"Claire’s Law- Newborn Hearing Screening was signed into effect on July 1, 2008 by Governor Bredesen."

About: Claire (3)

Age Range: 3 to 5 years

Perspective: Student interview of parent

County: Davidson

Michelle and Matt have been married for 7 years. Michelle works in a Kindergarten classroom and Matt works at a high school. Their first child, Claire, was born in 2006 in Nashville. At birth there was no diagnosis of a disability, but at 4 months that all changed, and Claire was diagnosed with profound sensorineural hearing loss.

Matt was the one to first suspect Claire’s hearing problems. And one day, he blew a whistle from behind Claire, while she was sitting in her highchair in the kitchen; when she didn’t flinch they knew that something was wrong. When Claire was 4-months-old they went to the pediatrician to have a test done. It was determined that Claire had severe hearing loss in both ears and they were referred to the Vanderbilt Bill Wilkerson Center for more testing.

It was discovered there that Claire had been born congenitally deaf, and since a newborn hearing screening test was not required in Tennessee at the time of her birth, one had not been preformed on Claire at the hospital. It had gone unnoticed.

Soon after the diagnosis, the family decided to have genetic testing done in order to determine what had caused Claire’s hearing loss, since neither family had a history of deafness. It was revealed that Claire has a Connexin 26 and 30 genetic disorder. Both Michelle and Matt are recessive carriers and there is a 25% chance that any child they have will be deaf.

Michelle praised the Vanderbilt Bill Wilkerson Center for throughout the process of diagnosis and testing being an amazing resource and a great help in dealing with Claire’s deafness. At the Bill Wilkerson Center, Claire received cochlear implant surgeries.

Prior to her surgeries, Claire was fitted for hearing aids in both ears. She was required to wear these hearing aids for at least six months, by insurance company regulations, before cochlear implants could be considered as an option (this was before the 1 year age requirement for cochlear implantation). However, the hearing aids were not helpful for Claire because of the extent of the severity of her hearing loss; she needed more than just amplification to be able to hear.

However, the family waited out the required time with two hearing aids and a week after her 1st birthday in 2007 Claire received her first cochlear implant, and a few months later she received the second. Michelle decided to take a year off from work to focus on Claire’s rehabilitation. Because during that year not only did Claire have to have two major surgeries, but she also had to have her cochlear implants tweaked and adjusted to fit her in the months after implantation, and needed to go to speech therapy and a number of meetings with her medical team at Vanderbilt. Claire is part of the Auditory-Verbal Therapy program, which is uses therapy to enhance both the listening and speech skills of children with cochlear implants, which has been proven to be very successful, especially in young children.

Claire has responded extremely well to her implants and therapy, Michelle has had others tell her that you almost can’t tell that she is deaf because of the progress she has made in her speech and listening skills. Though Michelle and Matt have yet to really address Claire’s deafness to her, as she is 3 years 10 months old she doesn’t understand much more than that she has “special ears”. She is very attached to her implants and at times forgets that she has them on. In the bathtub for instance, when she has to take off the implants, Michelle says that she doesn’t even realize that they are not there. They are so much a part of her; she’ll keep talking in the water as if she could hear. Claire works hard in speech therapy every week, but she doesn’t seem to think of herself as being any different than other children she goes to school with.

At this time, Claire attends the Susan Gray preschool on Vanderbilt’s Peabody campus. Michelle says that they ended up choosing the Susan Gray School for Claire so that she could have peer models and a more inclusive setting. The Susan Gray School is comprised of students both with and without disabilities. Claire has been at Susan Gray since she was 1.

The family is currently going through the assessment process before Claire’s IEP meeting. Verbally Claire scored in the 96% compared to hearing children (an actual age of 5 years 10 months) and expressively she scored in the 99% compared to her peers (an actual age of 6 years 2 months). Her impressive scores mean that she will be able to integrate into the regular education classroom when it is time for her to enter Kindergarten. And most likely, the only service she will require from her school in her IEP is an FM system in the classroom, as she uses currently in preschool.

Currently in spite of all of the progress she has made, Claire still has some health problems. She has cholesteatomas in both of her ears. Cholesteatomas are benign tumors, unrelated to her hearing loss, formed because her ear canal had started to collapse and formed tumors. They did not affect her hearing because the implants bypass the ear canal and do not use that part of the ear for hearing. However, removing them is a more complicated and sensitive a surgery when a child has cochlear implants. It is essential to make sure that in removing the tumors the doctor does not disturb the implants. Claire’s doctor performed a breakthrough surgery to remove the cholesteatomas from her ears. Michelle described the surgery as one more sign that it is never over. The cochlear implants are not a perfect or permanent fix, and parents have to know that you just don’t ever know what will happen and have to be able to roll with the punches.

Newborn Screening tests are designed to seek out developmental, genetic and metabolic disorders so that if found, action can be taken during the most critical stages before symptoms can further develop. All 50 states are required to perform tests screening for 21 or more disorders in order to be sure the child is clear of any disorders that could potentially lead to physical problems, mental retardation or even death. The March of Dimes Foundation is one of the forerunners in the advocacy of babies’ health trying to prevent birth defects, premature birth and infant mortality through research, outreach work and education. A report by the American College of Medical Genetics endorsed by a strong recommendation from the March of Dimes urges newborn screenings for 29 disorders. Currently there are 30 states screen for all 29 disorders (March of Dimes, 2010).

Here is a brief list of the disorders babies are screened for before the ever the leave the hospital in the state of Tennessee: Phenylketonuria (PKU), Congenital Hypothyroidism (CH), Galactosemia, Sickle cell disease, and Congenital Adrenal Hyperplasia. When Claire was born (2006) the state of Tennessee was among few states without a required hearing screening for newborns. When the family discovered this after finding out about Claire’s profound sensorineural hearing loss, both parents were confused as to why this form of a test was not required for Tennessee. Strongly believing that every child deserves to be screened for hearing impairments at birth, Claire’s mother, in her year of leave of absence from teaching, began a long, but very rewarding journey to get a law passed for this screening to be implemented.

During her leave of absence, Claire’s mom began advocating for a bill to be passed to require the state of Tennessee to screen all newborns for hearing impairments. Her process began with a trip to the state legislator with Claire in her stroller. She asked around trying to find someone to present her story to and eventually she was given the opportunity. Legislators from all over the state began asking her about her specific case and how her journey with Claire has been. She typed a one page summary of explaining Claire’s condition, how her and her husband became aware of her disorder, problems and benefits of the Cochlear Implants, and Claire’s overall success and failures of her first 3 years of life.

During this process, She discovered legislators had been trying to pass a bill of this sort for a while but just haven’t been able to find a family to connect the bill to. After her efforts in passing this bill were evident it almost became a shoe-in. Before the bill was sent before the committee, the legislators on the case asked her if it was okay if the law was named after Claire, and she answered with a huge smile on her face, “Of course not. I would love for the law to be named after her.”

Claire’s Law- Newborn Hearing Screening was signed into effect on July 1, 2008 by Governor Bredesen. The Tennessee Disability Coalition (TDC) ­is the organization who led the efforts for this bill to be passed. Their belief to advocate for public policies to ensure inclusion, empowerment, independence and self-determination (TDC, 2010) helped Claire’s family attempt to help protect all future babies who may have hearing loss in the state of Tennessee.

Updated on Thursday, December 8, 2011