What an exciting couple of days!

And I have a feeling it's only going to get better from here!

We had all of our pre-candidacy appointments at Boys Town over the past two days. It started with the vestibular testing on Wednesday morning. I was very nervous about it because the testing would help the doctors determine which ear to implant first, and possibly whether bilateral implants would even be an option for Audrena. We already knew that Audrena is missing two out of the three semicircular canals in her vestibular system. If the system had been working better on one side than the other, it would help the doctors make those important decisions. However, the tests showed that her vestibular system is not really functioning.

After the vestibular testing, we met with Dr. K. Our doctor, Dr. L., had a funeral to attend, and Dr. K. is the other implant surgeon. The team manager had assured me we would really like Dr. K., and she was right! He explained to us that, although it sounds bad that Audrena's vestibular system is not working, it's actually very good from a surgery standpoint. However, as we already knew, Audrena will have to rely on other senses for balance and walking skills. Water will also be more dangerous for her because people with absent vestibular function can't really tell which way is up in the water. So it will be very important for her to have swimming lessons, and swimming in a lake or river will be very dangerous because of the murky water where she would not be able to see the surface. As for the rest of our discussion, Dr. K. just spent time answering our questions and discussing genetics with us. I needed that. As Randy said to him, "You just lifted a huge weight off of her shoulders." Of course, nothing is concrete until we are able to meet with the geneticists and have testing done, but I had been worrying about a certain syndrome. Dr. K. doesn't feel that there is much of a chance that Audrena has it. Also during that appointment, Dr. L.'s awesome nurse (seriously, she's been really great to us throughout this process) gave us the immunization schedule for meningitis, and Audrena is up to par for the surgery. One less thing we have to worry about!

Then came the meeting with the Speech Language Pathologist. I was a little concerned about this meeting just because I had been told that in the past Boys Town recommended Total Communication. However, when we told her that we will be using Auditory Verbal Therapy, she was very supportive. We were happy to work out some details regarding coordinating therapies with our SLP here. Overall, the visit was very informative and just a really good conversation.

From there we took a lunch break at Perkins and went back to the hospital for our Audiology meeting. They did some sound booth testing and were able to get Audrena to respond to some very loud sounds. We still don't know if she was reacting to the sound, or to the vibrations. Then they went to a different room to test out the hearing aids. By this point, Audrena was ready for a nap, and things were not looking promising. However, when Randy saw the machine, he recognized it as something that Dr. M. had done at USD the day before. They were able to call and have the information faxed.

We had a good feeling at the end of the day. Then it was back to the hotel for a nap, and then we went to dinner and checked out Bass Pro Shop. Audrena loved the waterfall, the fish, and the glass elevator!

Yesterday morning, we started with the device orientation. It included a wide range of discussion about Audrena's history, our expectations (although there was a separate meeting for this), a little education about the workings of the ear, the surgery, and the opportunity to hold the equipment from each of the three cochlear implant manufacturers: Med El, Cochlear, and Advanced Bionics. We had already done our research and knew which manufacturer we wanted to use, so after we were given a thorough introduction to each company, we signed the paperwork for Audrena to receive an Advanced Bionics implant!

Our last appointment was with the CI team leader for our expectations discussion. He asked lots of questions about what we expect as an outcome for Audrena post-implantation, how we would feel and what we would do if for some reason the implants were a fail for her, and discussed the long-term commitments for therapy sessions and finances regarding equipment replacement. It was a really great discussion.

At some point, we also learned that Boys Town has the capability for private webcasts. So our therapists here could actually watch Audrena having a therapy session there to learn what kinds of things Boys Town recommends for her. When it comes time for the implant activations, they can record the session for us as well as broadcasting the session live. It would be a secure internet connection where our family and friends could watch from home as Audrena hears her first sounds! How exciting!

Keep in mind that the team makes a decision as to whether or not to proceed with implantation for Audrena. They together determine candidacy, not one single person on the team. They meet on Wednesdays, so next week they will review this week's appointments and make their decision. However, we were told that the team discussed Audrena a bit at Wednesday's appointment even though we had not completed all of the appointments. Hopefully that is a very good sign for us! We also heard some little bits of things that we interpret to mean we'll be receiving some good news next week.

Randy and I left Omaha feeling better than we have felt in a long time. Every single person we have encountered at Boys Town, and I do mean every single person from the receptionists to the doctors, has been wonderful. We feel like we will have a truly fantastic team of professionals working on Audrena's side to help us ensure she will achieve her full potential. So right now we're just waiting for that phone call!

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.

Beginning to Digest Everything

It has been a few days since our second opinion. We have cried, talked, cried some more, talked to family, and cried again.

Saturday morning at our local deli/bakery, Randy ran into a man with a cochlear implant. He approached the man, P., and asked if he could talk to him about his cochlear implant. They had a nice conversation, discussed the experience, the risks, and his results. This man had normal hearing and lost it suddenly overnight. The cochlear implant restored 90-95% of his hearing. He and his friends basically said, "How could you not give her a chance to hear?" Then they asked to put Audrena on their prayer list. ABSOLUTELY! Keep 'em coming!

We keep coming back to that question, "How could we not give her a chance to hear?" We have come to the conclusion that even some hearing would be a benefit. If Dr. L. was still comfortable doing the surgery, then things can't be that bad. Then the meningitis risk can't be that high. We vaccinate on time. Ok, so we do still need to know, and I did call his nurse this morning to ask about it, as well as to make sure Audrena's auditory nerve was really normal as the neurologist said. She was out today, so I will probably have an answer tomorrow.

Over the past few months, we have considered Total Communication (sign language as well as hearing, speaking, and lip reading), and we've decided that we will prefer to use Auditory Verbal or Auditory Oral Communication. The parent of an adult deaf man said to me, "Face it. It's a hearing world." She would have gotten an implant for her son, had they been available at the time. I know that there is a wonderful Deaf culture out there. Deaf people are very successful in life and have great careers. But I also know that I want to give my daughter the chance to hear. I want her to marvel at those chirping birds. Randy wants her to hear him say, "I love you." We want to hear her little voice. The studies I have read find that a small child (the younger the better) who receives cochlear implants and Auditory Verbal or Auditory Oral rehabilitation will do so much better with the implants. With the proper therapies, it's possible to have the child mainstreamed in Kindergarten, and really maybe even before. 30,000 words per day. I think I can. I think I can. I think I can.

I called our Birth to 3 coordinator today to tell her about Dr. L.'s recommendation for physical therapy. Audrena had not previously qualified because she showed fine in that area of the evaluation, although she's not yet walking. So Audrena will now receive physical therapy and speech therapy. The coordinator had also arranged for an outreach worker with the South Dakota School for the Deaf to be involved in Audrena's care. Wonderful! I had planned to contact them, but now I don't need to.

I received a call today from the genetics team. They meet once a month, and they only take 3 patients each time. It could take us 6 months or longer to see them. That should give me plenty of time to get insurance on board with the testing. I'm more worried about getting insurance to approve both implant surgeries. They've been good so far, so let's just keep praying. Time is of the essence for these surgeries.

As for Audrena's abnormal vestibular system, I really wonder if I have the same defect. I have kind of bad balance, especially at night. If I can't see, I have to steady myself with a wall, or else I fall all over the place. My mom's balance was not good, either. So maybe it is genetic. And maybe it's not going to be as bad as Dr. L. warned us it could be. I rode a bike. I walked a balance beam in gym class. Audrena seems pretty steady after her ear tube surgery. Maybe she's already compensating well. I think with some physical therapy, she'll do great. I bet she will ride a bike.

I'm a little afraid that the counselors on the cochlear implant team will think I'm really too unstable to take this on. They will want to discuss reasonable expectations for the results of the implantation. Reasonable expectations. I'm well aware that my expectations are probably completely unreasonable. I want as much therapy as possible for Audrena. WE are dedicated to getting her caught up to her peers in speech/vocabulary before Kindergarten. We want her to be mainstreamed. I envision her playing sports and participating in extracurricular activities. I see her becoming something hugely important, like the first deaf woman president. Small but mighty.

Will I need to adjust my expectations? Maybe. Will I be completely devastated if she doesn't have a fantastic outcome with the cochlear implants? Probably. But then I will pick myself up and move on. If she has some learning disabilities in the process and is not mainstreamed as we hope, we'll do our best. If cochlear implants do not work well for her and she needs sign language, then we'll change our focus. Because she is our child, we will make sure she has everything she needs to pave her path to success, whatever that success entails. But as of right now, I see no reason to adjust my expectations. Randy and I are dedicated parents. Besides, Dr. C. has proven over the years that she is rarely wrong. Small but mighty. I see a bright future ahead.

The Second Opinion

Last Thursday evening, we dropped Reyana and Kelton off at Grandma and Grandpa's house and traveled to Omaha with Audrena. We had booked a hotel room at the Sheraton since we would need to be at Boys Town by 5:30 am on Friday. The Sheraton offered a Boys Town patient discount, and it has been newly remodeled. Let's just say we highly recommend it. The room was clean and comfortable, with very nice furnishings, and it was affordable with the discount.

We arrived at Boys Town right at 5:30 and were checked in immediately. They took us to our room, where we put Audrena into a little pair of hospital pajamas and grippy socks. Then we went to the toy room to pass the time until they were ready for her.

At a little after 7:00, the anesthesiologist and nurse came to take Audrena. Another staff member brought us some breakfast. We waited for the ABR to be finished, not really expecting the results to be any different from the first ABR, even though Audrena would be under anesthesia for this one.

After awhile, Dr. L. came in to tell us that the ABR showed no response. That means they maxed out their equipment at 120 decibels, and Audrena couldn't hear any of it. She has profound hearing loss. In simple terms, she is deaf. I handled that news fine. I had prepared myself for it. Then Dr. L. explained that they would be taking her for an MRI next to check for the presence of an auditory nerve. Immediately we felt like he had yanked the rug out from under us. What?!? How could it be possible not to have an auditory nerve? I had never read that in any of my research. I vaguely remember asking what option we might have if she did not have an auditory nerve. He explained that there are limited options. They are just starting to do things like brainstem implants, and the only doctor doing them is in Italy. My world started to spin at that point. I wanted to throw up. I'm a planner. I have to know what is going to happen next. In this case, I was not prepared.

Dr. L. left the room, and I cried. Randy and I hugged each other and tried to convince ourselves it would be ok. After all, my cousin's daughter is deaf, and she is graduating high school, having lettered in sports, etc. She does wonderful. We know other people who cannot hear, and they do great in life. But they are not our baby girl.

Shortly after I finally got myself composed, the Audiologist came in to speak with us. She brought a hearing aid with her so we could see what Audrena might have. They typically do 3 months of hearing aid trials before cochlear implants for a few reasons. It gets the child used to something being on her head, and it stimulates the auditory nerve to some extent while the pre-op and insurance approval process takes place. I remember asking her what the chances were that Audrena would not have an auditory nerve. She said she didn't have any statistics, but she knew it was very rare. I cried again. Then she offered to forward the records to the Birth to 3 program and to Dr. M. at USD. She knows her from college days and had good things to say about her, suggesting that it really would save us a lot of time to work with Dr. M. on things that USD was equipped for. I signed some release forms, and off she went.

At some point the Audiologist came back with a cochlear implant packet. It had brochures and paperwork for us to fill out. We hesitatingly took that to mean that the MRI had shown an auditory nerve.

The nurses brought Audrena back to us, took her IV out, and told us we could take her to the toy room while we waited for Dr. L. I snuggled my girl and hoped for the best.

Dr. L. came in and said the neurologist said the auditory nerve was normal. He was not convinced it looked quite as it should, but it could have been a difference in computer screen contrast. He would have to call the neurologist and consult with him some more about it. They had also done the CT scan, which explained why things took a little longer.

Then he explained that Audrena has a larger-than-normal cochlear opening, which will cause a "gusher" in surgery. As he drills through the mastoid, spinal fluid will gush. He will need to pack it with tissue to seal it up. This is something he is used to handling, and he can take care of it. However, it will raise Audrena's risk for catching bacterial meningitis throughout her lifetime. As Randy got completely hung up on the word "gusher," I had it together enough to ask how vaccination affects that risk. Dr. L. said it does bring the risk down some, and he explained that as she is now, she's already at a higher risk due to that malformation. We were both trying to take it all in and neglected to ask for statistics or percentages so that we could assess what we're facing.

Dr. L. said Audrena also has smaller-than-normal mastoids, but again, he said he is used to dealing with that. All of this combined, though, means that he will not do a bilateral implant surgery. Audrena will have to have two separate surgeries, about 6 or so weeks apart. He wants to do the first one and assess how well she does with the implant before proceeding with the second surgery.

He did say that we could expedite hearing aid trials since we know they will not help. Our biggest delay, he said, will be getting insurance approval. He would get us scheduled to meet with the cochlear implant team in the meantime.

He told us that Audrena has a "very abnormal vestibular system," which means that her balance is bad. He said her vision will compensate for that, allowing her to walk, but she will need a night light or a flash light in the dark where she cannot see. Again, I had the rug pulled out from under me, and the only thing I could think to ask was, "Will she be able to do normal kid things like riding a bike?" He didn't know if she would be able to do those balance-dependent activities like bike riding. Randy had the presence of mind to ask if physical therapy would help, and Dr. L. did recommend it.

Dr. L. also referred us for genetic testing since we now know that Audrena's ear conditions are congenital, meaning she was born with them.

We left Boys Town feeling completely defeated. What if the neurologist was wrong, and her auditory nerve was not normal? How much benefit would she receive from cochlear implants? How huge of a risk would she be for meningitis compared to now? We were not prepared for two surgeries. And what about her vestibular system? How bad will it be for her? I was just sick, and the entire way home, I just wanted to throw up. Why couldn't have the MRI just been normal?