Time for an update.

UPDATE: I felt the need to come back and update this post as we have learned that Audrena's Usher Syndrome diagnosis was a mistake. Here is the post where we learned she does not have Usher Syndrome.

It has been awhile, so I thought it would be a good time to update! We have just been so busy! Audrena is really taking to being bilateral. She knows when the headpiece falls off, and she either puts it back on by herself, or she comes to us and pats her ear to let us know. We do have the alert beep as well, so we usually know before she reaches us. However, we are trying to teach her to be independent with her implants.

We did have some question as to whether she was getting much from the new implant yet since we're still on the first set of programs. Daddy took Audrena to her programming appointment yesterday, and she repeated "bird" to him after he had said it. That was with just the new implant turned on. So it's good to know things are at least starting to sound ok to her. Sometimes it does take awhile for the brain to adapt to the new implant.

They took her into the sound booth, and of course she was just not into it. Audrena rarely cooperates in the booth. She would rather be exploring. Even with that, she did cooperate just enough for them to determine that she is at least at 35-40 decibels with just the new implant on her first sort of generic MAPP, 30-40 with the first implant, and 30-40 with both. Dr. M. from USD has always said (and others have told us as well) that 30-40 decibels is sort of the best expectation for cochlear implant recipients. We do know it's possible to do better. We know plenty of implant patients who hear at 15-25 decibels, and that is our goal for Audrena once she gets old enough to give the Audiologist some verbal information about how she is hearing during programming. Also, we have gotten booth tests with her first implant that showed 20-30. That, combined with what we see at home, are enough for us to believe that she is hearing better than yesterday's test suggests. Both of our Audiologists (Boys Town and USD) have said she could very well be hearing those softer sounds, but she is just not paying enough attention to them to be motivated to turn away from her toy when she hears them.

Also, I forgot to include in some of my previous posts that Audrena heard birds (this was before the second implant)! She heard them before she saw them! We were at the police department's Bike Rodeo at the high school, and there were finches in the rafters of the lunch room. We were eating, and suddenly Audrena looked up (way up) at the birds, pointed, and said, "Tweet tweet!" She had to have heard them because she had not been looking up at all. She was eating. When we first found out that Audrena was deaf, I would take her for a walk, listen to the birds, and wallow in self-pity that my baby girl would never hear those beautiful outdoor sounds like birds chirping. Birds are generally about 15 decibels on the audiogram (although the chart below shows them even softer), so I never expected that she would hear them with implants either. That moment in the high school lunch room was a really defining moment for me, one of those WOW moments that just takes my breath away whenever I think back on it.

Here is an audiogram chart so you can see what I am talking about. The yellow area is the speech banana, or the range that speech sounds fall into.

From firstyears.org

Another little hiccup to report...we may be headed back to the operating room. Yesterday, Dr. M. couldn't find the tube in Audrena's left ear. That's the one Dr. L. inserted in March. Granted, her tools may not magnify quite as much as an ENT's tools, and those tubes are tiny. But she is good, so I trust that if it was there she would have found it. We have an ENT appointment tomorrow morning to check it out. If it's not there, then Dr. L. will need to insert another one. It's such a minor procedure, but it would be the 6th ear surgery and the 7th time under anesthesia for Audrena. And there is always the possibility that she will need a tube in her right ear in the future. So if you are the praying type, say a little prayer that the tube is in place tomorrow, although I don't have much hope that it is. Next week is also our appointment at University of Iowa with the Pediatric Ophthalmologist who will monitor her Usher Syndrome and the doctor with the genetics laboratory that conducted the genetic testing. So please keep Audrena in your prayers that everything turns out well there, too!

On a more positive note, Audrena is really on a role with her speech and language, and she is making progress with her balance! She is saying new words all the time and starting to put them together. She is beginning to understand that there are different words for the same thing, and starting to use them interchangeably. For example, "woof woof," "puppy," and "dog." She also says, "Look at that," or "Look at those." Here are a few of her favorite words/phrases lately.

Look at that/those!
What's that?
Where go? (Where Daddy go?)
Get down!
Good job!/Very good!
NO!
Stop!
Mine!
And when you call her name, she answers with, "What?"
She has also learned "Kelton, "Ana" (for Reyana), and "Zach" (my nephew).

Although we always question whether we could be doing more for her, or whether there is something we are missing, we are so pleased with Audrena's progress!

Another Surgery Down. One More to Go!

Audrena had surgery last Thursday. It was a long night in the hotel prior to surgery, as Audrena kept waking up for a drink. She cried, "More drink," repeatedly for at least an hour. It broke our hearts not to be able to give her anything. Then, after she calmed down, she tossed and turned all night, finally settling into a good sleep at 4:00 a.m. Our alarm went off at 4:30, so it was a short night for Mommy & Daddy.

We arrived at Boys Town at 6:00 a.m. for surgery. She had a deep pocket recession in her ear drum that needed to be fixed, and she needed a tube. Dr. L. did both, as well as a cartilage graft behind where the pocket had been to help support the ear drum. He was able to save her from the radiation exposure of another CT scan by doing a visual inspection to make sure the cholesteatoma had not returned. We received a wonderful update from the OR nurse...NO cholesteatoma! I hadn't quite realized how stressed I was about the possibility of another delay until we heard those words. It was like a huge weight lifted from our shoulders.

After surgery, Dr. L. came into the room with a handful of photos. He showed us exactly what he had done from start to finish. It had all been accomplished through the ear canal, so she did not have an external incision. He said we should be able to proceed with the second implant in 6-8 weeks! Music to our ears!

They brought Audrena to us about 45 minutes later. She was madder than a hornet at the iv and kept holding her arm up to the nurse like, "Take it off!" After we got her comfortable, she fell asleep. It was a long afternoon as we took turns holding her while she caught up on her rest after a very sleepless night. By the time we could consider going home, we were both too exhausted to make the drive. Instead, we booked a hotel room. Thank goodness Grandma & Grandpa planned to keep Reyana and Kelton another night!

Audrena finally woke up, starving and thirsty! We gave her some crackers and apple juice, which she kept down. Then it was time to be released. Surgery number four down, and hopefully just one more to go!

Here are a few photos.

Before surgery. She put her coat on and tried to escape!

The play room is wonderful for keeping kids occupied before surgery!

Relaxing with a snack and a drink at the hotel. She was feeling pretty good, and she felt even better after a bath!

Feeling Overwhelmed Today

I just talked with Dr. L. awhile ago. He was calling me back to answer some questions we had. Since he was at a conference and then trying to get caught up, it took awhile for him to get back to us.

First we discussed Audrena's risk factors for meningitis and the risk of facial nerve paralysis with the surgery. I'm relieved to hear that it is as we thought--those are not things that should keep us from going forward with the implants. The risk is still relatively small, especially because we do vaccinate on schedule. He has done at least 800 cochlear implant surgeries, and he has never had an issue with the facial nerve. Yes, these things are still risks, but even though they're low he still has to inform us.

We discussed Audrena's vestibular issues. He explained to me that a normal person has 3 semicircular canals, and she is missing 2 of them. The only one that formed is the superior semicircular canal. As a result, she'll be unsteady, and she'll have to use visual cues to be able to walk. He had explained that before, but now that I know exactly what the problem is, I'm a little more worried. I haven't had a whole lot of luck with finding information on how the absence of the other 2 canals will affect her. What I have found is that those canals control things like vertigo and posture, so I'm worried that her little world has been spinning and we don't know it. It's amazing what we take for granted. As part of the cochlear implant team's evaluations, she will undergo vestibular testing. I'm scared of what we'll find out, but I'm also looking forward to knowing more so that we know how to help her. I've said it before. Knowledge is power. In cases like this, when I don't know anything about what I'm facing, I feel weak and scared. I would just like to curl up in a ball and cry. So instead, I gather as much information as I can. I educate myself. If I know what I'm facing, if I have a game plan, then I deal much better with the situation. The problem right now is that I don't know, and I can't seem to find the information I need. So if anyone out there has experience with what happens when a person just doesn't have all 3 semicircular canals, please share it with me! Educate me.

As part of the discussion, I asked whether we would just treat any ear infections the same, using the Ciprodex drops. He explained that during the surgery, he would take Audrena's tubes out and patch the holes. That's when I essentially went, "Wait. I have big concerns with that." Audrena was very wobbly before her tube surgery. She had so much fluid built up that she was late to sit or crawl. She would steady herself with one hand while she sat. She army crawled to get around. After the tubes were put in, she stopped using her hands to steady herself, she crawled, and she began to cruise around the furniture. It was a HUGE improvement. Given that she has these vestibular abnormalities, I feel like she needs the tubes to keep the fluid at bay. He said what I already knew, that the tubes are an access point for bacteria. But he did say that he would consult with other surgeons around the country via e-mail to see what they would recommend in this situation.

He did reiterate that Audrena has other issues that will make surgery more challenging for him. She has small mastoids, which is where the implants are attached and where they drill to gain access for the electrode array. The auditory nerve canal opening is larger than normal, causing the potential for a spinal fluid leak which would have to be packed with tissue, and the auditory nerve canal narrows to a narrower-than-normal size. He did say that these are things he can deal with, but it does make things a challenge.

Now to the hearing aid trials. The cochlear implant team will meet today to review Audrena's case. They will discuss when to start the hearing aid trials and how long we will need them. That may determine whether we will be able to get them from USD, or whether we will need to get them from Boys Town.

I am just feeling a bit overwhelmed today. Some days are good. Some days I wonder how we will ever tackle all of this and get past it. Today is just an overwhelming day. But in the midst of this, I'm extremely thankful that we have a doctor (and a nurse) who is willing to take extra time to talk with us. He had patients waiting for him, yet he made sure my questions were answered. I'm SO grateful for that, and I do remember that when we are the waiting patients.

The Beginning of Audrena's Hearing Journey

Audrena is our third child. She was born at 39 weeks via a repeat c-section. When she was born, the OB performed the surgery, and our family doctor, Dr. C., was present as the pediatrician. Both doctors said she was little. They guessed her to be about 6 1/2 pounds. Then she began to cry, and our family doctor said, "She's small but mighty!" Well, Audrena had them fooled. She was not all that small, at 7 pounds 8 ounces.

Audrena's newborn hearing screening came back referred on the left ear. Dr. C. told us not to worry, that about 50% of her babies are referred and test normal at a later date. We were not worried. After all, our other children had normal hearing. We got involved with life, and I needed time to heal from my c-section, so Audrena was 5 weeks old when we took her back to have her hearing re-screened. She was milk and soy protein intolerant, and we had not worked through some of the congestion that went along with it. Both ears were referred that day. The Audiologist advised us to just come back when her head had cleared up.

Daddy took Audrena back when she was about 3 months old. The left ear passed screening, but then she became fussy and just would not cooperate for them to re-screen her right ear. The Audiologist called it a pass on both ears since she had passed screening on the right ear at birth.

We continued to really enjoy life as a family of 5. Our older children just adored their baby sister, and we felt like our family was complete with 3 children.

Fast forward a few months. When Audrena was about 7 months old, I began to wonder why she wasn't saying "mama," "dada," or "baba." We thought she might just be slower to talk than our other children, who were actually early. In fact, she was later to sit up, too. She finally sat at 7 months, but she was relatively wobbly. At Audrena's 9 month well-check, I asked Dr. C. about her lack of speech. She advised us to just keep an eye on her because she might just be a little later. The third child often doesn't have to talk as soon because the older siblings tend to talk for her. So we did just keep an eye on her.

At 10 months, I decided we really needed to have her hearing checked. She still was not talking, although she did make plenty of other noises, and she did not seem to react to noises much at all. I made an appointment for her to see the Audiologist at our son's ENT doctor's office. Audrena did not do well at that appointment. She was referred on both ears.

The Audiologist had us come back a week later when we could see the ENT, Dr. P. They repeated the Tympanogram and OAEs, and again she was referred on both ears. Dr. P. took one look in her ears and said, "I don't know how her ear drum could have showed any movement at all. Her ears are full of fluid, and she has a double ear infection." We made the decision to place tubes in Audrena's ears since she couldn't have an ABR with all of that fluid anyway. Dr. P. was confident that we would not need to do an ABR and would notice an improvement in her hearing after the surgery. That made sense to us. Our son had needed tubes.

Audrena had her tube surgery when she was 11 months old. We thought we noticed an improvement in Audrena's hearing, but we couldn't really be sure. It still seemed hit and miss as to whether she would respond to sounds. We did, however notice a huge improvement in her balance. She was steady, and she quickly began to crawl, pull herself up on furniture, and cruise. We went back a month later for repeat testing. She failed the OAEs yet again, but she had fluid in her ears. Dr. P. sent us to Sioux Falls for an ABR.

The ABR was done March 21st, when Audrena was 12 months old. The Audiologist told us Audrena had severe-to-profound hearing loss, that Audrena would need cochlear implants, and she referred us to Boys Town in Omaha for a second opinion. We were crushed. How could this be possible? How could such a perfect little angel not be so perfect after all? How could this happen when we had two other children with normal hearing? It must be a mistake. After all, we did take Audrena to Dr. C. just after the ABR, and she had strep throat and an ear infection. Her lymph nodes were swollen and pressing on her Eustachian tubes. That must have caused an inaccurate test. Right?

In the meantime, I buried myself in research as a way of dealing with my grief. I read everything I could find: research studies, websites, blogs, books. I learned about the tests that would be needed to determine whether a person was a cochlear implant candidate. I felt like we were missing tests. I cried. I tried to prepare myself for the future. I rocked Audrena to sleep, and I cried. I tried to make it through my days at work without tears. I tried to explain to Reyana and Kelton that Audrena couldn't hear them when she talked to them because her ears didn't work. She couldn't hear things that they could hear. I cried. We learned a few signs and taught them to Audrena. We cried some more. I contacted our Birth to 3 Connections program to get Audrena signed up. I looked for any programs that might help us, but it was like walking blindfolded in a maze. I didn't know where to go or what to do next. I requested all of Audrena's medical records that might help explain her hearing loss, and I gathered them in a binder. One day, I found an e-mail address on the University of South Dakota's website, and it put me in contact with an Audiologist, Dr. M. In between it all, I cried some more.

We went to see Dr. M. at the end of March. She is USD's cochlear implant specialist and had started her career in Omaha. She went over Audrena's history and previous testing with us. After a few minutes she told us that no one could possibly tell us from the tests Audrena had done up to that point that she would need cochlear implants. Key pieces of the puzzle were missing. We needed more. She did do a Tympanogram and OAEs that day. Audrena did not pass. But we felt validated. Someone had listened to us! Someone really genuinely cared. Someone agreed that we were missing information. It was the first good day in months.

Dr. M. called Boys Town and consulted with them before our appointment. When we arrived, the Audiologists at Boys Town did a Tympanogram, OAEs, and some sound booth testing. Audrena tested the same. Then we met Dr. L.'s nurse, and she went over the medical questions. I gave her the binder so she could make copies of all of the records. Through a crack in the doorway, I could see Dr. L. reviewing things. He came into the room, introduced himself, and then stood against the wall as I unleashed a myriad of questions and concerns. Finally, when I was done, he examined Audrena and explained that we really did need more thorough tests. He was very considerate of our travel time, costs, and the need to put Audrena under anesthesia. Dr. L. told us that we would need a sedated ABR, and they typically do those at the downtown location. However, because he wanted to minimize anesthesia and travel, he would schedule Audrena for OAEs, sedated ABR, bone conduction, and a CT scan all at once at their West campus. He would be there to see everything as it was done, and we would have answers that day.

I explained to Dr. L. that we did appreciate that, but we would also need to have detailed notes from him explaining the need for each test so that our insurance company could approve it since Boys Town is not in network. He told us he would do it right away, and we could take a copy home. I told him it would be ok to fax a copy.

He explained it would take a couple weeks to get us in for the tests, but a couple weeks was ok. He acknowledged our concerns that Audrena was in a critical developmental time frame, and we could not delay testing any longer than necessary.

We left our appointment feeling very confident that we had found a great doctor. We had not even left Omaha yet when Dr. L.'s nurse called to say that she had faxed the notes, and that Dr. L. had been consulting with another doctor about Audrena. They had decided to also book an MRI at the same time. If he decided that day that he didn't need it, then no harm done. At least if we did need it, we could have it done immediately. Wonderful!

In the next weeks, we still grieved and cried. I read more blogs. One day I stumbled upon a blog by a family just a few hours from here. Their daughter had received cochlear implants at 10 months old, and they had the same doctor! I felt like I hit the jackpot. Not only did they have a fantastic experience, but their daughter was completely caught up (even ahead of her peers maybe) before kindergarten. They also included tons of resources on their blog. I e-mailed them to thank them for compiling the information in one area, and for sharing their success story. They e-mailed me back to say that Dr. L. is wonderful, but to be prepared that he's meticulous and slow in the operating room and will lay out all the risks in advance. They told me we would be completely scared by it, but just to know that he his GOOD. They also told us the same thing that the Audiologist in Sioux Falls had told us, and that I had read in numerous places: the success of a child with cochlear implants is hugely dependent on the dedication of the parents. In order to get her caught up, we should be prepared to speak 30,000 words per day to Audrena. We talk a lot in our family. We could do that. That was when I decided that I would not let go of those dreams for Audrena that I had thought to be broken. I would not adjust my expectations. With our help, she would succeed.

I took Audrena for a walk one evening, and I wallowed in self-pity at the fact that she could not hear the birds chirping or the cars driving by. I have a lot of those days. But I also have days where I think that Dr. C.'s words at her birth would prove to be true...she is small, but she is mighty. Audrena is a persistent and smart little girl. Daddy told me she scored at the 2 year old level in some areas on her developmental assessment with the Birth to 3 professionals. I couldn't be there that day, so he was there for the evaluation. Overall, the early childhood education teacher was not concerned with any other area of development aside from the areas affected by speech/hearing/communication. Small but mighty. That's my girl.