Looking to the Light (Part II)

(To read Part I, click here)

Praise God everything looked normal in the MRI.

My husband and I definitely know that God has his hand in our journey with Ella Marie. We know that "our plan" is not always the same as his. Looking back, we can see how God has prepared us for this point. For instance, my degree is in elementary education. I have had the opportunity to work with a child with a cochlear implant. I love to teach children how to read. Since Ella Marie will most likely have difficulty in her oral language development and reading and writing at first, I have the patience to work with her and try many strategies with her. My husband is the most loving and patient person I know. Ella Marie already has him wrapped around her little finger. He is such a hands-on daddy. We are fortunate that Ella Marie will have parents, family, therapists, and friends committed to her pathway of learning.

Where We Are Now:

Ella Marie will be having surgery in September, 2010 at Children’s Hospital with Dr. Woolley as her surgeon. She will be 15 ½ months old. Actually, Ella Marie was scheduled for surgery on August 9, 2010, but caught bronchitis in late July. Children’s Hospital informed us that any time a patient has an upper respiratory issue, surgery must be rescheduled for 4-6 weeks from the time symptoms are gone. As mentioned above, we had a feeling that Ella Marie would be a candidate for bilateral implants. We were definitely headed in that direction. We wanted them as fast as we could have them. We battled with the decision on having simultaneous or sequential surgeries. On the one hand, one surgery means one recovery, activation, and stress on us. On the other hand Ella Marie has done extremely well with her hearing aids. According to testing she is not showing a delay in language at this time. I have read a lot of research and had her tested multiple times at multiple hospitals/pediatric audiologists/cochlear implant centers, and decided to try one implant at this time while knowing that we may choose to implant the other ear soon. It was a very hard decision because this was a complete 180 in the decision that we first thought we had made earlier in the process. Ella Marie attends Auditory Verbal Therapy at the Hear Center every other week and will receive therapy weekly following her surgery. Through genetic testing, we discovered she has Connexin 26. I have started a blog for Ella Marie describing our journey. I am currently typing up all of my written notes, and it should be updated soon. You can access and follow our blog at Learning to Hear God. In the blog, I try to cover all of the activities and developmental play that we engage Ella Marie in which has made her blossom.

How God Has Worked In Our Life Through Ella Marie

I titled this “Looking to the Light” a very special reason. Being our first child, Ryan and I had nothing to compare Ella Marie to. If she was crying, we didn’t know if she was hurting or if it was due to just being a baby. Being prone to ear infections, we knew that she cried a lot from those. One thing that we noticed when she was very small was that when she was crying or very upset there was one thing that would make her feel better and comfort her. She loved the chandelier light in her bedroom. When she would start crying uncontrollably, Ryan or I would stand under the chandelier and raise her up and down under the light. She was fixated on the light. It always made her feel better. What a lesson to us! Throughout this process there have been a lot of ups and downs. There have been a lot of days where I cried and wanted things to be different but Ella Marie taught us that we need to “Look to the Light” just like she did. Just like Ella Marie, when I looked upward I felt better. When I feel like life has gotten so complicated, Ella Marie was teaching me a simple lesson “Look to the Light”, Jesus Christ. Although Ella Marie’s hearing loss was not what we wanted, when we focused on the Light, God began to unfold His plant on how he had prepared us our whole lives for having a little girl with hearing loss. From teaching a first grade student in a previous year with a cochlear implant to my career choice as first a first grade teacher and currently university professor where my focus is early childhood language and reading, to providing us with supportive family and friends who all pitch in and are committed to supporting Ella Marie. God taught me this lesson through our little girl. We are committed to serving as a resource for parents of children with hearing loss.

Below, I have listed a few Bible verses that have spoken to us throughout this journey.

When Jesus spoke again to the people, he said, "I am the light of the world. Whoever follows me will never walk in darkness, but will have the light of life." John 8:12 (NIV)


Look to the Lord and his strength; seek his face always. I Chronicles 16:11 (NIV)


For I know the plans I have for you," declares the Lord, "plans to prosper you and not to harm you, plans to give you hope and a future. Jeremiah 29:11 (NIV)

Lisa Clayton, mom to Ella Marie

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!!!

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.

The Genes We Wear....

“What does it sound like to take a bath?” This is just one of many questions I’ve been asked since becoming a parent to deaf children. I had to frantically search my mental thesaurus to describe to my children how the water sounds to me but feels to them. By day, they wear cochlear implants, electronic devices where one part is surgically implanted under the skin near the ear. A second part, the external processor, is removable by the patient during activities such as swimming, bathing and sleeping. Once removed, my children are in a world of silence.

My son Gage, was born with Goldenhar Syndrome, explained by Dr. Nathaniel Robin, Professor of Genetics and Pediatrics at UAB. “This is a rare craniofacial disorder in which affected children have underdevelopment of the face and ears, with one side being more severely affected than the other. Hearing is often affected, and other birth defects are common. These include abnormalities of the eye, spine, heart and kidneys. While the physical appearance may be striking, the vast majority of people with Goldenhar have normal intelligence. This is why early identification and correction of any hearing deficit is so important.”


Among many other abnormalities noted at birth, he failed his newborn hearing screening ... he was profoundly deaf. Little did I know that his little sister Brooklyn, born two and a half years later, would follow his path to silence. Passing her newborn hearing screening and with no apparent syndrome, you can imagine my surprise when I found out she couldn’t hear well at nine months of age. Her brother had just received a cochlear implant, so shouldn’t I have seen the warning signs?


Unlike her profoundly deaf brother, she had some hearing, so if I raised my voice or moved in closer, she could hear me. Having one deaf child already, this was “our normal.” Her progressive hearing loss soon led her to qualify for cochlear implants also. Knowing that Gage had a syndrome, a reason for his deafness, we decided to have genetic testing on the whole family since she may question her hearing loss later in life or when she decided to have children of her own. No genetic links between the two were found as reason for their hearing loss.

As Dr. Robin further explains, “Over half of all hearing loss in children is caused by genetic factors. In most cases, these genetic anomalies cause no other abnormalities, and are therefore termed ‘isolated.’ In other cases there are associated problems, and the hearing loss is called ‘syndromic.’ But the genetic cause is not known for all syndromes. Goldenhar is one such example. While we believe it is low, we cannot with certainty state what the likelihood is that Gage or his siblings will have a hearing-impaired child.”


Ironically, it is possible for deaf children to become good listeners. According to Natalie Baldwin, an Auditory-Verbal Therapist/Speech-Language Pathologist at The Children’s HEAR Center, “It is my job to educate the family how to stimulate speech, language and auditory development in their child. Through weekly sessions, we target vocabulary, language, listening and speech tasks that will help the child develop just like their typically hearing peers.” Natalie warns that even a minimal hearing loss can impact a child’s speech and language development. If you have concerns regarding your child’s hearing, she suggests you ask your pediatrician for a referral to a pediatric audiologist.

It’s common practice at my house to narrate daily activities. In fact, this part of auditory-verbal therapy could benefit almost all children, with or without hearing loss, since its focus is language input. When it’s time to cook dinner, I let them help, exposing them to as much language as possible. Also, I may ask them retell the list of ingredients in a dish to Dad during a meal, giving them a chance to verbalize and improve their memory skills. So try to include the children in your daily activities, they might become better listeners!

Written by Val, mom to Gage and Brook

with special thanks to Dr. Robin and Natalie Baldwin for their contributions
http://www.deafkidscanhear.blogspot.com/

Cochlear Kids

Meet brother and sister team Gage and Brooklyn Blakely. Gage is now eight years old but was born deaf. He has Goldenhar Syndrome and wore hearing aids for over three years until he received his first cochlear implant. At age seven he became bilateral and will be in third grade this Fall at his mainstream public school. His sister Brook was born hearing but began her progressive hearing loss as an infant. She too wore hearing aids until she was almost three when she received her first cochlear implant and a year later became bilateral. She will be in Kindergarten this Fall at the same school her brother already attends. Both have limited support services at school. These two are very active and love being outside. Gage is all about trucks, he even makes and customizes his own toy vehicles. Although he's rarely caught sitting still having ADHD, he can sit for the longest if he just has some type of truck to work on or a big box of Legos. Brooklyn just wants to be a mom and takes very good care of her two favorite baby dolls. She can be found pushing them in grocery carts or in a baby stroller everywhere we go. She's even been spotted feeding them in a local restaurant as any good mother would do. Both used Auditory Verbal Therapy to learn to hear and speak. We are thankful to live in an area where we have access to great audiological and therapy services. We have always had a large support team to guide us through the difficult process from finding out our children were deaf to treatment for their hearing loss. We thank our doctors, therapists, family and friends for lending their much needed support over the last few years.

You can keep up with the Cochlear Kids at our family blog http://www.deafkidscanhear.blogspot.com/

Written by mom Val Blakely