Time to Give Thanks

As Thanksgiving approaches, I am always thankful for the gift of cochlear implants. This hearing journey is certainly not something we ever expected to endure, and I say "endure" because it has not been easy. However, it has been rewarding in the most awe-inspiring ways. It has taken us places we never dreamed and tested our family and even our marriage a bit at times. But we have come out stronger and happier. Audrena has come so far in the past 15 months, much farther than we expected in some ways.

We are thankful for Advanced Bionics. We are thankful for Boys Town National Research Hospital and every single person who works there. We are thankful for the University of South Dakota Scottish Rite Speech and Hearing Clinic staff, for our top notch Audiologist and Speech-Language Pathologists, and for the enthusiastic students. We are thankful for South Dakota School for the Deaf. We are thankful for a fantastic Physical Therapist. And we are thankful for the many friends we have made along the way, the families that we have connected with, and the mentors we have found.

We can't forget to be thankful for our two older kids, Reyana and Kelton. They have always been loving to their little sister and really watch out for her. They are the best language models for her, and she would not have come so far if it were not for them. I am sure of it. Our family, friends, and coworkers have also been huge supporters throughout the past couple of years.

In the past 15 months, Audrena went from hearing nothing to hearing whispers, hearing at 20 decibels with her implants. She went from saying only mamamamama to putting 3-4 word sentences together. Thanks to her physical therapist, she now walks good, runs, navigates uneven surfaces, and is even beginning to go down steps without constantly holding on to the railing. She did 4 steps the other night while holding a sippy cup and a treat, and with me hovering over her like a Nervous Nelly. Hey, in my defense there was concrete at the bottom!

I was a very shy child, and it has taken me a long time to come out of my shell. I'm not a very outgoing person, and neither is Randy. But this journey has put us in places we never thought we would be. We are finding ourselves filling the role of advocate as Audrena gets ready to transition out of Birth to 3 and into the school system. We have been mentors of sorts for other families, while in turn being mentored by other families further along this journey. Most recently we were asked to be involved in a PSA (which I think will just involve Audrena's picture being part of the announcement) and to be parent advocates for hearing screening legislation that is currently being drafted.

This hearing journey is certainly not something we would have chosen. Given the choice of Audrena with normal hearing, or Audrena with cochlear implants, I think I can safely say we would choose Audrena with normal hearing simply because normal hearing would be just easier for her. However, we love Audrena as she is, and if this is the path God has chosen for her, then maybe her purpose is to make a difference in the world of hearing loss. I have no doubt that she will go far in life. Who knows, maybe she will be a cochlear implant surgeon. Or maybe she will be the first deaf woman president. The moon and the stars are the limit. We are just thankful for great people and great technology to help her along the way.

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!

Audrena Goes Bilateral!

The past few weeks have been so busy that I haven't had time for an update. Audrena had her second CI surgery May 13th. The surgery took much longer than anticipated...about 1 1/2 hours longer!

The doctor ran into more scar tissue than he thought she would have. We received an update from the OR nurse that he was going to start the cochleostomy (ok, not sure how to spell that!). Later she called the room to say that he encountered some ossification and was still working on the cochleostomy. I was instantly sick to my stomach. I know that ossification in the cochlea can be a real problem for implantation. We waited awhile longer, all the while praying it would be ok. The nurse came into the room to say the implant was in, and they were testing it. I asked if he got a full insertion, and she said she didn't know. She doesn't get that information. I wondered if she was just dodging bad news.

Finally, the doctor came into the room. He said everything went well, he got a full insertion, and the wave patterns were beautiful. I breathed an enormous sigh of relief and immediately asked about her cochlea. He said it was fine. Puzzled, I asked about the ossification. He explained it was where he had drilled during the first attempt at implantation, and it had grown shut more than he expected. I explained that I must have misunderstood the nurse, and he agreed it would have been a big complication if there had been ossification in the cochlea. Everything went very well, and she did not even have a gusher this time!

It wasn't long, and they brought her to us. Audrena slept a long time, and eventually she started to look uncomfortable. The doctors approved some morphine, and shortly afterward the anesthesiologist and surgeon both stopped in to check on her. They said that with a 4 1/2 hour surgery and the extra "digging around" he had to do, she probably needed that extra dose of morphine to get her over the hump. The surgeon was comfortable releasing us to go to the hotel once Audrena woke up to eat and drink. At least she would be nearby if she needed anything at all. Soon enough, Audrena was devouring some hospital cafeteria meatloaf and apple juice.

We were released around supper time, so we ordered takeout and settled in at the hotel. I gave Audrena a sponge bath to freshen her up, and it was off to sleep for our little princess.

The rest is history. It was an easy...and on to activation!

Our rare flower...

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.

"The flower that blooms in adversity is the rarest and most beautiful of all." ~ Mulan

We have always known that Audrena is a rare, beautiful flower. Ok, so we think all three of our kids are perfect, handsome, beautiful little people, but Friday we found out just how genetically rare Audrena really is. At Boys Town, we met with two of the country's top Usher Syndrome researchers. One of them opened up our meeting by explaining that Audrena is one of only 20-25 people in the world diagnosed with Type 2C. That's right. Only 20-25 people in the entire world have been diagnosed with this particular type of Usher Syndrome. He said he has seen half of them, and the next youngest was around 18 years old. It's pretty remarkable that Audrena was diagnosed at two years old.

So what does that mean for us? Well, for starters, they can only tell us what they know, which is not a whole lot considering they have so few people to draw information from. However, they do expect Retinitis Pigmentosa to be mild for her, with night vision loss starting in the late teens and peripheral vision loss starting in the mid-twenties to thirties. If you ask me, "mild" is a relative term, considering that Usher Type 1 patients typically lose their vision at an earlier age and a faster pace. He explained that for them to participate in research studies, they need to have an assistant and usually a guide dog to travel. So I feel like it's all relative because for a "normal" person any vision loss is a big deal. Audrena may someday still need that assistant and that guide dog. It just might happen later in life for her. But we'll take it. Slower is better.

They were able to advise us about some things to protect her retinas, such as sunglasses and transitions lenses for her regular glasses. We asked about high doses of Vitamin A palmitate, but there is a fine line between slowing the retinal degeneration with the supplements and damaging other organs. Ultimately, we'll let the doctors help us navigate that path if it's an option.

We also discussed the genetics of Usher Type 2C. I didn't write down the statistics and can't remember what they told us, but suffice it to say that it was a freak thing for me and Randy to find each other. It's not likely for anyone in our families who happens to be a carrier to ever find a partner who is also a carrier. Likewise, our children should not worry too much. And then that brought us to our next question. Is it ever possible for a person to not have a hearing loss and still have Usher. The answer was that if our other children have normal hearing, then we shouldn't worry about them. Statistically, we have a 25% chance of having a child with Usher. Audrena just hit the jackpot, in more ways than one.

They discussed Audrena's abnormal vestibular system, the way her cochleas are wider at the opening and narrower at the opposite end, and they had reviewed the CT scan and operative report from her CI surgery. It was something new for them. Typically, a Type 2C patient doesn't have a CT scan because they are not diagnosed until roughly their mid-twenties and have only ever needed hearing aids, not cochlear implants. So they were unable to tell us whether any of Audrena's anatomical abnormalities are related to the Usher Syndrome, but they said it might very well be two different things happening to the same person.

Since we received the genetic test results, I have read a lot of news articles regarding research to cure Usher Syndrome and also Retinitis Pigmentosa. Many times I have heard that a cure is probably 10-15 years away. I asked them, "Would it be unreasonable for us to hope for a cure within 10-15 years?" The answer was no. It's not unreasonable at all. There has been progress with gene therapy, and things are moving along faster than what had been generally expected years ago. Plus, the actual research process is getting much less expensive, so they can do more with the funds they have available. One of them told us he expects Audrena to benefit from a cure because she is young, and she has time. She likely has that 10-15 years. That is what I call hope, right from the expert's mouth.

The appointment ended with them asking permission to do a write up for a medical journal about her case. Of course, we agreed because anything that helps the research toward a cure is a benefit.

We left the appointment feeling pretty good. The news was the best we could have hoped for, and our questions have been answered. Plus, they told us that we will be seeing the right doctors at University of Iowa. Audrena will be in good hands. We have hope. And we already know we have a small, mighty, rare flower who certainly IS blooming in the face of adversity.