A True Champion-John's Story

John's teacher Lisa, John, therapist Courtney

You may remember reading about John a few months ago on Bama Ears when we highlighted his mom Jennifer. Click here to read that story if you haven't already. A lot has changed since our summer story on this family of four. When Bama Ears last spoke to the family, Will (John's younger brother) had bilateral cochlear implants and John was wearing bilateral hearing aids. Both boys are deaf as result of Connexin 26. Over the summer, this family received the news that John's hearing and aided benefit had declined to the point where his audiologist decided that he was now a cochlear implant candidate. Below you'll find the whirlwind of events and decisions that led this first-grader into the operating room, where he received bilateral cochlear implants last Monday then walked back into his classroom on Thursday, just three days after surgery! I encourage you to read the first story if you haven't already and you will see exactly where John gets his bravery.

Mom Jennifer writes:

It was a whirlwind decision making process once we knew where we were headed: one side or both, to wait for a break in school or operate as soon as possible, etc. We first thought we would go with just one side, and wait for a break such as Columbus Day to do the other side. Things changed tremendously when we consulted his teacher Lisa, who happens to be the mother of a hearing-impaired, bilateral cochlear implant-wearing precious baby girl (click here to read their story). Lisa advised us to go as soon as possible in order to get him up and running and caught up before the 2nd 9 weeks of school. We finally got the anticipated insurance approval and with all that falling into place, we looked at September being "his month." Dr. Woolley was fitting us into his last bilateral slot in order to take advantage of a trial Cochlear was doing with the imminently-FDA approved Nucleus 5, which I had only recently become aware of thanks to Val (a Cochlear Volunteer) and her network. One week before his scheduled surgery, the FDA announced its approval of the Nucleus 5. At our pre-op appointment with Dr. Woolley that week, I became fully aware that God of course was in charge, and that my son was going to be just fine in the best hands possible (Dr. Woolley's and Lisa's), and we were getting the Nucleus 5s, the newest cochlear implants on the market! My mourning and grieving for John's hearing were over.

It took some jumping through hoops from our cochlear implant team, but the newest devices arrived and were ready on surgery day. John was so brave and excited about his new implants. Lisa and my husband had done a wonderful job preparing him for what was to come. That doesn't mean he was thrilled about being in the hospital, but he truly was inspiring with his bravery. It took around six hours for the surgery to be completed and to give him the chance to hear. We spent the night in the hospital, and he woke up once or twice and asked about his bandages. Finally at 5:45 he awoke and said, "When can I wake up?" That first day home, he expressed some concern about his partially-shaved head and had a little pain. He was down from his usual energetic self, but that afternoon he began receiving visitors, including his teacher Lisa, and Courtney, his speech therapist at school. That's all he needed to bounce right back. He began getting little gifts and sweets, and he has never been down since. The next day he was running in the yard again, and we took the dog for a long walk. We thank God he can read, so we can communicate with him by writing.

Last Thursday, 3 days after surgery, John showed his bravery once again. He entered his classroom, a little self-conscious about his hair and with no hearing. Right now he goes in for a couple of hours to read and do some of his assignments and take appropriate tests. Lisa had the class make pictures for him, and a student held up a sign that said, "Your haircut looks cool." It was an amazing moment! Another parent has told me how much her daughter has learned through all of this. John has rarely if at all complained. He is excited about this Wednesday, when we begin the activation process. I believe he will get Freedoms first, because the Nucleus 5 processors don't roll out until October. That is how under the wire all of this was!

I have met some of the most wonderful people of late. I happened to meet a man at the barbershop 3 days before surgery who had a C.I.! He is a lawyer who used to work with my husband, and I met a woman whose 4-month-old will be having this surgery in the next year. While I was introduced to her in order to tell her how great her daughter will do, she ended up comforting me at the beginning of our process as I mourned for John. Having been through this already with our younger son Will, hearing the news that your child needs surgery can be scary at first.

The Cochlear marketing rep for this area, Scott Rinehart, had a local informational meeting the day after John's surgery. I attended the meeting and met some wonderful people there as well. I am so incredibly grateful for my friends who are parents of wonderful hearing-impaired children – people I wouldn't have known otherwise but who I now cannot live without! Of course our doctor, nurses, audiologists and therapists all fall into that category as well. Finally, I have the pleasure and honor of working with Lisa's daughter at The Bell Center for Early Intervention Programs, where I volunteer. I have no doubt that God is taking care of all of us.

Will is enjoying his time at the Alabama School for the Hearing Impaired, a program of the Children's HEAR Center. He is also taking good care of his big brother while we continue his journey toward hearing again.

Bama Ears will continue to follow John on his journey and update his story after his activation, when he begins to hear sounds he never knew existed.

Kate's First HEARING Birthday

Kate celebrated her 1st Hearing Birthday with friends and family in August. She has made great progress and enjoyed celebrating and having party! Kate's genetic testing results showed that she did have hearing loss due to the Connexin 26 gene. She should have no other problems and is doing well with her cochlear implants. Click here to read Kate's story previously published on Bama Ears.

When Kate's not celebrating with her family and friends, she's having a blast at gymnastics class.

Happy First HEARING Birthday Kate!!!

Bama's Bright Light

Every so often Bama Ears would like to shine the Bright Light on an exceptional parent. Our first to highlight is Mrs. Jennifer Andress. Her two children are hearing impaired due to Connexin 26. John (age 6) is currently using hearing aids, and Will (age 4 ½) wears bilateral cochlear implants. Jennifer serves as V.P. of the Service Guild, which is a service organization solely dedicated to The Bell Center’s Early Intervention Programs. When she’s not running marathons chasing after her two boys, she's running them for The Bell Center partners-in-training program for the Mercedes Marathon. She and husband Keith also run in Atlanta’s Thanksgiving Day Marathon as well as other races in our area. Looking at her active lifestyle you would never guess her past. Here’s a little hint ...

(She and her son John are closing the van's back door.)

When her first child, John, was 11 months old, he needed tubes for his ears, and this is when they found out he had a hearing loss. With Jennifer already carrying baby number two, John was fitted for aids and soon after, she found out she had precancerous growth in one of her breasts. She has a long history of breast cancer in her family so while 24 weeks pregnant and a 1-year-old, she had a mastectomy. A few months later baby Will was born, and she soon began radiation. But before that treatment began came Will’s hearing tests, in which he was also determined to have hearing impairment just like his big brother.

A few months after completing her radiation, Jennifer had a second prophylactic mastectomy and breast reconstruction with an eight-week recovery period. Remember, Jennifer also has two hearing-impaired children to care for, but she manages along with her very supportive and loving husband. Within weeks of her final recovery, baby Will had his first cochlear implant surgery and a year later had his second.

Jennifer says she’s had great support throughout from all of their doctors, health care professionals, church, friends, family, and notes “God is great!" Today when she’s not making commercials for Breast Cancer Awareness, or helping with the Bell Center’s service organization, or running half-marathons, she’s preparing John for first grade and still has a year to prep Will for kindergarten. Congratulations, Jennifer, Bama's Bright Light is shining on you, a true inspiration.

If you would like to nominate someone as an exceptional parent for Bama's Bright Light, please email us at bamaears@yahoo.com . They need to be an Alabama Parent of at least one child with hearing loss.

Under the Microscope

When you find out your child has hearing loss, often times you are offered genetic counseling/testing. Why would parents want to have their genes or their child's genes put under the microscope? Should we just accept that “it is what it is” or “it was just meant to be?” It is a very personal choice, and we wanted to give you our thoughts on why we chose genetic testing. There are several benefits of genetic counseling:

1. As children get older, they will have questions such as “Why me?” or “Why don’t my ears work?” or a number of other questions that you need to be able to answer.
2. Also, when children become adults and want a family of their own, is there any likelihood that they too will have deaf children? Or was their hearing loss an isolated case?

3. Having the counseling can also help you answer questions such as, "What is our risk to have another child with hearing loss?" "Should we have more children? Do we want to have more children?” Some people really struggle with these questions that can be an emotional concern as well as a financial and a major health concern.

4. Health! Up to 40 percent of children with hearing loss have other issues, which is almost half of the kids with hearing loss (according to www.raisingdeafkids.org/speical)! Sometimes children may have an underlying syndrome, and hearing loss is secondary to that syndrome. Many kids with syndromes may appear to be fine to the untrained eye, but with a full exam from a genetics specialist, it may be determined that the child has syndromic hearing loss. Finding this out ahead of time can really be beneficial. A child with a syndrome may need other testing to determine if there are other complications or concerns that need to be addressed. Sometimes as a child with a syndrome ages, other things develop that can cause concern that could possibly be addressed earlier. The child could develop cognitive delays, growth abnormalities or visual impairments, ADHD, or even more serious developments such as kidney or heart or spinal problems. Having these issues addressed in the beginning can help you decide which follow-up care or continuing care to pursue for your child. It helps you be on the lookout for certain issues to develop, and if they do, you’ll know whom to contact.

5. Relief. Genetic counselors can also test for nonsyndromic hearing loss. For example, a mutation in the connexin 26 gene (called GJB2) causes only hearing loss, so if your child has that gene mutation and nothing else, you’re pretty well assured that hearing loss is all you’re dealing with. Mutations in other genes can be associated with other medical problems.

We wanted to talk more with Dr. Nathaniel Robin, Professor of Genetics and Pediatrics at UAB, on the subject of genetics and here’s what he says...

"As you mentioned, there are many benefits to a visit to the geneticist. But because very few people even know what a geneticist is, they are often nervous about coming to see us. A visit to the geneticist is very similar to any other doctor visit in some ways, but very different in others. For example, we spend a lot of time getting background information on your child as well as your family. Then, when I examine your child I look for very subtle things, like facial characteristics. Are the ears and nose normal in appearance? Are there unusual birthmarks, or fingerprint patterns? This is mostly done just by looking and observing, very little poking. If there is no other unusual findings, we often recommend testing for isolated hearing loss genes. However, if there are other findings we discuss what they mean, and what testing if any should be carried out. Genetic testing typically involves nothing more invasive than a simple blood draw.
All in all, most people find these visits helpful, as many questions are answered, or at least addressed.
A visit can get very emotional, and parents will often become upset because we are discussing potential health risks for their child. We always follow up each visit with a comprehensive and detailed note, as well as additional reading material as appropriate. Another often unspoken concern is if a genetic evaluation and testing is covered by insurance. The answer is almost always yes, but if that is a concern we can check before your visit.
To make an appointment with UAB's Genetic Clinic call 205-934-9528."

So you can see, at least in our opinions, a visit to the genetics office can be quite beneficial. Talk to your audiologist or ENT about genetic testing if you are interested and they will surely point you in the right direction.

Tiny Dancer

Riley and her six Little Mermaid classmates pranced onto the stage in front of about 2,000 at the VBC and did their routine to "Kiss the Girl" like it was no big deal. She knew the dance better than her teacher, who told me as much after the show. She didn't miss a beat or a step ... not bad for a deaf girl! Those cochlear implants really work.

Here are a couple of pics, and a video from the dress rehearsal (complete with curlers).

Chloe's Story

My name is Nicole. My niece Chloe is almost 18 months old and has bilateral Cochlear implants. She was diagnosed at 5 1/2 months old with severe-profound sensorineural hearing loss that was hereditary. Chloe initially failed her newborn hearing screening, but later passed it at her first well-child check-up. At almost 4 months old her parents, Paul (my brother) and Candace, became concerned about the possibility of hearing loss, when Chloe did not wake up to the sound of a skill saw, which was running in the next room. Later that week at her 4 month check-up, these concerns were expressed to her pediatrician and a hearing test was performed. Chloe did not pass the screening and was referred to Children's Hospital where her hearing was further evaluated, again with the same results. On April 19, 2008, Chloe had tubes placed and ABR testing which revealed "at least severe hearing loss." About a month later, Chloe received her hearing aids, and again the news was not good. She had a delayed reaction to extremely loud sounds, which meant being aided by hearing aids alone would not allow her enough access to sounds to develop speech. She was diagnosed with a profound hearing loss.

Chloe at 8 months of age, we had to get creative and keep her busy hands away from those hearing aids

With the diagnosis of profound hearing loss, our journey to cochlear implants began. Chloe was evaluated by the HEAR Center and she was soon a candidate for bilateral implants. Her surgery date was set for November 14, however in mid-October we received the news that her insurance company had denied payment for her second implant. Their reason: bilateral implants are not "medically necessary." That answer was not acceptable. The surgery date was rescheduled for December 8 and then December 15 to allow time to resubmit the claim. Meetings were attended, calls were made, but we were getting no where as the claim was again denied. FINALLY on December 10, after a trip to Montgomery to plead Chloe's case, just 5 days before her surgery date, the claim was not only approved for Chloe, but for any other child needing bilateral implants with this insurance company. Coverage of bilateral cochlear implants would now be their policy.

December 15, 2008 (just after her first birthday) The big day FINALLY arrived. After 6 hours in the OR, we received the GREATEST news EVER....

Chloe's surgery was a success and responses had been detected from electrodes in each ear. CHLOE WOULD HEAR!!!
Chloe the following morning after her bilateral surgery

Chloe's Activation Videos

One Month Later...
I was completely amazed at her progress in just one short month. Chloe started babbling and repeating sounds. This child was completely silent just one month before these videos were shot.

Now, at almost 5 months post-activation, Chloe continues to learn new sounds every week. She goes to AVT (auditory verbal therapy) weekly and mapping sessions (audiology) about once every 3 weeks. Her vocabulary includes: more, mouth, mama, bye, hi, "g" sounds, and "y" sounds. She is imitating more and more sounds every day. Check my personal blog for more updates on Chloe's progress: http://www.kaylynandluke.blogspot.com/

Written by Aunt Nicole