Happy 4th Birthday, Audrena!

Today is Audrena's 4th birthday. It never ceases to amaze me how far we have come in the past 3 years. On Audrena's 1st birthday, we still did not know for certain that she was profoundly deaf. We were really just beginning her hearing journey. In the past 3 years, she has endured several surgeries, countless hours of programming appointments, Aural Habilitation (speech/listening therapy) appointments, physical therapy, doctor visits, and most recently full day preschool. As parents, we have supported her through all of the same plus 19 1/2 hours of IEP meetings. (Yes, I am keeping track. Is there a medal at the end?)

And while we have come this far, there are still so many more years of this marathon ahead of us. Audrena has made leaps and bounds of progress this school year, but her articulation is still bad. It's pretty difficult for new people to understand her, so we continue to work on it. For example, she is now much better with the beginning and ending sounds that she used to leave off, and we finally have a /k/! The /g/ is still not there. We can see that she knows it is supposed to made in the back, and that it is different than the /d/. She just has not figured it out yet. But the progress is not fast enough for me some days. I will fully admit that it's hard for me because I wish the hearing loss was the only obstacle. Plus, Reyana and Kelton talked so early and so clearly that it's just what I am used to. I know we'll get there, though, because we have some really dedicated people on Audrena's team.

Aside from articulation, there has been progress with language, but there are still areas we need to work on like retelling stories, answering questions, including articles like "a" and "the," and not mixing up works like "I want marshmallow pink," instead of "I want the pink marshmallow." Again, we will get there. It's a marathon, not a sprint, right?

If I remind myself of the broad picture, the miracles are crystal clear. We went from not knowing that Audrena was deaf at her 1st birthday to having a pretty normal 4 year old who can communicate, even if it's not yet perfect. Her teachers tell us what we already knew...that Audrena is a smart little girl that will go places. Ok, I am boasting. We love all of our kids, and they are ALL going places in life. But she IS a smart little girl. She is an adorable, happy, healthy, spunky, stubborn little redhead. Some days that headstrong little girl makes me crazy, but at the end of the day I wouldn't have it any other way. I wouldn't trade our baby, or the hearing loss, for anything in the world. This journey has been hard and has tested us time and again, but it has been fantastic in ways we never could have imagined. We have learned so much about ourselves, our children, and our family as a whole. It has brought us closer and showed us to appreciate the small things.

We have big dreams for Audrena. Wouldn't it be fantastic if she grew up to become a cochlear implant surgeon? Or the first woman president? No matter what she chooses to do in life, we are confident that she will do it with that unmatched red-headed determination and with that trademark one-sided double dimple of a smile to win everyone over. Look out world! Here she comes!

And about this birthday? This year she partied with Chuck E. Cheese and told me which color flower she wanted to eat off of her birthday cake (the birthday child chooses the first piece). This birthday she can actually sing Happy Birthday to herself with a better sense of tune than I have! This birthday we gave her a bike because we know that, even with an imperfect vestibular system, she will learn to ride in order to keep up with Reyana and Kelton because that's the way she rolls.

Happy 4th Birthday, Audrena! You are a truly amazing gift from God.

Grandma and Grandpa gave Audrena this dress for her birthday. She told me she was wearing her pink and orange dress (check out the pink and orange balloons on it) and that she can twirl in it. :)

Waardenburg Syndrome?

From the beginning of our journey, I started reading about the genetic causes of hearing loss. I read about the characteristics of Waardenburg Syndrome, and I started to ask questions. We were always told no, that Audrena would have unique facial features if that were the case.

Since the Usher Syndrome mix-up, we have been seeing a really great ophthalmologist at University of Iowa Hospitals & Clinics. We see this doctor periodically to keep up with Audrena's eyeglass prescription and also because she works in genetics as well. She has been working hard to determine what caused the unique characteristics that Audrena has: heterochromia (her eyes are 2 different shades of blue), hearing loss, inner ear/vestibular structure abnormalities, etc. Over the summer, she asked if we had ever heard of Waardenburg Syndrome. Randy explained that yes, we had always questioned it because heterochromia and a lighter forelock runs on my (Carri's) side of the family. She explained that because I have the lighter color forelock and because of Audrena's characteristics, she would like to send a sample to be tested for Waardenburg Syndrome. Type II does not have the distinct facial features, and only roughly 20% of people with Type II have hearing loss. However, she said it does often come with vestibular structure changes. Of course, we agreed to the testing.

The test results showed one variation on each of two different Waardenburg Syndrome genes. However, those variations were ruled "not likely." Because the testing is relatively new in the world of genetics, the doctor asked us to do further testing. She will now be comparing samples from me and other members of my family who have the traits to try to prove her theory that we have a mild variation of Waardenburg Syndrome. If we do, the good news is that it does not change anything for Audrena. It is not progressive, and nothing else will develop. It is what it is. For the rest of us, it could be very informative, especially as the younger generation starts to think about having children.

However, if she does not prove anything with the testing, then we still do not have an answer. I trust this doctor, though, and I think she may be correct. I guess time will tell, but given what we went through thinking that Audrena might have Usher Syndrome I will take Waardenburg Syndrome, or even the unknown. In the meantime, we will continue to see this doctor for Audrena's glasses. It's a long drive, but it is worth it for the quality of care!

School Update

Today is the 100th day of school. I can't believe how fast those 100 days went! Reyana loves her teacher and is really enjoying 2nd grade. Kelton has matured so much in Kindergarten, and his teacher is fantastic. It has been a great year for everyone, especially Audrena.

We have seen a huge growth with Audrena this year. It seems we ("we" meaning her IEP team as well) have found a good combination of services this year with full day school, her SLP-A, and her speech therapy. I know we have a long way to go to get her caught up, but I also can't believe the amount of progress she has made this year. She is asking more questions, and more importantly, she is answering questions and taking turns in conversation so much better. Her vocabulary has expanded, and she is talking so much more. The average length of utterances has also gone from 3-4 to more like 4-6, and occasionally even 7 words.

Audrena has developed quite the personality this year. She keeps us all laughing, and she is the typical bossy little sister to Reyana and Kelton. Even when we are in our van on the interstate, she hears what Reyana and Kelton are doing behind her and tattles on them every chance she gets. Kelton was taking pictures with my iPhone one day as we drove down the interstate, and she made sure to tell us when she heard the camera click! Like every other child out there right now, she loves all things Frozen, and she tries to sing along with the songs even when she doesn't know all the words.

In school, they have been working on typical preschool things like letters, numbers, shapes, and colors. She can identify almost half of the letters randomly, she writes her name, and she knows her shapes and colors. I lost track of how far she can count, but I think she gets to around 15 before she starts missing numbers.

I still worry that she will not be caught up by Kindergarten, but I can safely say that I feel much better about where we are than I did over the summer. I can finally see the pace starting to pick up. We will continue to keep the ball rolling and even speed it up.

Recently in the sound booth, Audrena tested from 5-25 dB. There was one frequency where she was at 5 dB, and one where she was at 25 dB. Overall, she was hovering consistently in the 15 dB area. We are so impressed with her programming! Audrena has started to tell us when her batteries are low, or when she needs more toupee tape on her Naida processors. She asks for her "water ears" at bath time (the Neptune processors). And she has become a good little lip reader. We use very little sign language at this point, and yet if she is in bed and doesn't have her processors on, she still often answers our questions appropriately.

The miracle of cochlear implants never ceases to amaze us! Even with its limitations, the technology has provided Audrena with so much more in life than we ever dreamed possible when we started this journey. We knew that some access to sound would be better than none at all, but the results have far exceeded our expectations!