Friday, September 24, 2010

The results are in...

Cochlear implants, here we come!!! Yesterday, Sophie's ENT called with the results of her MRI and CT scan and everything is completely normal. Her cochleas and auditory nerves are fully developed and she is an excellent candidate for cochlear implants! I am so relieved and happy! I know it is rare that someone with Sophie's degree of hearing loss cannot get implanted, but it was extremely nerve-wracking all week long waiting for her ENT to call. My cell phone never left my side and my stomach dropped every time it rang. So glad the waiting is over!

We also had audiology and therapy with Moog yesterday. Have I mentioned yet how much I love her school? Everyone is so amazing there. The teachers and audiologists are incredibly knowledgeable and very supportive. The other parents have been welcoming and encouraging. Each time we visit, seeing all of the other children playing and talking reminds me that Sophie is going to be just fine!

Anyway, we had a booth test yesterday (unaided) and Sophie did great. She is such a little trooper! They are working on teaching her to let us know when she hears a sound. To do this, she sits in a Bumbo seat and her therapist sits directly in front of her. There are TV screens on either side of her. Her audiologist speaks and plays sounds and tones through a tiny earphone at different levels and frequencies. When she responds to a sound, her therapist directs her attention to one of the TV screens and a bright picture appears. It took a few tries yesterday, but she was able to turn to the screen all on her own when she heard a sound. She is learning how to tell us she heard something. She is only 6 months old and we are already so proud of her! Her results from yesterday have been very consistent with all of her other hearing tests. Without her hearing aids, she is responding to speech at 95-100 dB, which is about the equivalent of a lawn mower right next to you.

Since we are now certain she is a cochlear implant (CI) candidate, we have a lot of decisions to make in the coming months. It's a good thing we have been doing a lot of research! I'm pretty sure we have decided to get bilateral implants. There are several pros and cons involved, but I know she will benefit the most from two implants. Also, we still need to pick a company and device. We have a good idea of what we want, but haven't finalized that decision yet. We have an appointment with her ENT on 10/4 and we will be talking about the timeline for her surgery. I would like to have her surgery in December, so that she can be activated and "hearing" by the time she is 10 months old (she will turn 9 months on 12/26). Moog is supportive of this decision, so I am hoping that her ENT concurs. We'll see! He is a very conservative surgeon and hesitant to perform CI surgery on infants less than one year of age. However, he has told us in the past that he is comfortable implanting Sophie around 9 or 10 months.

I'm feeling very excited and positive about everything lately. Sophie is ahead in all other areas of development and I'm optimistic that her speech delay will be minimal. I know we still have a very long road ahead of us and endless hours of therapy and audiology, but I'm looking forward to it.

Monday, September 13, 2010

Five years ago today...

Jared asked me to marry him. We were married a little over a year later. It feels like such a short time ago, but we have been through so much together in the past few years. At this point, I really feel like we can take on the world. We struggled for quite some time to even have Sophie, so when we found out about her hearing loss, we felt defeated. We just wanted to be able to be happy and stay happy. It was hard at first, but it would have been 100 times harder without each other. Times of adversity can be very telling about a person or a relationship. There is no other partner that I would rather have at my side during times of struggle. My husband is absolutely amazing and I do not credit him as often as I should. He is the perfect combination of optimism and rationality in a crisis and he completely balances my emotions, yet he knows when to just "be there" and let me cry. He is also the most eager, loving, hands-on dad I know. Sophie and I are so lucky to have him! Happy almost anniversary to my wonderful husband!

Sophie had therapy with Moog last week and we are working on teaching her to imitate us. In order to learn how to speak, babies must be able to imitiate. Most babies learn the ability to imitate by the time they are around seven months old. Jared has been working with Sophie and teaching her to stick out her tongue when he does. It is the cutest thing ever and she's pretty good at it! We are so proud of her!

In other news, Sophie has been pulling out her hearing aids even more. I know she's doing it for attention because she will go through the motions of grabbing at them when they aren't even on her ears. Then she gets upset that she is grabbing and nothing is happening-no one comes running to her! ;) She is sure developing a little personality!

Thanks to Lily's Mom, I also called Sophie's ENT and requested that they do the blood draw to test for Connexin during her testing on Friday and he will do it for us. Yay! Maybe we can get answers sooner.

Now, I will leave you with a picture of my little stinker:

Monday, September 6, 2010


It's official, Sophie has started pulling out her hearing aids every chance she gets. I was hoping this day would never come, but now I guess I have to face this new challenge. The other day, I was showering and Sophie was playing in her jumper in the doorway to the bathroom. Not two seconds after I got in, I hear a loud buzzing, look out, and see her chewing on her right hearing aid. Stinker!! I jump out and take it from her, not bothering to put it back in because I am soaking wet. A few minutes later, I hear buzzing again! I peek out to see her left hearing aid just inches away from the open toilet. She literally yanked it out and threw it across the room! I hastily finished my shower and made a mental note to ALWAYS make sure the toilet seats are down-her hearing aids are expensive!

She has been very tolerant of her hearing aids so far, so I just hope this is a phase and not the beginning of a daily battle! I don't want to resort to making her wear a pilot cap to keep them on. Ugh!

On another note, I have been reflecting lately on the last few months and all of the information we have been bombarded with. It is a lot to take in and we still have several big decisions to make regarding Sophie's future, but I think the worst part is that I still feel like we are in limbo. We don't know the cause of Sophie's hearing loss and I'm not sure if we ever will. As I stated earlier, there is no history of hearing loss on either side of our family and there were no environmental factors during pregnancy or delivery that could be the culprit. There is a recessive gene, Connexin 26, that could possibly be the cause. Everybody has two copies of this gene, and if Jared or I carry a flawed copy and passed it to Sophie, that is the cause of her hearing loss. 1 in 33 people carry a flawed copy of this gene, so it is relatively common. Her ENT is going to test for Connexin when he performs the surgery for her cochlear implant(s). I wish he would test her sooner, but it requires a complicated blood draw and she needs to be sedated. If we are carriers of the flawed Connexin gene, then we have a 25% chance of having another child with hearing loss. At this point, I'm not sure what I even want. Part of me wants Connexin to be the reason because at least then we will have an answer.

Sometimes, I feel like our life is on hold and, in a way, that is one of the hardest parts of dealing with Sophie's hearing loss. We can't buy a new house because we have to save for Sophie's tuition instead. We don't want to plan for another child until we discover the cause of her hearing loss, if ever. And now, geographically, we must stay in St. Louis because Sophie's school is here. Not that we were wanting to move anyway, but we both always have job opportunities that would require relocation. I start thinking about all of this and get frustrated because other people don't have to deal with issues like this. They have normal every day stresses and their biggest concern is something trivial. Then I start to feel guilty for feeling this way because I realize that things could be a lot worse. Sophie is amazing and she is going to be fine. There are parents out there who are helping their children battle a much more severe disability than we are, so what I am complaining about? It's seriously a vicious cycle that I fight: feelings of anger and frustration, and then feelings of guilt. Luckily, as time has passed, I've gotten better. Most of the time, I'm able to stop thinking negatively I soon as I recognize I am doing it. I realize how incredibly lucky Jared and I are to have the resources and support to help Sophie in every way possible. I know there are parents that are not as blessed and my heart goes out to them and their families...

Thursday, September 2, 2010

Can you hear me now?!

Sophie has had her hearing aids since she was about 10 weeks old. Everyone's first question is, "Can she hear?" You'd think that is a simple question, right?? Well, we're not sure. She has been tested with her hearing aids in and has shown several responses to sounds, but the tests are behavior-based and very subjective. We are hopeful that her hearing aids are benefiting her, but I'm not sure if we will ever be certain. There are times when she has no reactions to sounds and other times when I am certain she heard a noise. If I stop and over-analyze every single situation, I will lose my mind very quickly. It absolutely breaks my heart to imagine Sophie not hearing us talk to her and read her stories. Therefore, I force myself to be positive and assume she is hearing and her hearing aids are benefiting her. I have to, for my mental state. So, when people ask if she can hear, I say, "Yes, she can hear us." In the meantime, we are just counting down the days until she can (hopefully) get cochlear implants.

On 9/17, Sophie will be having an MRI and CT scan to confirm whether or not she is a candidate for cochlear implants. Her ENT has to determine how her cochleas are formed and confirm that her auditory nerves are intact. We have waited so long to have this testing performed because she will have to be sedated for several hours. I'm both excited and nervous for the tests! It is very rare that a child cannot get an implant, so I'm feeling pretty good about everything. I just want the results so we can move forward!

TGIF tomorrow! And a 3-day weekend! ;)

Wednesday, September 1, 2010

Our story

Our story can pretty much be summarized by me telling you about the best and worst day of my life - all of which happened within a month of each other.

I felt instant and unconditional love the day my daughter, Sophia, was born. I had such an overpowering urge to protect her from everything the minute she was placed into my arms. My husband and I chose not to find out if we were having a boy or a girl and I was so incredibly happy to have a daughter. It was the biggest surprise I had ever experienced. I knew by glancing at her daddy that she already had him wrapped around her finger as well, just seconds old. This was the best day of my life.

Sophia failed her newborn hearing screening at the hospital, but we were told that this was fairly common and not to worry. We scheduled a follow-up test at 4 weeks old. After bringing her home, we became worried when she didn't startle to loud noises, such as our dog barking. We discussed our concerns with her pediatrician, who also told us not to worry and she thought it was because Sophia has such a mild and easy temperament. My husband (Jared) and I vowed not to worry until we had the follow-up test. We took Sophia in for the ABR (auditory brain stem response) on 4/26/10. During this test, an audiologist placed electrodes on Sophia's head and tiny earphones in her ears and played a series of sounds and tones. Her brain stem response to these sounds was measured.

The testing itself is very nerve wracking for a first-time mom because they want the baby to be asleep the entire time. Very hard to plan for a one month old!! It was seriously the longest two hours of my life! I remember the audiologist coming in the booth at one point to check the earphones and my husband asked her how Sophia was doing. She looked at us with pity and I could tell she was choosing her words carefully. "Well, she's sleeping very well. You guys are doing a great job." It was at that minute that I knew the results. I vaguely remember leaving the booth and going to the nearest bathroom in a daze. You know the feeling where it is like you are outside of your body, looking down on yourself? That is what I felt. I was watching myself, as if I were in a dream. Surreal. I remember thinking, "Is this really happening? Am I awake? What is going on? What are we going to do?" I forced myself to go back into the booth, but I could not meet my husband's tearful eyes because I knew he realized it too. We could not pretend that everything was okay any longer. This was the worst day of my life.

Our fears were confirmed and Sophia was diagnosed with severe to profound hearing loss in both ears. At first, we were both very angry and confused. I just didn't understand. There is no history of hearing loss and I had a very healthy pregnancy. I did everything right! I wouldn't even chew sugar-free gum while I was pregnant! Why is this happening to us? It's not fair! This lasted a day or so, and then I went into research mode. Within a week, Sophia was enrolled at the Moog Center for Deaf Education and we had scheduled an appointment for her to be fitted for hearing aids.

Today, my husband and I are in a much better place and we are thankful every day for this beautiful gift we have been blessed with. Sophia, or Sophie as we call her, is absolutely amazing and I would change nothing about her. She is our angel. Right now, we are going through the ups and downs of being new parents and balancing life. We have the added responsibilities of visits to the audiologist, ENT, therapy sessions, and the joys of caring for hearings aids, but we seem to be doing a pretty great job so far.


This is long overdue, since I have been meaning to start this blog months ago. Our daughter, Sophia, was diagnosed with profound hearing loss in both ears when she was just 4 weeks old. Since getting this devastating news, I have found many blogs helpful and I have vowed to start one of my own. This will help express my feelings and keep others updated on Sophia's progress, but most of all I hope this blog can also help ease another family's mind, as other blogs have eased mine.