This fall Rachel Skergan will be four years old. Like any typical girl her age, Rachel is afraid of the dark and blessed with a beautiful laugh. She loves to read books, and even though Rachel has profound hearing loss in both ears, her language and social skills are on level with her same-age peers. Rachel’s mother, Natalie Skergan, a nurse practitioner, attributes her daughter’s success to bilateral cochlear implants (CIs), a supportive family and helpful professionals.
Rachel’s parents were shocked when they learned that Rachel had failed her newborn hearing screening in both ears. “Initially we were very upset by this until we were able to get a grasp of what we needed to do for her,” Skergan remembers. “Our family was devastated when Rachel was diagnosed with hearing loss and we really didn’t know what was possible for her at that time. We quickly learned all we could and gathered all of the information necessary to find the best treatment option for her and we were excited by the realistic possibility of her learning spoken language.”
Because they were told after her newborn hearing screening that Rachel’s problem could potentially resolve given time, the Skergans waited through two re-tests before Rachel was finally diagnosed with bilateral severe to profound sensorineural hearing loss, which was attributed to a gene mutation, connexin 26. Rachel began wearing hearing aids and receiving auditory-verbal therapy when she was three months old.
“We were very diligent with doing the therapy exercises but she was not making the progress she should have been making with the hearing aids. After seven months of wearing them she was just making one vowel sound. She wasn’t babbling or anything when she was 10 months old,” Skergan recalls.
The Skergans began researching CIs and selected the MED-EL brand. At 10 months, Rachel was implanted in one ear.
Rachel’s parents contemplated bilateral implants from the start, but in 2004 when she was implanted, bilateral implants were still quite uncommon. Also, Rachel had residual hearing that they were trying to stimulate with a hearing aid. Around age two, Rachel began to reject her hearing aid by saying “No, thank you” and handing it back to her parents. That was when they decided to look again into bilateral implants, especially since it had become a more accepted procedure.
Rachel received her second MED-EL CI in May 2006. At the recommendation of the professionals, the Skergans took away her first CI “cold turkey” until her speech and hearing skills with the second implant only were comparable to those she had developed with the first CI. It took about seven weeks and then Rachel was allowed to wear both CIs and has done so ever since.
The Skergans are hopeful that Rachel will not have to undergo surgeries every 10 years to replace the implants but they are realistic about the possibility of Rachel needing a surgical revision of her CIs at any time. “There are some people who have had an implant in for 20 years. There’s one girl who had had hers for 20 years and not had a failure. But it’s hard to say with electronics when and if a device is going to fail. She is going to have to have another surgery or two or three. It’s not going to be guaranteed for life. We look at it as a good thing because she’ll be able to get the updated technology at that time,” says Skergan.
Rachel attends a private, mainstream preschool and receives auditory-verbal therapy from a therapist – part of Rachel’s individualized education plan (IEP). “I really think that at the rate of progress she’s making, we will see her move from an IEP to a simple plan for accommodations. She’s doing so well because of her therapy.” And Skergan reiterates how essential CIs are to Rachel’s progress: “She can’t read lips well enough and she doesn’t know sign language. She could not function in the classroom. She needs her cochlear implants.”
Skergan says they initially thought of a second CI as a backup but have since realized the greatest benefits are Rachel’s improved hearing in the presence of background noise and the ease of listening overall. “David and I are on a daily basis looking at each other in amazement. I mean she really amazes us. We don’t take this for granted at all. It is a modern medical miracle.”
Indeed, sound localization and hearing in noise are the justifications their insurance company used in deciding to pay for a second CI. “We were fortunate that we had good insurance that covered the CIs,” says Skergan.
For those whose insurance companies have denied their claims or refuse to provide coverage, Skergan recommends the Let Them Hear Foundation (www.letthemhear.org.) The foundation helps patients at no cost to appeal denied insurance claims for CIs.
“We’ve all grown from this,” reflects Skergan. “We’ve met lots of professionals and other parents and children. We’ve become friends with people we wouldn’t have met if we hadn’t experienced all of this. Hearing loss is not life threatening but it is quality of life threatening and we wanted to give our daughter the best quality of life possible. We also wanted to raise her to be independent and to be able to thrive in this world and most importantly, to be happy. We have had a very good road with Rachel. She is very happy and well adjusted, very confident, loves preschool and has many friends. I just want to help other families and children have that too.”
Contact Natalie Skergan at skergan@nc.rr.com.
Donate Now!
DRF funds research that changes lives.