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.

CRS-Children's Rehab Services of Alabama

Bama Ears recently had a chance to talk to Emma, a Social Work Administrator for CRS (Children's Rehab Services). We asked Emma to explain what CRS is and who is eligible. Here is what she told Bama Ears.

"Established in 1935, Children’s Rehabilitation Services (CRS) is a division of
the Alabama Department of Rehabilitation Services. CRS is a statewide
organization of skilled, well-trained professionals committed to providing
quality, individualized services to children and youth with special health care
needs in homes, schools and other community settings.
Any child younger than 21 who is a resident of Alabama and has a special health care need is eligible for CRS. Individuals of all ages with hemophilia are eligible. Families participate in the cost of services for their child based on a sliding fee schedule and by using any Medicaid or insurance coverage."

What services does CRS offer?

"CRS services are available to children and youth in every county in Alabama through a network of 15 community-based offices. The CRS network provides specialty clinic and other services by doctors, nurses, social workers, physical therapists, speech-language pathologists, audiologists, occupational therapists and registered dietitians. Services include medications, surgery, assistive technology, hearing aids, hospitalization and much more."

What happens when a child ages out of Children's Rehab Services and they still need help?

"All adolescents in CRS receive transition services from a trained social work transition specialist. All eligible CRS youth are referred to Vocational Rehabilitation Service (VRS) to receive assistance with college or job placement. VRS provides specialized employment- and education-related services and training to assist teens and adults with disabilities in becoming employed. In addition, VRS works with middle schools, high schools, junior colleges and universities statewide to assist students with disabilities in receiving appropriate educational opportunities.
The ultimate goal of CRS is to transition youth with special health care needs to all aspects of adult life, including adult health care, work and independence."

What if a child has more than one disability? Are you guys equipped to handle multiple needs?

"CRS has well-trained staff that work on a daily basis with children and adolescents who have dual diagnoses. We offer specialty clinical medical programs and clinical evaluation programs in order to meet the needs of this special population."

Bama Ears specifically asked Emma what CRS can do for our hearing impaired children in Alabama.

"CRS offers hearings services, which include Newborn Hearing Screening, Hearing Assessment Clinic, Hearing Clinic and Hearing Aid Clinic. CRS audiologists have the experience to provide top-notch audiological services to Alabama's children. With state-of-the-art testing equipment, children receive the same quality evaluations on a local level that they would receive in larger facilities. If a child is found to need hearing aids, the audiologist has a vast selection of hearing aid companies and products to choose from. Many of these products would cost the parents $1,700 to $3,600 per set if purchased from a private clinic."

For more information, visit the CRS website

Bama's Bright Light

Rachel from "Signing Time" with Julie and Adele

This month, Bama Ears is shining its bright light on Julie Brandrup, mom to Adele and Margo. This busy mom just returned from Chicago where the CHARGE conference was held this year. If she and daughter Adele look familiar, it may be because they have been in many magazines and on TV as well, featured in Blue Cross/Blue Shield advertisements. Before she had Adele, Julie actually worked in advertising and branding. She says that her experience in finding the positive angles of each product and enhancing them has really come in handy since she's become a mother to a special needs child.

Julie has a positive outlook on everything, right down to her own health. She's had two back surgeries since having Adele (one while pregnant with her now 2-1/2-year-old). She notes that despite all of that, she rediscovered her love for swimming, which became helpful for her postsurgery back. She was also forced to rely on some great baby-sitters since she couldn't even lift anything for six weeks after her surgeries. Those sitters also come in handy when she and her husband have their date night, which has always remained somewhere on their busy calendars. Julie and her husband Jay actually share more than this lovely family – they also share the same birthday (Julie is older by two hours). They can often be found at the annual "Rhinestones and Wranglers" charity event held by The Charity League of Birmingham, or other charity events for UCP and the Bell Center . Jay is actually on the Fundraising Campaign Committee for the Bell Center and a UCP board member. Julie says her husband and family have been unbelievably supportive through everything. She also says,

"I am also thankful to God, who undoubtedly provides the strength I need every day to rise to the challenges of having a child with special needs; without Him we would be a big mess."

We are proud to be shining Bama's Bright Light on Mrs. Julie Brandrup. Next week, read more about Adele's experiences and how she is learning to communicate and how her little sister Margo is also picking up language in a variety of forms as well! Julie helps many families each time she shares Adele's background, and we are pleased to bring you more in next week's article.

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.

Little Lilly

Lilly is 19 months old with bilateral cochlear implants, or “magic ears” as I tell my young curious students. We couldn’t be happier that Lilly has finally entered the hearing world, although the road to get here was not an easy one.

Lilly failed both hearing screenings in the hospital when she was born, and we were told not to be concerned. However with years of experience with infants I knew this was no ”vernix.” We were able to see the audiology team at Children’s ENT two weeks later and were told before she was 3 weeks old that Lilly had a profound sensorineural hearing loss. At 7 weeks old, Lilly received hearing aids and so began the challenge of keeping those little suckers on her head and ear molds in her ears!

And then it all sped up; it seems like a blur now. AIDB (Alabama Institute for the Deaf and Blind) assigned us a case manager who did an initial screening which confirmed other delays. As a result, she assigned to us a physical therapist to go along with the speech therapist and, of course, the audiology team we have come to know and love at the HEAR Center. We saw a genetics specialist at UAB, a neurologist, an ophthalmologist and, of course, Dr. Woolley (a.k.a. the Guru, big dog, head cheese, our hero ...) Lilly even began supplemental therapy with the Bell Center to try to close the gap in her gross motor delays. CT, MRI, Muscle Biopsy – more bad news. We felt like we were drowning – sound familiar?!? It felt as if the not knowing why and not knowing what to do were the worst.

Causes were sought out; CMV was the culprit for a while so we went to a CMV clinic. Results were inconclusive; she had CMV, but not congenital CMV. After a muscle biopsy, it was determined that it was Mitochondrial Disease that caused abnormal white matter in the brain, low birth weight (4.15 lbs) and congenital hearing loss.

But then things got better, as they always do. Lilly continued to make progress with her motor skills, just at her own pace. Pilot caps kept the hearing aids on! She made it into the 10th percentile for weight on the growth chart! (woo-hoo!) And the decision for cochlear implants was made (a double-edged sword but finally a decision!)

Lilly’s first cochlear implant surgery was the day after her 1st birthday. Recovery was hard, but after the activation, we had the best Christmas of our lives! (see video below)

There was a battle for the insurance company to approve the second surgery, but thanks to Papa Jack, it was able to take place on a very lucky Friday the 13th. It’s been almost 2 months since that activation and her language development is astounding!

Lilly identifies animals and their sounds, practices the Ling 6, loves to read books (identifying even non "Learning to Listen" items), she crows like a rooster and growls like an Auburn tiger! She can say more please, bubbles, Mama, Daddy, Nana, Katie, Pop, doggie, eat, hot, hat, up, ball and something new every week! Oh, and Lilly LOVES to sing!

Our journey is not over, but we now have a stable vehicle to travel in! We are still waiting for more results of the biopsy that determined the abnormal mitochondria, and we will then visit a specialist in Atlanta. Although Lilly wears custom splints, she still prefers to “cruise” rather than walk. And the new tubes Dr. Woolley gave us will hopefully ward off any other ear infections.

Wow! All this happened in less than 2 years. It sounds crazy when I type it all out. The tears of sadness and heartache were definitely worth the tears of joy and amazement. I look forward to meeting you in the waiting rooms!

Written by Lisa, Lilly's mom

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