Sedated ABR/ MRI

When Kendall was 6 months old she had her last hearing tests to verify her degree of hearing loss. During these tests they looked at her brain activity to see any reaction to varying noise frequencies. To make sure they would get accurate results and not get any interference from her moving they sedated her. The entire process took about two hours total and she tolerated the sedation well. The results were as expected- profound bilateral hearing loss.

The last step in our cochlear implant candidacy process was to get an MRI. They needed to make sure that her cochleas and auditory nerves were fully developed. We were not too nervous about getting these results since we already knew that a flawed Connexin 26 gene was the cause of her hearing loss, but we still had to rule out any other causes.

She was such a champ and had no issues at all. The only problem we had was trying to keep all the medical equipment out of her mouth!!
 When Kemper had his MRI  they gave him IV sedation. This time with Kendall they just used gas to put her under and it was so much better. She had an easier time coming out of sedation and was back to normal in a very short time. It seemed like it took forever for K just to wake up post MRI.

Thankfully the MRI results were as expected too-  her auditory canal structure is intact as well as her auditory nerve so she is a perfect candidate for cochlear implants!

Heartbroken Part 2

6/24/2013 

I didn't mention this in Kendall's birth story post, but while she was in the hospital she failed her new born hearing screens. Four weeks later she failed her ABR and multiple other hearing tests. It is now confirmed that like her brother she too has a profound hearing loss. She is deaf.

It was like we were replaying the same scene from four years ago, but at least we were prepared this time. We knew that Kemper's hearing loss was genetic and that we had a 25% chance of  passing this on to any future children.  It had taken us a while to decide on having any other children but ultimately we knew we were not done. In addition we never wanted K to think that if we never had any more children it was because we were afraid they would be deaf too. We went into this pregnancy knowing this baby could be deaf and we were at peace with that possibility. The entire pregnancy I did my best not to think or worry about it. I took the Scarlett O'Hara approach to it " I'll think about it tomorrow."
  So when it came time for Kendall's hearing test we had a plan. When we had originally checked in the hospital we told the staff that we wanted her hearing test to be done at the latest time possible . We just wanted to enjoy her and this special time and not have to deal with it for a couple of days. We knew that either way the results went we were going to cry. It was going to be a very emotional time so we were in no rush.

The night before the test I was nursing Kendall and I couldn't believe how much she was like her brother. She looked, felt,and acted so much like him. She made the same noises, nursed,cuddled, cried all the same way. As I handed Kendall to the nurse that was gong to take her back to the nursery she made a comment about how Kendall was the quietest best sleeper in the nursery. Right then and there it hit me right in the heart. She was deaf.  I remember the nurses saying the exact same thing about Kemper after he was born. That gut feeling/ mothers intuition  I had been waiting for finally came. I immediately broke down crying and told my mom and husband what I felt. I think they were shocked because I hadn't voiced any thoughts before and I just blurted it out of nowhere. They tried to calm me down and tell me it was just hormones and nerves, but just like with her brother, I knew.
The next day when they came to take Kendall for the hearing screen we asked them if they could do it in the room with just my husband and I watching as opposed to taking her and then bringing her back with the results. They agreed and started the test. Shortly into the screening we could already tell what the answer was. When they get real quite and start nervously shifting in their seats you know something is up. Finally when it was over she turned to us and stated that she did not get a response in either ear. She quickly started giving us the "it could be fluid in her ears speech" blah blah blah. We knew what it meant. This wasn't our first rodeo.  Even though I already knew the answer in my heart it was still just as painful as was when we had received Kemper's results. Except this time for a brief moment I was pissed. This was the one thing I desperately wanted to be wrong about. I remember looking around as if looking for something to throw. All I could think was why? Why again? Did we not learn what we needed to the first time? We had a 75% chance of normal hearing, more than half!! WTH?? My husband and I just cried, hugged, and kissed our baby girl. I think one of the hardest parts was telling our family. To see their crushed expressions after we told them broke my heart. I know it's stupid but for some reason I felt like I let them down, I don't know why I felt like this but I did. Thankfully we have the most supportive family and just having them there was a reminder that we were not alone in this journey.

Later that evening my husband and I were treated to a celebratory dinner of lobster and steak by the hospital. They do this for all the parents of new babies, and in case you are wondering it did not taste like hospital food it was wonderful :).  We had originally planned to eat our dinner together privately so we could talk and mourn over Kendall's test results. K had a different idea. He wanted to stay with us and refused to go back home with his grandma. We tried to convince him he would have more fun if he went but he still said no. So we let him stay and had one of our family members pick him up his version of steak and lobster- chicken nuggets and fries from McDonald's. Turns out letting him stay with us was what we really needed at the time but didn't know it. He had us laughing and smiling the entire time. He was just talking and talking and talking.... Then it dawned on us, what are we worried about??? Look at him! he's perfect! He's a happy boy doing great. His sister is going to do just fine. We did it once and we can do it again. Enough tears and worry, we were not going to let this rob us of special moments like it did before.

I look back at this picture of myself and K and I can still remember the pain and agony I felt after his diagnosis. I was in a fog, my world was collapsing, and I couldn't even enjoy my nephew's first birthday party. I wish I could go back in time here and tell myself that it truly was going to be okay. Thankfully this time with Kendall I can.

We were at the Perot Museum and couldn't help but take a picture in front of this. We know all about the Genetic Lottery!!!

My heart is broken

After a great debate with myself I have decided to make this post. On Monday we took Baby Kemper to have a follow up hearing test since he failed his hearing screens at the hospital. He had an ABR ( Auditory brain stem response) which is a neurological test, where they introduce a noise to see if his brain has any reaction so they can test his hearing levels. They had me take him in a soundproof booth and they attached electrodes to his fore head and ears all while he was sleeping peacefully. I sat next to him and held his little hands and head so that he would not move in order to get an accurate reading. His father and my mother sat across from us waiting patiently. The test did not take long, about thirty minutes. When it was finished the audiologist confirmed my worst fears and told us there was "no response" Our darling little boy has a severe to possibly a profound hearing loss. My heart felt like it was ripped out of my chest. All I could think was WHY? WHY? WHY?!! I still want to know why, but that is something we may never know. Right now we are struggling trying to understand the situation. We are looking to God for help and support yet we are so angry with him at the same time. I know in the end we will all get through this but right now the hurt has taken over and we feel like we are in a fog watching the world continue as we stand still. I know there are alot of new technological advancements in medicine and options for little Kemper, so there is a little glimmer of hope. We will raise Kemper without treating him differently we just hope the world will do the same. Please pray for our little boy.