Waiting for Answers the left ear

Do you ever feel like you are in limbo? Stuck just on the crest of some BIG discovery. A life changing discovery! We are there. Rather I am there. Waiting. Braydon has the BAHA Attract in the left ear allowing him to hear but the mucousalized tissue causes him constant pain. We have tried it all. The silver nitrate, the creams, the powders and prayers. None of it seems to help in the middle of the night when he lies next to me crying that it hurts so much. 

I have pleaded with my doctor to please tell me about that one magic card I have been convinced is hiding in his pocket. He kept telling me he was asking plastic surgeons and other specialists for thoughts. All to no avail. I had to take matters into my own hands. I just knew there was an answer other than his suggestion out there.

Why? Because my son refused his suggestion and I am his advocate. What suggestion? The one where they perform yet another rare surgery to completely obliterate the left ear canal. What does that entail? Basically the doctor removes the bad tissue then folds down the ligaments "and stuff" to fill the canal sealing it at the end (where we would usually see an opening) with cartilage. 

Pros: 90% chance of success

Cons: 10% chance of failure, my son is adamant that he doesn't want it. He doesn't want anything else that makes him look different. 

Well, what is any self-respecting advocate to do? Drive to the next state over for another opinion (of course). It was my honest belief (however ignorant or delusional) that by going 1 1/2 hours East we would somehow encounter a doctor who knows all about this "other" procedure. You know the one my son says yes to & has a 100% success rate! Ah yes you know the one!

Believe it or not this doctor suggested what our surgeon was already telling us. I think it was the most devastating day we have had in quite some time. After the appointment I couldn't even discuss it. I seriously believed there was going to be an answer there. My husband was not even a bit surprised. Clearly we live in 2 very different realities. 

While talking with our surgeon the other day my son had a moment where he hid his face and refused to discuss the topic further. The doctor agreed to give us some "take home" powder to try to keep the ear under control for now. 

We will continue to wait. You probably wonder what I am waiting for. That makes 2 of us! Doc keeps saying "it doesn't exist yet" so I suppose I am just waiting for "it" to exist or my son to change his mind about the obliteration. I know you probably wonder why I let him(Braydon) decide. 

Well..It is his body after-all. This is his journey. These choices we make affect him for the rest of his life. If I don't let him be in control.of his body (within reason) now then when does he get control? Breaking it down a bit simpler, a toddler says no to a hug to a family member. They should have that right. It is their body that is being touched, after all. So, until medically necessary we will wait, on the crest of something new to fix this issue.

BAHA Attract System - A new way to hear

When Braydon was 4 I remember the moment I learned that he would never have normal hearing again. I just sat there staring blankly at the surgeon who had humbly sat down next to me. He told me and my husband that the cholesteatoma had spread out like a spider web. When it did this it had eroded 2 of the 3 hearing bones (ossicles) necessary for hearing. This was the good ear. All I could think was ... but this is the good ear! What hope did that leave for the 'bad' ear?

As I was in free-fall from the news I became determined to learn all I could to help my son hear. I watched an informative video about the BAHA. This is a Bone Anchored Hearing Aid. The great thing was the lady on the video talked about the hope. The worst thing I ever did was watch that video. The video was in the OR and actually showed the process necessary for implantation. This, I assure you, is something no parent should ever watch. I vowed that day that we would find another way.

We tried hearing aids. The purpose of the hearing aid is to amplify the sound to the bones. The problem is that my son only had 1 of the 3 necessary bones in both ears. The molds also left his diseased ears infected. While this is not a common issue for children with hearing loss, this was our experience because of chronic ear disease.

We finally began a new look at the BAHA system. A Bone Anchored Hearing Aid is just that a hearing aid that is anchored to the skull. This device is helpful for kids with a conductive hearing loss. The processor transmits the sound waves through the bone that then sends them to the healthy cochlea. From there the sound waves travel along the normal path to the brain. At this time 2011 our only option was the traditional implant. There would be an anchor implanted 4 millimeters into his skull with an abutment that would penetrate the skin. The BAHA processor would then clip onto the abutment. Braydon was 8 and assured me that this was not an option for him.

Defeated but determined we started looking again. I kept telling everyone who was 'in the know' that Cochlear had to be coming out with magnetic technology. They had the magnet in the Cochlear implant and they had the bone anchored portion in the BAHA. Audiologist's and surgeons alike were telling me it was coming. I was so excited when beta testing began in 2013. I checked all the findings over and over. Everything was coming out beautifully. In anticipation of the FDA approval I began the petitioning process with my insurance company in August 2013. Braydon finally received his BAHA's in January 2014. Until he could get the implant he wore them on a headband.

I have to say that while I was frustrated at the amount of time it took to get my son the hearing devices necessary for him, the wait was worth it. We ended up getting the newest version. This version offers Bluetooth capabilities. Many wonder what that means for a 10 year old. It means that he can hear the phone conversations with his friends and family. It means he can stream music from his Ipod. It also means that he can stream his FM system in class!

Everyone is wondering how I went from 'That will never be us' to excitement. I think the answer lies somewhere in my ability to finally accept my son's true condition. Yes it clearly took me years. However, I think that every parent of a hearing impaired child likes to believe that a miracle will happen and hearing will magically be restored. Unfortunately, that was not our families reality.

I am proud to announce that in February of 2014 Braydon became the first person in Indiana to be implanted with the new magnetic version of the BAHA. This version is called the BAHA Attract System. There are approximately 100 people in the USA that have this implant. I was so nervous leading up to the surgery but my 10 year old was brave and he assured me that we were making the best decision for him.

I believe that it is important to bring our children in on decision making when possible. They are the ones that have to wear the devices, endure and recover from the surgeries and ultimately live with the decisions made. Braydon knew from day one that the possible skin overgrowth and infections was not something he was willing to deal with from the traditional BAHA abutment. However, the magnet was something that excited him. It took away his concerns.

The surgery went splendidly. The predicted amount of time was spot on. The after care was very well described by our surgeon. A C shaped incision was made behind Braydon's ear. The C faces away from his face. An anchor was implanted 4 millimeters into the skull. This takes approximately 30 days to fuse with the skill. This gives the implant added strength. Then a magnet was attached to the anchor and the incision was closed.

The first week was a bit rough. The swelling was painful and controlled with hydrocodone. The bruising was quite minimal. Around the 2nd week Braydon was feeling quite a bit better. He was weaning off the pain medication and feeling much better. Our follow up appointment was great. He is scheduled to have his implant activated on March 26, 2014. They allow 30 days for everything to heal completely.

I look forward to updating everyone after activation on March 26th. We have booked a Disney vacation to celebrate the freedom of hearing right after activation. Please feel free to contact me with any questions or concerns. If this is something that you feel will benefit your child I would recommend contacting my son's surgeon at Riley Children's Hospital. Dr. Charles Yates. He is currently the only doctor that I am aware of performing the surgery.

Many Blessings,
Braydon's Mom

 

 Braydon 1 week post op

Braydon 3 weeks post op

Feb 2014 updates

Braydon had to go to the emergency room recently. A lump formed behind his left ear. It seemed to us it might be yet another mastoid infection. Most people are unfamiliar with this type of infection so I wanted to take a moment to explain about it.
Signs:
Swollen/red area behind ear
Hot area behind ear
Pain in jaw
Dizziness

What is it?
From my understanding (as a mom) the initial cause is a middle ear infection. The middle ear and the mastoid cavity are connected. The mastoid cavity is an air filled cavity. The bone that surrounds this cavity looks like a sponge with deep contours. The are is not a sponge and non-absorbent. The infection overflows into this air filled area and creates a mastoid infection.
In Braydon's case (4 times) it required hospitalization, surgery to drain the infection and 1 week of IV antibiotics. After the 4th infection we had the area 'drilled out' basically the surgeon smoothed out the deep contours. Since then we have not had another infection. This had to be done on Braydon bi-laterally.

If you suspect this type of infection please seek medical treatment immediately!

Coming up!
On February 25th Braydon will be the first child at Riley Children's Hospital to receive the BAHA Attract System implant. This is very exciting to us. We chose this option vs the traditional BAHA abutment because there is no skin penetration. The magnet is about the size of a quarter and is drilled 4 millimeters into the skull. This product is different from the Sophono because it will actually fuse to the bone. Approximately 3 months later he will have the other side implanted. If you have questions regarding this new technology please let me know. I am excited to answer to the best of my ability.
Warmest regards,
Braydon's mom

The beginning... cholesteatoma

Common questions I am asked 'was he born with it?", "was there something wrong in your pregnancy?". To answer this we must start at the beginning. I was 26 when I was pregnant with Braydon. He did attempt to come into the world a bit early (30 weeks) but I was prescribed tributaline and bedrest (strict bedrest). He made it to 37 weeks.

From the start he was a screaming baby. Hard to nurse. Did not enjoy contact or life for that matter. I finally got him nursing and learned that if I wore him in a baby carrier on my chest with him forward facing and straight up he would finally stop screaming. He hated car rides, the swing, laying down, you get the jist.

His first eardrum burst at 3 months old. By 12 months old he was only saying dada and throwing horrible temper tantrums. His walking was not on schedule. We saw our first ENT (Ear Nose & Throat doctor). This resulted in a referral to a speach therapist and an occupational therapist. He had 7 sets of tubes in the next 2 years and his adnoids removed. He did begin walking and some talking but clearly things were not right.

By the age of 3 his eardrums were just gone. He had large amounts of green and yellow goo draining down from his ears. We asked for another opinion. We were directed to Riley Children's Hospital in Indianapolis, IN. where we met Dr. Micheal Fritsch.

Our first meeting with Dr. Fritsch he announced that Braydon had pollups (begnine bumps) in his ear canal. He did surgery and removed them. Within 4 months the snotty drainage was back. His poor ears were raw from the constant drainage.

At age 4 Dr. Fritsch went into surgery to remove those pesky pollups again and found that each ear (bi-lateral meaning both sides) had a cholesteatoma. This was upsetting for everyone. He scheduled surgery for the 1st removal the next week.

A cholesteatoma is usually caused when the eardrum sucks into the middle ear and then bursts. The tissue (skin) develops an enzyme that can eat through bone. The normal ear has 3 bones (ossicles) that are used for hearing. The eardrum transmits the sound to the ossicles which vibrate and send the sound on. In Braydon's case the cholesteatoma wrapped around 2 of the 3 bones in both ears.

I must tell you that having a cholesteatoma is very rare. Having bi-lateral cholesteatoma's is unbelievable. To remove Braydon's cholesteatoma an incision was made behind the ear. The ear was pulled forward exposing the middle ear. The cholesteatoma and damaged bones and tissue were removed. The ear was sewn back in place.

An attempt was made in the left ear to replicate the ossicle chain. This worked for a small time which allowed the doctor to remove the cholesteatoma from the right ear. Since each removal rendered him nearly deaf in each ear the goal was to restore hearing in one ear before moving onto the next ear. Of course the entire time the cholesteatoma was left in, it continued its path of destruction.

At the age of 6 in May 2006 a new nightmare began. It started in the form of a fever and an earache. I thought nothing of it. Called the pediatrician asking for an antibiotic (it was the Friday before the Memorial Day weekend). My request was denied. I resigned and took Braydon to the Riley Children's Hospital Emergency room. My thought was we would be done in 2 hours with our antibiotic.

Unfortunately, that was not our situation. Braydon had what is called a mastoid infection. Your mastoid is the bone behind your ear that has little to no hair on it. Braydon was given 2 cat-scan's and admitted. He was started on 1 IV antibiotic. After 12 hours his fever was at 104.8 and a 2nd antibiotic was added. I noticed his lymph nodes on his neck were the size of golf balls. I had one very sick child.

He was rushed into surgery where an incision was made into his ear drum and the infection was suctioned out. We stayed in the hospital another 4 days until his fever finally broke. Turns out we would repeat that infection hospital stay and surgical intervention within 2 months. At that our surgeon said we had no choice but to have a 'mastoidectomy'. What this means is the surgeon made the incision behind the ear again. He then shaved the inside portion of that bone down to smooth to eliminate the possibility of another infection.

Wouldn't you guess that within a very short time the other ear began having these exact same infections. Each time we were in the hospital for about a week, IV antibiotics and emergency surgeries to reduce infection.
Ultimately, a mastoidectomy was needed on the left side as well.
So, by 2nd grade Braydon had each ear removed 2 times. He had a cholesteatoma removed from each ear and a complete mastoidectomy bi-laterally.

Right now you are asking "what are the signs?". The first sign is a high fever, then pain behind the ear in the mastoid area, it may be hot to the touch, it will become swollen, it may even turn red, swollen lymph nodes. If you have swelling in the mastoid area for any reason please rush to the ER. This can be a very fast spreading infection.

2012 started the next part of Braydon's journey.. fungus. The problem started out with me noticing that Braydon's ear drum had a hole. Then while watching the hole over a period of about 2 months the hole was getting noticeably larger. Before long the entire ear drum was GONE! Then it started. The black fungus. The never ending pain.

At this point we began seeing Dr. Charles Yates as our new ENT. He is the top specialist at Riley Children's Hospital. He is very kind, patient and intelligent. I can only explain what an intelligent doctor he is by continuing with Braydon's journey.

The pain behind his ear was so intrusive and intense he began seeing a neurologist and a psychologist at the Riley Pain Clinic. They determined that the repeated need for cuts behind Braydon's ears had caused nerve damage. This nerve damage causes Braydon to have constant pain. We are slowly learning things that make the pain worse but yet to find anything to relieve the pain.

Back to the fungus. We tried surgically removing it. It grew back. We tried creams and concoctions only to have it grow back. Finally, insurance approved us to go to Cincinnatic Children's Hospital. Dr. Arjmand had a new idea. A skin graft would be taken and placed where the bad tissue was.

Back in Indiana we went for surgery in September of 2013. In Braydon's left ear the mucosalized tissue was removed. It went all the way to the bone.

What is mucosalized tissue? Well besides most likely being misspelled it is wet tissue. In our normal ear the skin is dry like the skin on your arm. Behind the ear drum it is wet like in your nose or mouth. It is a mucosa tissue. When Braydon's ear had no eardrum for over a year there was no boundary for his skin to separate the middle ear from the ear canal.
In a 5 hour surgery the mucosalized tissue was removed and a graft was taken from his leg.

After this surgery his air sacks were not absorbing the oxygen. He was not panting or gasping. Everytime he fell asleep his oxygen level would drop dramatically. It was very scary. We stayed in the hospital for 48 hours. A respiratory therapist worked with him to get he air sacks to open back up. Finally, his lungs were working! We went home to heal. We have seen the surgeon every 2 weeks since the surgery in September and it appears as though we have finally rid ourselves of the fungus!

Braydon has a condition where he creates a fluid in his middle ear that is thicker than what a normal person creates. In my ear and your ear the fluid drains down our estuation tube. His tubes were ruined by the cholesteatoma. So his fluid has no where to go. That is why his ear drums burst. The simple answer is put a tube in the drum. However, because his mucous is so thick the tubes only last about 6 months and he still suffers from frequent infections.

If your child suffers from this problem we have found that (doctor approved and recommended) 1/2 water and 1/2 white vinegar is a great natural fix. You want to ensure that the mixture is room temperature or your child might actually throw up. The temperature change actually triggers vomiting. You can use this twice a day when the ear is clogged or when the drainage from an active cholesteatoma is bad. We have reached a point where we use this as maintenance. It keeps the mucous thin and helps us to prevent infections. We are still having problems keeping tubes where they belong.
We have tried regular tubes, butterfly tubes and T-tubes. Currently, we are using a T-tube in the right ear.

Back to the left ear. When the skin graft was constructed a new ear drum was also constructed from tissue under Braydon's scalp. A silicone filled ball was placed in the middle ear space to effectively create a middle ear space and to hopefully prevent the new eardrum from sucking back into the middle ear and creating yet another cholesteatoma. So far.. it has worked!

We currently are working on hearing reconstruction. Here is what we have tried so far. First, Braydon had an ossicular chain reconstruction. "A what" you say. This is a combination of titanium and cadaver bone constructed to mimic the ossicles. This is actually a great fix. However, due to Braydon's thick mucous this did not last long.

We have tried hearing aids. This seems to trap moisture in the ear canal and create.. you guessed it..fungus.

Now what? When the bones are gone you have what is called a conductive hearing loss. There is a great device known as a BAHA (Bone Anchored Hearing Aid) that will fix this. The older version had what is called an abutment that is implanted into the skull and sticks through the skin, behind the ear, and the BAHA device is attached to that. The BAHA device is the processor and translates the sound. The abutment just conducts it through the bone. Essentially fixing the problem. You can get the BAHA and not have an implant (if the abutment is not for you) but wear it on a special headband.

This has been Braydon's preferred method of several months now. We have been waiting for Cochlear to come forward with a magnet that would allow the BAHA to magnetically stick to the head and conduct sound. There is another company (Sophono) who offers this however we have personally decided that they were not the best company/product for our son. Our son chose not to have an abutment so he wears the band. It is my personal belief that if it is not a life saving surgery I will allow Braydon to have input. This is his life, after all.

Today, Braydon is 10 years old. He attends mainstream 4th grade school in Indiana. He has had 31 ear surgeries to date.

We have just received word that the FDA has approved Cochlea's magnetic implant for the BAHA. Braydon's 32nd ear surgery is scheduled for February 25. During this surgery we have a lot going on. The right ear will have the current tube removed, a patch placed over it's current location and a new tube inserted in another place in the eardrum.

In the left ear, the silicone ball will be removed. If there is enough room possible the surgeon would like to try another titanium implant. While I don't personally have a lot of hope in that process I respect what the doctor hopes to accomplish.

Now for the exciting part... Braydon will be the first child to be implanted at Riley Children's hospital with the magnetic BAHA option. It will take 30 days before he can 'go live'. That means attach his BAHA to his new magnet implant. 90 days after the first magnet is implanted Braydon will be eligible for his implant on the other side.

We have been seeing Dr. Charles Yates since 2011. He will be performing the upcoming surgery in February. We are equally excited to see how things turn out!

So now you know the beginning of Braydon's journey. Stay tuned and I will be posting ideas that our family has been implementing to help make your hearing impaired child's life a little easier.

Quick example: Your child finds Closed Captioning to hard to follow/read. We bought a karaoke game and the words scroll across the screen just like CC. Guess whose son learned to watch TV with CC in less than a week? This is something that your school is obligated to provide CC for any shows watched in class.

Companies that I recommend for help:
Hands and Voices
Guide By Your Side
Hear Indiana
Alexander Graham Bell Association

I can't wait to hear your feedback, questions and suggestions.
Sincerely,
Braydon's Mom