up could optimize use of public health resources. A standardized public health definition for cCMV is important for consistency in measuring disease prevalence across jurisdictions and over time2. 

Despite the serious consequences that can result from a cCMV infection, studies have reported low knowledge and frequency of congenital CMV counseling among obstetric specialists and pediatricians. Also mixed knowledge and low comfort with caring for children with cCMV. Other studies have reported

that knowledge about cCMV is low amongst expectant parents, and parents of children who have congenital CMV emphasized that they would have benefited from clear guidance and support post-diagnosis, and from information about cCMV prevention during pregnancy7. 

In 2019, the National CMV Foundation submitted a proposal to the Recommended Uniform Screening Panel (RUSP) for inclusion of cCMV as an entity for universal newborn testing. In Pritchett’s opinion, while ultimately this proposal was not adopted, the response from the Advisory Committee on Heritable Disorders in Newborns and Children was informative, and it is important to individually pursue addressing the five points articulated in the Committee’s response with the intent to move forward the adoption of congenital CMV screening as part of RUSP.

Having legislation and/or guidelines or being part of RUSP only helps bring awareness and helps drive testing compliance. However, for the implementation of routine testing to be successful, it must also be accompanied by a multidisciplinary approach that includes education and training in all areas involved. Something as simple as sample collection is as important as the test itself, which means that all areas play a vital role in the success of testing for congenital CMV. 

The Future of Congenital CMV Screening – Federal Legislation

Stop CMV Act of 2024 is a proposed Bill under the Federal government that, if passed, will apply to all states equally. So far, the states that have enacted legislation related to congenital CMV either requiring testing or testing and education or education only are limited to their corresponding state.  

The Stop CMV Act of 2024 has been introduced in the U.S. Senate by Senators Richard Blumenthal (D-CT), Chris Murphy (D-CT), and Roger Marshall (R-KS) and in the U.S. House by Representatives Mike Lawler (R-NY) and Greg Landsman (D-OH). Specifically, the bill authorizes funding to states for hospitals and other health care entities caring for infants to administer to every infant a congenital CMV test and encourages state healthcare agencies to prescribe standards and procedures for the administration of these tests. The bill also creates grant programs to provide funds to administer cCMV tests, improve cCMV data collection systems, and assist in cCMV education and training. Lastly, the bill advances National Institutes of Health research for screening techniques, diagnostics, prevention, vaccines, and treatments.

The Senate version of the bill (S.3864) is available here. The House version of the bill (H.R.7542) is available here. 

The National CMV Foundation has been an integral part in the progress that we have witnessed when it comes to congenital CMV, not only bringing awareness and education, but also working to bring congenital CMV recognition to the state level and beyond.

In a statement, Khaliah Fleming, executive director at the National CMV Foundation, notes, “We are truly delighted to witness the significant progress in cCMV screening and testing efforts. The tireless efforts and unwavering support from families and CMV advocates have been instrumental in this journey. Achieving this milestone has been a result of years of hard work, dedication, and advocacy, and it’s incredibly gratifying to see the fruits of our labor. While there’s still much work ahead of us, it’s important to pause and celebrate these victories. The progress made is a testament to the power of collaboration and persistence. As we continue to expand our federal advocacy efforts, The National CMV Foundation fully supports the Stop CMV Act as this legislation represents the first coordinated federal effort to improve and invest in cCMV research, testing, education, and data collection. This will undoubtedly increase cCMV awareness, support high-impact cCMV research, and play a pivotal role in reducing the impact associated with cCMV in newborns. We believe that supporting this act is crucial in advancing our mission to prevent pregnancy loss, childhood death, and disability due to cCMV.”12

From a physician perspective, Cedric Pritchett, MD, states, “I am supportive of the Stop CMV Act, and very much impressed with its approach and language. It is inclusive and comprehensive (aiming to maximize children tested while addressing data tracking, follow-up reviews, and educational needs). It preserves state individuality while demanding state accountability and action. It acknowledges the need for Federal support in funding testing (HRSA), developing appropriate data collection systems and educational resources (CDC), and the advancement of technology, screening diagnostic, vaccination, prevention, and treatment options for CMV infection (NIH).”

Overall, the advancements that have taken place, especially in the last several years, are significant and will have a lasting impact, but additional progress is still needed to close the gap and bring congenital CMV awareness and education, as well as testing and patient care to an even higher level. Ideally, the Stop CMV Act of 2024 will pass and go into effect soon. But in the meantime, there is work to be done.The Foundation has worked with other advocates for several months to develop this important bipartisan legislation. Now it’s our turn to lend a hand! Please contact your Members of Congress using the template language below:

House Letter Template
Senate Letter Template

About the Author

With more than 40 years of experience in IVD at Meridian Bioscience, Frances C. Esteve is currently a marketing product manager. During her tenure with the company, she has held various positions in the areas of product research and development, manufacturing, international sales, and marketing. In addition, she has served as an ASQC Certified Quality Auditor.

This article was originally published by The Hearing Review’s sister brand, CLP, with the headline “Better Congenital CMV Screening Needs More Awareness, Legislative Action.”

Photo: Dreamstime

References:

  1. American Academy of Pediatrics Colorado Chapter https://aapcolorado.org/wp-content/uploads/2021/03/Information-about-congenital-CMV-for-pediatric-hospitalists-and-other-clinicians-who-see-newborns.pdf
  2. Raines, K. et al. Congenital CMV surveillance in the US. Birth Defects Research, Sept 2023; 115:11-20.
  3. What is SET-NET? CDC What is SET-NET? | CDC  
  4. Doutre, S. et al. Losing Ground: Awareness of Congenital Cytomegalovirus in the United States. The Journal of Early Hearing Detection and Intervention, 2016; 1(2): 39-48.
  5. Aldè, M. et al. Hearing outcomes in children with congenital CMV infection: From management controversies to lack of parents’ knowledge, International Journal of Pediatric Otorhinolaryngology, Jan 2023; Vol 164, 111420.
  6. Kalb, S. et al. A Qualitative Assessment of Clinical Practice Guidelines and Patterns of Congenital CMV in the US. Int J Neonatal Screen, 2023, 9, 37.
  7. Lanzieri, T. et al. Parental Perspective on Communication from Health Care Providers Following a Newborn Diagnosis of cCMV infection: A Secondary Analysis of a Qualitative Study. Intl J of Neonatal Screening, 2023, 9, 49.
  8. Rohren, L. et al. Congenital CMV-Associated Sensorineural Hearing Loss in Children: Identification Following Universal Newborn Hearing Screening, Effect of Antiviral Treatment, and Long-Term Hearing Outcomes. Ear & Hearing, 2024; 45:198-206.
  9. Ronner, E. et al.  Congenital CMV Targeted Screening Implementation and Outcomes: A Retrospective Chart Review. Original Research-Pediatric Otolaryngology-Head and Neck Surgery 2022, vol 167(1) 178-182.
  10. Krishna, S. et al. Early Extended Neonatal Screening for Congenital CMV Infection: A Quality Improvement Initiative. The Joint Commission Journal on Quality and Patient Safety, Sept. 2020, Vol 46, No. 9, p 516.
  11. Meridian Bioscience website https://www.meridianbioscience.com/diagnostics/disease-areas/pediatric-neonatal/cmv/?country=US
  12. National CMV Foundation website CMV Legislation | National CMV Foundation | National CMV Foundation