Activation #2

Yesterday was Audrena's second CI activation. We checked in at the doctor's office and had two x-rays to confirm that the implant was in its place. Dr. L. came into the room and had me sit in the exam chair with Audrena on my lap. He was having trouble getting the chair to raise up, so he bent over behind the chair to have a look. Audrena peeked around me and asked, "Where go?" I think Dr. L. just loves to hear the progress! And it sure made Randy and me smile!

Then it was time for Dr. L. to become the bad guy. He used some adhesive remover and took the steri strips off of her incision. It healed beautifully! Things looked good, and he cleared her for activation!

After lunch, we returned to The Lied Center. The activation was actually very anticlimactic this time. Audrena didn't give any exciting responses. However, she did hang in there long enough to give them a full audiogram using both implants. She also surprised us by putting the new CI back on when it got bumped off. She just started putting the first on by herself a little over a month ago. We were so proud, especially given that it was nap time!

And when we were done, some good friends from home were waiting in the lobby for us! They were in Omaha for their own reasons and stopped to say hi. We had some ice cream in the cafeteria and then headed for home with our bilateral girl!

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!

Learning to Deal with Usher Syndrome

 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.

We have had our ups and downs in the past month. Usher Syndrome has been a big pill to swallow, and I think those ups and downs are going to just be our normal. The past week has been a little more difficult for me. I think it's because summer is coming. We are starting to talk about camping, swimming, and other sunny activities. Every time I look outside at the sun, I think about the potential damage to Audrena's eyes. Bright light speeds up the progression of Retinitis Pigmentosa.

Sunglasses will always be a standard part of Audrena's life. That's not such a big deal. It's just plain healthier for everyone to wear them. But right now she doesn't have any. The big debate has been whether to order prescription sunglasses or transitions lenses now, or to wait until we see the doctor at University of Iowa. In the meantime, whenever Audrena is playing outdoors I think of those precious eyes. So Randy called University of Iowa and talked to the nurse. He explained that our appointment is not until mid-June. Should we order sunglasses now, or should we wait until she sees the doctor in case she makes a change in the prescription? They did recommend that we wait. Our options are clip-on sunglasses for her regular glasses, or a sun hat with a brim. Although logically good solutions, neither of those seem like fantastic options for us. Wal-Mart told me they don't make clip-ons small enough. I need to check elsewhere to confirm that. And a hat? Well, with a child who will soon have bilateral CIs, I see a huge hassle in the making. It's hard enough to keep one on when she plays with necklaces and hats indoors. Two will be even more difficult. We're not really sure what to do at this point, but we know we want to protect Audrena's retinas as much as possible.

When I look at old photos of Audrena in the first few months, it seems like I am looking in at another person's life from the outside. We were so carefree. Sometimes ignorance is bliss. But regardless of the struggles we are facing now, I am thankful we know now about USH2C because we can be proactive. I am thankful for cochlear implants because they have made a phenomenal difference in Audrena's life, and her future might be very different without them. We don't know what USH2C has in store for her. Retinitis Pigmentosa might be very mild for her. Some people make it into their 40s without any vision loss. Most people don't. Realistically, they might make it to their mid-teens. There might be a cure by the time Audrena needs one. Or there might not be a cure. But what I do know is that at least she will be able to hear, and she will be able to speak.

A friend, Susan, sent me a message last night. She is a fellow CI parent and an amazing mentor. I shared some of my worries with her, and I hope she doesn't mind me sharing what she said. She wrote, "All you can do is take one year at a time. No use carrying that big bag of anxiety too far out." Her advice has always been really great, so when I read that message I decided that I would try my best to do exactly what she said. As for preschool, she said, "The school seems much scarier than it is. You'll figure it out. Just make sure you have good teachers and FM/soundfield. She'll do amazing!" Thanks, Susan! You are wonderful.

1 Year IFSP 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.

Monday was our annual IFSP review meeting. The team wrote new outcomes, and I mostly watched. I was a little under the weather with a bad case of laryngitis, and Randy wasn't able to get away from work. Plus, I have said before that the goals are not my strong point. Thankfully, we have some very experienced professionals! Some of the things we will be working on with Audrena in the next year are ling-6 discrimination and using 3 or more words to express her wants and needs. Those things seem pretty simple on the surface, but there are plenty of things to work on behind the scenes that contribute to Audrena's overall success.

We also discussed Usher Syndrome a bit. Audrena has Type 2C, but I think she must be an unusual case. For those who might not be regular readers, Audrena is missing two out of the three semicircular canals in her vestibular system. They just didn't form. So she has poor balance, much like people with Usher Syndrome Type 1. Our Audiologist explained that balance is made up of three components: the vestibular system, vision, and proprioception. Basically, her proprioceptive sense will be all that is left when her vision starts to narrow. Things like gymnastics are actually very good for her because it will help her learn to compensate. Audrena's physical therapist already does a phenomenal job working on balance-related activities with her. He sets up obstacle courses with stairs, uneven surfaces, balance foam, etc. The new physical therapy goals included things like working on walking stairs without a handrail and jumping.

One of Audrena's SLPs asked how Usher Syndrome will affect literacy for Audrena. My answer was that hopefully she shouldn't have to worry about that in the immediate future. The Usher expert that we met with a few weeks ago said that most patients still retain enough central vision to read even at 50 years old. It was a valid topic of discussion, though!

We also discussed the upcoming CI surgery and whether we should expect the new "ear" to fully catch up with the first. Hopefully it will happen, but there are certain milestones (18 months, 24 months, 3 years), and we've already sort of missed two of them with the second CI. With surgery approaching in less than two weeks, we are just praying for a successful implantation and will worry about catching up once that second implant is activated.

Also, we did check out the preschool classroom that Audrena will be in next year. Our Audiologist and SD School for the Deaf Outreach Consultant looked at things like class size, carpeting, wall coverings, curtains, and the presence of a soundfield system. I will fully admit that the idea of transitioning out of Birth to 3 Connections and into the school district scares me. It's unfamiliar territory, and I don't do well with that. So I guess it's a good thing that I have a year to educate myself! And thankfully Audrena has a fantastic team working hard to ensure that she has the best possible learning environment! We are incredibly grateful for every single person on Audrena's team.