Wednesday, January 27, 2010

Final Days with John Tracy

We also learned about a hearing device called a BAHA. It is a bone conductor hearing aid for children like August. It is like a hammer hammering on the skull so the bone can transport the sound to the cochlear (normally the canal and eardrum do this). It is a very small box which is placed on a band like a headband. We wanted to get one for August so he could get his cochlear (inner ear) exercised and used. It just made sense to use the cochlear since it is functioning. I didnt want his brain to forget that it was there. However, our insurance would only pay for some of it so we didnt want to purchase one unless it made sense.
Was August going to want to use it? He had become a very independent little man and determined to get is way. He knew what he had. He understood that his ear wasnt the same as the other one. But still would he wear this BAHA. JTC allowed us to borrow one for six months so we could make a choice to purchase one or not.
We got it the day after his second birthday. I was so excited! This meant that August would be able to hear with both ears while using this hearing aid. It was so perfect.
Well, not so much for August. He was not so excited. We tried every tactic we could think of the get him to use it. But he wouldnt have it. I am not sure if it was because he had this headband around his head or maybe because sounds were clear and it was strange for him. He would hide his Atresia ear with his hand as if he didnt want to have the sound. It was a battle. But when he would use it, it was so amazing. He would hear things that he couldn't usually hear, things far away etc. It was great to see his response to sounds.
It was important to give the BAHA a try. Had August had the opportunity to try it as an infant, he would have been okay with it as he grew older.
After about four months of trying we gave the BAHA back to JTC.
While struggling to get August to use the BAHA, his good ear was progressing. His hearing tests just kept getting better and better. To us he was hearing like any normal child with two ears. Not to mention he was maturing like any other child as well.
A love for baseball, golf, race cars, trains, Elmo, Nemo, etc. He amazed so many people with all his passions. The ear was not slowing him down, not one bit!
Since August was finally doing so well with his speech and development and he kept showing no signs of being behind in any way, we stopped our visits at JTC. It was best to let another child, that needed JTC, be in our place. So we discontinued out visits when we gave the BAHA back.

Sunday, January 17, 2010

Our journey continues

On June 2, 2008 we brought our family to live in America. August was now sixteen months old. It was time for some real hard core studies of atresia microtia.
I started calling any affiliation to the deaf and hard of hearing. At this point, I was sure that August only had hearing in the left ear and there was only hope of ear reconstruction to the right ear. We did not know any other family with children that had atresia microtia. We had no mentors or guides to help us get more answers so I started just dialing and Googling everything possible.
At some point in July, I was given a group to contact called GLAD, the Greater Los Angeles for the Deaf. I spoke to a gentleman who finally understood that I was a mother looking for answers for my son.
I had called numbers of ENT's and the staff had no idea what atresia microtia even was. I didn't want to take my son to a doctors office that didn't understand what we were there for.
So you must understand how happy I was when I finally had someone that knew what atresia microtia was and that I needed answers. He soon had me connected to other families with children whom are deaf and/or hard of hearing. I was invited to private gatherings with these families to get familiar with others in the same boat. I had a phone conversation with a few and met with others.
But it wasn't until I met two beautiful ladies in Venice Beach one summer afternoon. August and I spent a few hours walking and talking with Jessica and Maddy. It was a turning point in our lives. Beautiful little Maddy was also born with atresia microtia and her mother was searching for answers as well.
As we comforted one another of our little gifts from God, we shared everything that we knew about atresia microtia. Jessica told me about the John Tracy Clinic and House Ear Institute. I shared with her about Early Start and programs that were available to help with early intervention.
But for me the turning point in my life was getting a call back from John Tracy Clinic telling me that they would give August an audiogram right away. They gave us a consultation and many connections to institutions to help us make the right decision for August.
At nineteen months old, we still had no idea if August had a working cochlea or not in the right ear. We still considered him to be deaf in that ear. So you could only imagine the flow of tears from my eyes when I witnessed my son for the first time respond to a hearing test on the atresia ear. Thank you John Tracy clinic!
The test showed that August's inner ear, the cochlea, was fully functioning. The issue was that he could not get sound through all the bone that was in the way of what should be his canal.
He could hear! He could hear! He actually could hear in that ear without a canal but things had to be extremely loud to get through the bones. It was the best day since August was born so far.
We were set up to meet with a speech therapist at John Tracy Clinic to help him get caught up. I was asked to attend meetings as well at JTC to meet with other families like ours and learn more about the deaf and hard of hearing.
What JTC did for our family is priceless. I was able to get all my tears and fears out and get more comfortable with what was to come for our August. They finally gave us the answer we had been waiting for on August's hearing ability.
For almost a year I stayed involved with JTC. August progressed in speech with the help of Mary at JTC. Everyone at JTC helped me understand and be comfortable with what we were dealing with in having a child with atresia microtia. They even gave us a BAHA (Bone Anchored Hearing Aid) to borrow for August. Which was something I thought was important to try before we spent tons of money on for nothing. Thank you JTC for your help, love, concern and support. Thanks to all the families I met at JTC. You too helped me heal and understand.

In our journey to reconstruct August's ear

We learned that the ears are developed in the first trimester of pregnancy along with the kidneys. So it was important to rule out any issues with August's kidney's as well. He had an ultrasound at UCLA during our visit to the US in 2007.
We also had x-rays of this spine and neck. To make sure that his bones were strong enough to hold his head. In some cases the neck vertebrae and skull/jaw are mis-formed as well.
He checked out perfect on both. Thank God!
We learned of the test called ABR which can determine if the cochlea in the Atresia ear worked or not. (The inner ear or snail part of the ear.)
We were so excited to learn that there was a test that could determine if the deformed ear was a hearing ear or not. It was a relief to know that he might actually be able to hear in that ear. The hard part was to wait until we were back in America to have more answers.
We continued having August's check up on this normal ear every six months. He continued to show a perfectly good hearing left ear. He was growing and maturing like a normal little boy. We were so pleased with his progress.
Just two weeks shy of his first birthday, August took his first steps. He showed no sign of being off balance.
He showed a love for balls as early as 9 months old. He would grab our fingers and want us to walk him around as he kicked the ball all over our small flat. Today he still has a scar above his left eye from trying to stand on a ball and hold himself up by our coffee table, he was only 10 months old. He hadn't even taken his first steps alone yet. A determined little guy.
He took a love for drums at an early age as well. This was a surprise to most since his sound of hearing was distorted from only having one good hearing ear. When he saw a drum set, his arms and legs went crazy. He barely sat up on his own but he wanted to hit the drums and listen to someone play them.
There was just one thing that kept me worried as August grew older. His speech was not coming in as most children's did at his age. As time went on we just kept speaking to him as if he had normal hearing in hopes that he would just start talking like normal.
In some cases I was feeling like I was just waiting for time to pass until we got back to my country to get the answers we needed. There was still so much to learn.

Research continues

When we returned home to Denmark, I continued to look for answers. We learned from the cranial facial team, August could have ear repair surgery using tissue from his ribs. However, it could not be done until he was 8 or older. Some even suggested to wait until he was approximately 12-13. The surgery would require the surgeon to take cartilage from his ribs to form an ear.
We did not learn too much about the atresia repair. So I wanted to be more informed on that procedure. I also wanted options were the microtia was concerned. Using cartilage was not so exciting to me.
I did a Google search on atresia microtia and found out so much information and that there was help for our son.
Unfortunately, the Danish doctors, told us that they did not have the technology to do the tests that we were told about. It was just best to wait until he was old enough to make decisions on his own, around 18 years of age.
In my research, I found that children with unilateral (one-sided) hearing loss are usually held back in school and suffer in school due to the inability to hear everything the teacher is saying. Especially if he did not get preferred seating to have his good ear to the teacher when at a chalk board, etc.
I also found that many children with atresia microtia can suffer from lesser self confidence and become introverted while in school. Their self esteem is lost due to trying to hide the ear and/or other children giving them a hard time about their appearance, etc.
So I knew that this was not something we could wait on to be fixed until age 18. All his school years would be over and I was not going to take the chance of his studies and self confidence being altered because of his ear.
More things I found out was;
- This is more common in boys than girls
- Averages occurrence is 1 in 6,000 when averaged across all ethnic groups
- 60% of these cases are of the right side and unilateral only (one side not both)
- Atresia can be a symptom of a larger syndrome, such as Treacher Collins,
Crouzon's, Alpert's, Preiffer, Klippel-Feil, BOR (Branchio-Oto-Renal), 18-q
chromosome as well as Hemifacial Microsomia.
- The rib graft surgery would be at least 2-4 surgeries before he would be free of
surgery for a while. (this does not include the microtia repair)
- The costs involved for both surgeries would be approximately $65,000.00
We were lucky that we were only dealing with atresia microtia and not any other symptom. However, the more I learned about the rib graft surgery, I was wanting to find another option.
I was not pleased about putting him through 2-4 surgeries just to reconstruct his ear, knowing he would still need another major surgery on the atresia repair. So I continued my research.

Saturday, January 16, 2010

The search for answers

August was born in Denmark, his daddy's native country. With me being American and not understanding enough of the Danish language, it was extremely difficult to understand what the doctors were telling us most of the time. My husband would translate as much as possible but with all the medical terms that were used it was not so easy to translate.
We were set up with a Danish ENT doctor whom explained as best he could in English. We did learn however, that the deformity was called Atresia Microtia. Atresia means, malformation of the external ear and most of the time the middle ear as well. Microtia, is Latin for small ear.
But again we were told that there was nothing we could do at this time. He was too little to have a hearing test to that ear and it was a bad idea to do anything until he was at least eighteen years old. He was to have a normal life growing up with just one ear. I should not be worried a bit.
I was still not pleased with that answer. I was determined, as his mother, to know as much information as I could about what we were dealing with. He was not going to be lost in the system and not get every opportunity he deserved.
So I continued my research on websites. I spoke to ENT's in the US and finally got in touch with a cranial facial team at UCLA.
On our visit to America, when August was just three months,UCLA was gracious enough to squeeze us in with short notice. August was be evaluated by the entire facial team. The panel of doctors explained in detail surgery options and hearing tests.
We met with oral surgeons, cosmetic surgeons, atresia repair surgeons, etc. We were told August could be a candidate for repairing the outer ear at the age of eight. He could also have atresia repair but he would have to wait to find out until he was about two and a half. He could undergo a CT scan that would determine the likelihood of him being able to hear or not, if the bones and facial nerve would or would not interfere with a canal surgery and if the middle bones were intact.
Since we were on a short visit of only four weeks to America, we had no time to run a hearing test in the microtia ear. So we would have to wait until our return to America on a later date. A hearing test would help us understand the depth of hearing August already had even without the canal.

August early days of life

When a child is born as parents you expect a perfect little bundle. The bundle should have two hands, two legs with two feet, all five fingers and toes, two functioning eyes and ears, etc, etc. You are never prepared for any of these things to be wrong or missing.
When our little August was born we were expecting the same as all parents. He was born pre-mature but a healthy pre-mature weighing in at 5.6 pounds. He was ready to conquer the world at 34.4 weeks in the oven. He spent about three days in ICU and another two weeks in the hospital with me, his mommy. We were ready to move on and go home and get started with our new lives as a family.
But I will never forget the words that came out of my husbands mouth as the midwife placed him on my belly with his right side facing up. "Wow, he has a very small ear!" My reply was, "is there something wrong!" I couldn't see him in the same angle as my husband. I could only feel and it seemed not right to me.
While my husband and the doctor took our new baby boy to be taken away to be cleaned up and get warm, I was left alone to wonder if everything was okay.
After what felt like hours, which was truly about one hour, my husband came to take me to see our little guy. But before we left, he said, "he is so perfect and beautiful but there is something wrong." I felt my stomach turn as he said the words. He advised me that his right ear never formed on the outside. He did not know if he could hear or not from that side or what it meant. He just didn't have an ear on the right side. I didn't know what to think or feel. It just didn't make sense. I was sure that it was just something simple and we could fix it for him right away.
When I was finally joined with little August, he brought tears of joy to my eyes. He was the best thing I had ever laid eyes on. He was so beautiful just as my husband had said. But yes, he was missing his right ear and it too brought me to tears. I wanted answers now! Did I cause this to happen, was I already a bad mother and he hadn't even been in this world more than an hour or so. I was so devastated.
I can remember thinking, how he would get teased at school, will he be able to walk with the right balance, will he get his speech when he is supposed to, tons of things came to mind. So much worry! I just wanted answers!
We had a tough start with August, aside from his ear. He had pyloric stenosis and had surgery at three weeks old. He then developed a bad case of acid reflux until he was nine months old. We never slept. He couldn't sleep because lying down made him vomit and he would choke. It was very exhausting for all of us for about the first 4-5 months.
With all this going on it was hard to find time to research the small ear that August had. We were told that he did not have hearing in the ear while at the hospital for the two weeks after birth. We were also told that he did have perfect hearing in the left ear. With that, we were not to worry since he could at least hear from one ear.
I was not satisfied with that answer. I was determined to find out what this small ear was all about.