April 30, 2012

How a Cochlear Implant Works

For those of you wondering what a cochlear implant does and how it works, here is a link to Advanced Bionics' website. There is a section called "How Hearing with a Cochlear Implant System Works." It explains things pretty well but in easy-to-understand terms. There are actually 3 cochlear implant manufacturers. We will discuss the options for equipment with the implant team at Boys Town, but with the research we've done so far, we're considering the Advanced Bionics Neptune for Audrena. It's waterproof, so she would be able to wear it during swimming lessons.

I do plan to continue to post informational links and resources on this blog as we go. Hopefully they will help another family just starting out on the hearing journey.

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?

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.