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Sunday, July 5, 2015

Congenital Cytomegalovirus (cCMV) and Connecticut’s New Law


Congenital Cytomegalovirus (cCMV) and Connecticut’s New Law

by
Lisa Saunders
 Parent Representative, Congenital Cytomegalovirus Foundation*


In May 2015, Connecticut became the second state in the U.S., after Utah, to pass a law on the leading viral cause of birth defects, which causes more disabilities than Down syndrome. Governor Dan Malloy signed House Bill 5525-An Act Concerning Cytomegalovirus:
This act states: "any newborn infant who fails a newborn hearing screening, as described in section 19a-59, a screening test for cytomegalovirus, provided such screening test shall be administered within available appropriations on and after January 1, 2016. Such screening tests shall be administered as soon after birth as is medically appropriate."

"With bipartisan support, the legislature approved and the governor signed a bill that will help mitigate the devastating impact that this disease has on families. The bill requires that hospitals and other health care institutions test newborn infants for CMV if they fail a newborn hearing test. This will help parents intervene early and get their newborn child the help it needs," states the State Senate Democrats of the Connecticut General Assembly in a press release.

Ever since Lisa Saunders, the parent representative of the Congenital Cytomegalovirus (CMV) Foundation and mother of Elizabeth born severely disabled by this preventable virus, learned Utah passed a CMV bill  in 2013, she has been asking Connecticut to do the same. A Connecticut CMV bill was passed by the House in 2014, but the Senate failed to vote on it before the end of the legislative session. So, Lisa and others parents whose children were disabled by congenital CMV, plus those in the medical community who supported the bill (see their Public Hearing Testimonies), had to start their campaign all over again in January 2015. House Bill 5525: "An Act Concerning Cytomegalovirus" finally became law when passed by the Public Health Committee, House (watch Lisa’s representative urge support and remember her daughter), Senate, and signed by Governor Dan Malloy on May 26, 2015.

When Lisa learned the governor had signed the bill, she was
ecstatic. She hopes that testing will not only save Connecticut’s residents heartache by quickly diagnosing and providing early intervention for children born with cCMV, but that the money saved by the state will lead to more funds being invested in prevention education until an effective vaccine is developed.

Q. What is Congenital Cytomegalovirus (cCMV)?
A. It is the #1 viral cause of birth defects. It causes mental retardation, liver disease, cerebral palsy and deafness as a result of infection in pregnant women‎. Congenital CMVis the most common cause of nonhereditary sensorineural hearing loss in childhood.” According to the Centers for Disease Control and Prevention (CDC), in the U.S.:


  • Every hour, cCMV causes one child to become disabled.
  • Approximately 1 in 150 children is born with cCMV infection (30,000 each year).
  • About 1 in 750 children is born with or develops permanent problems due to congenital CMV infection.
  • More than 5,000 children in the U.S. each year suffer permanent problems caused by cCMV.
  • Between 50% and 80% of adults in the U.S. are infected with CMV by 40 years of age.
                            
Q. What is the benefit of this law?
A. “CMV is known to cause hearing loss and therefore to test for it is reasonable. Testing infants who fail their newborn hearing screen will not only increase awareness and hopefully prevention of CMV transmission, but we will be able to identify early those infants with congenital CMV and therefore have the opportunity to intervene earlier to optimize their outcomes,” states Dr. Brenda Kinsella Balch, a Connecticut pediatrician and Chapter Champion for the American Academy of Pediatrics Early Hearing Detection and Intervention Program.

“The law encourages the proper timing of the test, so that diagnosis is not delayed beyond the critical time period of diagnostic accuracy for congenital CMV--the first three weeks of life,” states Dr. Demmler-Harrison of Texas, Director, Congenital CMV Disease Research, Clinic & Registry.

"This is a game-changing step forward in our ability to determine CMV causality and give parents a viable option for early treatment," said Scott R. Schoem, MD, FAAP, Director of Otolaryngology, Connecticut Children’s Medical Center.

Q. What does this law mean to health care providers?
A. According to Clinical Advisor’s article, Connecticut passes cytomegalovirus screening law for newborns, “Timing is key when identifying CMV infection. Providers should test via viral urine culture or urine PCR within the first three weeks of life. If a newborn tests positive, the next step is to refer the infant immediately to a pediatric disease specialist…”

According to Dr. Demmler-Harrison, Director, Congenital CMV Disease Research, Clinic & Registry, “This law is not dictating what doctors should do, but rather provides them the tools to practice good medicine in all arenas, including newborn care of babies with congenital hearing loss. Often, CMV is the last thing doctors think of, and it should be one of the first things they think of.”  

Q. How many people know about congenital CMV?

Q. Why haven’t most people heard of this leading cause of birth defects before?
A. Doctors don’t realize how prevalent it is. Fewer than half (44%) of OB/GYNs surveyed reported counseling their patients about preventing CMV infection. The virtual absence of a prevention message has been due, in part, to the low profile of congenital CMV. Infection is usually asymptomatic in both mother and infant, and when symptoms do occur, they are non-specific, so most CMV infections go undiagnosed,” according to “Washing our hands of the congenital cytomegalovirus disease epidemic.”

Q. Is this the “kitty litter” disease?
A. No, that is toxoplasmosis, which causes fewer birth defects than cCMV. Like toxoplasmosis, cCMV is preventable, but most women of childbearing age have never heard of the precautions to take.

Q. What about a vaccine against CMV?
A. Researchers have been working on a vaccine against HCMV (human cytomegalovirus) for years. One reason for delay in successful development is “there has been insufficient education about the problem of HCMV infection…” Pharmaceutical companies need to know that a vaccine will be used because people know about the disease.

Q. Overall numbers of newborns affected by cCMV in Connecticut?
A. In 2013, the number of live births in Connecticut was 36,085. Assuming that is the approximate number born in Connecticut each year, and using the CDC's statistics that 1 in 750 babies are born disabled by cCMV, then it can be estimated that there are 47 children born disabled by cCMV in Connecticut each year [ 36,085 births X .0013 (1/750) cCMV disabled = 47 babies born with cCMV in Connecticut].

Q. How many women are infected with cytomegalovirus while pregnant?
A. “The annual rate of a pregnant woman who is CMV antibody negative catching CMV for the first time in pregnancy has been estimated to be between 1-4% of all pregnant women. Higher risk groups may have higher rates of infection per year: day care workers estimated 10%, and women who have a toddler shedding CMV in the home, who do not practice precautions, up to 50%,”states Dr. Demmler-Harrison of Texas, Director, Congenital CMV Disease Research, Clinic and Registry.

Q. Which women are most at risk for contracting CMV?
A. “75% of women with a primary infection during pregnancy acquire CMV from their own child under two years of age,” said Stuart Adler, M.D


Q. What is the estimated cost of caring for children disabled by cCMV in Connecticut?
A. “In the early 1990s, the expense to the US health care system associated with congenital HCMV [Human Cytomegalovirus] infection was estimated at approximately $1.9 billion annually, with a average cost per child of over $300,000." Although $300,000 per child annually is an old cost figure, Connecticut’s annual cost of caring for children disabled by cCMV can still be estimated at:  36,085 births X .0013 cCMV disabled = 47 babies (born disabled by cCMV in Connecticut each year) X $300,000/year = $14,100,000, or, over $14 million annually.

“This figure is almost certainly an underestimate, especially now with enhanced mobility aids, surgical interventions, cochlear implants, antiviral therapies, occupational and physical therapies, etc., which were not likely included in the older cost estimates,” says Dr. Demmler-Harrison of Texas, Director, Congenital CMV Disease Registry and Research Program. For example, one of her patients born in 2001 needed a hemispherectomy [half of her brain removed] to control intractable seizures from polymicrgyria cortical malformation of one side of her brain caused by congenital CMV.

The little girl’s mother, Sandra Salerno of Texas, who resigned from her position as a financial analyst to be the primary caretaker of  Lillian, said of her daughter’s medical costs, “I have tried to sum up her medical costs for the first three years of life and they exceed $1.4 million. The surgery and five-week subsequent hospital stay was probably half of the total costs. She attends ten weekly therapies that last an hour each. At one point, she was taking seven medications. I believe she's been sedated 20 times in her life. Multiple MRI's, heart echos, PET scan, brain surgery, ear tube placement and several Auditory Brainstem Response studies.  Something that is probably underappreciated is also the cost of education for people with significant disabilities due to congenital cytomegalovirus. Lillian was offered (but we turned down) placement at the age of three for a full day school program with our district. The class ratio is 3:1 and she also qualified under multiple impairments for additional services. I have no idea how much that costs the state, and my daughter is probably higher functioning than others with cCMV, so I can't imagine the costs of children more disabled.”

Q. What are the costs of screening?
A. “The cytomegalovirus test costs approximately $150.00. As only a few infants annually fail the newborn hearing screening at UCHC [University of Connecticut Health Center], any cost incurred at UCHC is expected to be minimal,” states the House Bill 5525 webpage.

Q. How does the cost of screening compare to the costs saved through early detection?
A.
1. “Usually, if the CMV test is positive, no further testing, such genetic testing (which can cost thousands of dollars and may require pre-approvals), is done, unless there are unusual circumstances. Certainly, on rare occasion, a poor baby can be born with two conditions, but that is not at all common. Therefore, early diagnosis of a clear condition with a clear diagnostic test saves the doctor and the family a costly and heart breaking/stressful diagnostic odyssey,” states Dr. Demmler-Harrison, Director, Congenital CMV Disease Registry and Research Program. Fox News reported on the terrible consequences of a delayed cCMV diagnosis for the child Lillian Salerno mentioned above.

2. In addition to saving on the cost of more expensive diagnostic tests by first testing for CMV when a baby fails their hearing screen, an earlier diagnosis of cCMV means earlier intervention, which can mean a less disabled child. The less disabled a child is, the less they cost the state to care for.

“The Connecticut legislation extends important education and protection to families and infants from the devastating effects of CMV. The impact on lives and the reduction of state dollars needed to serve those impaired by CMV is immeasurable,” said Ronda Rudd Menlove, Ph.D., the representative responsible for passing and enacting the bill in Utah. She is the co-founder of the Utah CMV Council, along with her daughter Sara Menlove Doutre, a special education and early intervention policy consultant whose daughter is affected by congenital CMV. 

Q. What do we do if a baby tests positive for cCMV?

A. Until Connecticut develops their own protocol, Utah posts informational flyers on cCMV to health care providers on their webpage at: http://www.health.utah.gov/cshcn/CHSS/CMV.html
There, you will find:

PRINTABLE BROCHURES:
For Health Care and Newborn Hearing Screening Providers:


 ***FREE ONLINE WEBINAR!***
"cCMV 101: Congenital Cytomegalovirus from Prevention to Treatment"
Presented by: Dr. Michael Cannon of the Centers for Disease Control and Prevention (CDC)

Q. Can cCMV be treated if diagnosed?

A. The treating physician may recommend treating the child with an antiviral. “CMV infection in newborns can be treated with ganciclovir by IV or valganciclovir by oral route -- treatment reduces hearing loss progression and improves growth and head size/brain growth and improves developmental milestones,” says Dr. Demmler-Harrison, Director, Congenital CMV Disease Research, Clinic and Registry.

According to the article, “Valganciclovir for Symptomatic Congenital Cytomegalovirus Disease,” by Kimberlin, M.D., et al. (2015), which studied very affected babies, “Treated infants had fewer developmental delays…than untreated infants.”

Q. How much does this cCMV antiviral cost?
A. Cost is estimated at $1,000 TO $1500 per 100 ML bottle.  One bottle is needed each month for a 6-month treatment. [This figure needs confirmation.]

Q. Will all babies destined to lose their hearing from cCMV show a hearing loss in the newborn screen?
A. No. Hearing loss from cCMV is known to be progressive, and therefore, not all children who will have disabilities from cCMV will be detected early. That is why many in the medical community are proponents of screening every newborn for CMV. But this law will at least help 25% of the children who will experience a hearing loss from cCMV receive early intervention.

Q. How can CMV be avoided?
A. See CDC: CMV Prevention tips at: http://www.cdc.gov/cmv/index.html. Prevention tips include:
·         Wash hands often with soap and water for 15-20 seconds, especially after wiping runny noses, changing diapers, picking up toys, etc. If soap and water are not available, use alcohol-based hand gel.
·         Use soap and water or a disinfectant to clean hard surfaces that have been contaminated by secretions.
·         Don’t kiss young children on the lips or share food, drinks, or eating utensils with them.
·         If you work in a day care center, limit close contact with children younger than 2½ years of age, especially if you've never been infected with CMV or don't know if you've been infected.

Q. Will Connecticut save money if CMV prevention is taught?
A. Yes, because teaching prevention reduces the rate of transmission of CMV, according to studies in the U.S. and France. “Studies have shown that women who know they are CMV seronegative, know they are pregnant, and know about their toddler's CMV shedding are the most likely to prevent CMV transmission and reduce their risk from over 50 percent during pregnancy to a risk of less than 5 percent during pregnancy.  It is not likely that isolated instances of exposure to saliva or drool will result in transmission. Most studies suggest prolonged repeated exposures over time are important for CMV transmission,” says Dr. Demmler-Harrison.

Q. In addition to testing newborns for CMV if they fail their hearing screen, Utah’s H.B 81 requires CMV prevention brochures for doctors, parents, and daycare providers. Why doesn’t the Connecticut law require prevention education?
A.  There is a cost to prevention education.  In 2014, Connecticut estimated the cost to the state at $40,000 the first year, then $26,000 the following years.

Q. Shouldn’t we try to educate the public that pregnant women shouldn’t kiss their toddlers around the mouth or share food with them? Shouldn’t that be common knowledge like pregnant women shouldn’t change the kitty litter to prevent the birth defects caused by toxoplasmosis?
A. “Yes. In addition to saving money, you can prevent indescribable human suffering by providing prevention education,” said Lisa Saunders, parent representative, Congenital Cytomegalovirus (CMV) Foundation. “In fact, the United States Senate passed legislation designating the month of June as National Congenital CMV Awareness Month, while recommending that ‘more effort be taken to counsel women of childbearing age of the effect this virus can have on their children.’” Saunders’ daughter, Elizabeth, was born severely disabled by cCMV. She didn’t know she was putting her pregnancy at risk when operating a licensed daycare center in her home. Saunders hopes Connecticut will now add cCMV prevention information to their daycare licensing literature and post cCMV flyers at daycare centers.

Q. The cost for prevention education seems minimal compared to cost of caring for a child disabled by cCMV ($40,000 annually for prevention education versus $300,000 annually to care for EACH child disabled by cCMV). Without using state dollars, how can we start our own prevention campaign?

A. "You can start by providing cCMV prevention education on your website. Perhaps you know an organization that would provide grant money for printing brochures and posters, and/or you can contact colleges who have an internship programs for medical, public health or marketing students. Since daycare workers are at higher risk for contracting CMV and passing it onto their unborn, daycare centers can easily post flyers on how to minimize one’s risk. You can easily post downloadable cCMV prevention brochures such as the CDC's flyer: ‘What Women Should Know About Cytomegalovirus (CMV) on your website, which can be found at: http://congenitalcmv.org/CDCbrochure.pdf,” states Lisa Saunders, parent representative, Congenital Cytomegalovirus (CMV) Foundation.

If you would like to replicate Utah’s prevention brochures for doctors, parents, and daycare providers by inserting your organization’s contact information and logo, contact Stephanie Browning McVicar, Au.D., CCC-A, Utah Department of Health, Director, Early Hearing Detection and Intervention / Cytomegalovirus Public Health Initiative. McVicar said, “I am so excited that another state is bringing attention to congenital cytomegalovirus.  Utah is looking forward to partnering with Connecticut in their upcoming endeavors."

For more information on how Utah is handling their CMV law, contact the Utah Department of Health Early Hearing Detection and Intervention program at (801) 584-8215 or smcvicar@utah.gov. To see Utah’s cCMV brochures and information, which also includes the free webinar, "cCMV 101: Congenital Cytomegalovirus from Prevention to Treatment," by Dr. Michael Cannon of the Centers for Disease Control and Prevention (CDC), visit their website at: http://www.health.utah.gov/cshcn/CHSS/CMV.html

To learn more about congenital cytomegalovirus and how to prevent it, visit the Centers for Disease Control and Prevention at: http://www.cdc.gov/cmv/index.html

###


* Lisa Saunders of Mystic, Connecticut, is author of memoir, "Anything But a Dog! The perfect pet for a girl with congenital CMV (cytomegalovirus)," and the parent representative of the Congenital Cytomegalovirus Foundation, which raises awareness about maternal testing for first infection during pregnancy, newborn testing and the need to develop a vaccine (Lenore Pereira, Ph.D., Professor, Cell and Tissue Biology Department, University of California San Francisco, is the Foundation founder and can be reached at: lenore.pereira@ucsf.edu). 

Saunders said, “My OB/GYNs didn’t tell me how to prevent congenital CMV, namely by avoiding kissing my toddler near the mouth or sharing food with her, until after my second daughter was born profoundly mentally and physically disabled by the disease. It was then that I received literature stating women who work in daycare, or have a young child in daycare, are at a higher risk for catching it as toddlers are the majority of carriers. While I was pregnant with Elizabeth, I not only had a toddler of my own, I was also running a licensed daycare center in my home. Nowhere in the licensing literature was there a CMV prevention message. In milder cases, children may lose hearing or struggle with learning, but Elizabeth's case was not a mild one.” Elizabeth died at age 16 during a seizure in 2006.

CONGENITAL CYTOMEGALOVIRUS CONTACTS

Dr. Brenda K. Balch, MD, CT’s American Academy of Pediatrics Early Hearing Detection & Intervention Chapter Champion. She has a strong interest because congenital CMV is a major cause of hearing loss. bkbalch@sbcglobal.net. (Sent Testimony to CT’s HB 5525).

Gail J Demmler-Harrison, MD, Professor, Pediatrics, Section Infectious Diseases, Baylor College of Medicine, Attending Physician, Infectious Diseases Service, Texas Children's Hospital, CMV Registry, CMV Research and CMV Clinic. Contact: 832-824-4330, gjdemmle@texaschildrens.org. The CMV Registry supports CMV research, disseminates information and provides parent support. (Sent Letter of Testimony to CT’s HB 5525).

CDC’s Michael Cannon, Ph.D. mrc7@cdc.gov, a research epidemiologist at the Centers for Disease Control and Prevention. Dr. Cannon is particularly interested in developing strategies to prevent congenital CMV infection through public awareness and education.

Lenore Pereira, Ph.D., Founder of Congenital Cytomegalovirus Foundation, and Professor, Cell and Tissue Biology Department, University of California San Francisco. lenore.pereira@ucsf.edu, or visit www.congenitalcmv.org. The Congenital CMV Foundation raises awareness about maternal testing for first infection during pregnancy, newborn testing and the need to develop a vaccine.

Stuart Adler, M.D., Professor Emeritus of Pediatrics and Professor of Microbiology and Immunology, Virginia Commonwealth University. sadler@vcu.edu (sent Letter of Testimony to CT’s HB 5147).

Staley Plotkin, MD, Professor Emeritus of Pediatrics, University of Pennsylvania, Vaccinology Consultant (sent Letter of Testimony to CT’s HB 5147). Contact: stanley.plotkin@vaxconsult.com

Sara Menlove Doutre, President and Education Policy Consultant at Doutre Consulting (her daughter was affected by congenital cytomegalovirus) and co-founder of Utah CMV Council. Contact: sara@doutreconsulting.com  

Stephanie Browning McVicar, Au.D., CCC-A, DOCTOR OF AUDIOLOGY (worked tirelessly to pass the Utah bill), Specialty Services Program Manager, State EHDI Director, State of Utah Department of Health, Children with Special Healthcare Needs, Children's Hearing and Speech Services, (801) 584-8218, smcvicar@utah.gov.

Ronda Rudd Menlove, PhD. She was the representative responsible for passing and enacting the bill in Utah (2013) and has offered to advise CT politicians about the legislation and needed funding. Utah CMV Council, Ronda.menlove@gmail.com.

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