An Update from Grumpyland

Christian did fantastic today---he is SUCH an incredible patient. I have to give the kid props :)

I had a hard time getting out of bed this morning. I was out late with some of my girlfriends from the neighborhood, and didn't even go to bed until 11:30pm. We had to wake Christian up at 6:10, therefore ruling out any possibility of him even having the thawed Mommy Milk that I had for him. He was ok in the car, but started his chorus of "Baa Baaa" and vigorously signing "Milk" just when we hopped onto 95.

By the time we got to Hopkins, and navigated the massive campus to the pediatric neuro-radiology department, he was definitely hungry. I know how I feel when I am starving---I get cranky and snap at everyone. Christian definitely takes after me, as he was just a handful and kept signing "Milk" and "Eat". Poor kid. He probably thought we forgot about him.

The sedation was done by oral cholryl-hydrate, which definitely didn't taste good as it made him gag and freak out. You would think they would flavor it for these kids! After a bit of coaxing, rocking, and swaying, the monkey passed out and was taken away for the scan.

I peaked my head into the scan room just as they were starting the test. They had him wrapped up in a little bundle, and placed him in the massive machine. Even though he is such a big boy, he looked so small laying there. The nurse asked me to leave as soon as my eyes welled up. Of course, I cried---thank goodness Chuck is level headed and strong, or I think I would've shed a lot more tears than I did.

The test only took 10 minutes, and he was slowly coaxed awake by rubbing a wet washcloth on his face. He looked like a little drunk baby, with his head bobbing and swaying and his eyes opening and closing. He perked up when he was allowed to suck down 10 oz of his "Ba", the first thing in his tummy since 6:30pm last night. POOR KID!

So, the little hungover guy is home, resting and wobbling today. He's slept on and off, and he is recovering well. I'm glad we were able to get this test done so fast, and that he did so well with it. Here's hoping we won't have to sedate him again until if/when he gets his CI.

Getting Ready for a Grumpy Day tomorrow

We have to be at Hopkins by 7:45am tomorrow for Christian's CT Scan. This is just one of the many prerequisite appointments and tests that we have to take part in during the CI Candidacy Process.

I'm not really worried about the actual test--for those of you who know my kid, he's pretty easy, and is used to people poking and proding him. He's going to be sedated, which is always tough to see--but again, for those of you who know my kid, he's also VERY active.

I think the hardest part of the whole process tomorrow (other than me having to get out of bed before 7:00am), is not being able to give Christian his morning bottle. He's not allowed to have any solids or milk after midnight tonight.This will be his 3rd time being sedated, but our first since he's stopped nursing. For whatever reason, breast milk is allowed prior to sedation. A very nice nurse who called me this afternoon to give me this news recommended that perhaps I wake up and give him WATER or APPLE JUICE before 6am. OH GOD. I am SO not a morning person first of all. 6am! And my little Pooh Bear will turn into a Grumpy Eeyore if I try to pull that on him. Seriously....APPLE JUICE?

There is nothing that little man wants more than his AM milk. Chuck and I don't set an alarm anymore. We rely on the calls of "BA BA BA BA BA" coming from the nursery between 6:45am-7:30am every morning. He sucks down 10 oz of milk faster than any kid I've met.

So, I'm contemplating hitting the freezer stash I have of frozen breast milk and using that instead. Buddy hasn't had the good stuff in quite some time, but God knows I made enough of it for 3 babies and still have a pretty good stash built up.

Every little bit helps

We had behavioral booth testing today, and all I can say is OH MY GOD.

Something we're doing is working.

Maybe it's the constant enunciation. Maybe it's always making eye contact. Maybe it's reducing the background noise in our house. Maybe it's the repetition. Maybe it's just the aids working. Maybe he's gotten the hang of "Learning to Listen". Maybe it's because I'm literally in my kids face ALL WAKING HOURS speaking and signing to him and making every moment of our day together a LANGUAGE OPPORTUNITY.

Or maybe it's just because he's older, we're wiser, and he's a rockstar.

Whatever it is, we're going to keep doing it.

Christian's hearing loss is in the severe-profound range. Without his aids, he hears in the 85db-80db range. Today in the booth he had CONSISTENT responses in the 45-40db level. That puts his hearing loss in the moderate range aided. No kidding. We knew he was hearing alot more than we thought he was when he started throwing out words like Hat and Cat. I think that while he is doing alot of lipreading, that he really can hear that hard "T" sound. I never would've expected him hearing THIS well though. It's just wonderful.

Still, even with his fantastic progress, our audiologist today indicated that she believes he would benefit from a CI. The aids cannot be amplified anymore. In fact, she told us that other than for ear molds, there's not much more that she needs to do with him.

So, we're waiting for the Hopkins appointment, and we'll see what they say. In the meantime, I'll just continue to brag about how well my kid is doing.

Hopkins and Hoping

So, we took the plunge. After MUCH discussion with our Early Intervention team and audiologists, some prayer, and LOTS of anxiety, we made the call and got an appointment in a couple of weeks for a consult at The Listening Center at Johns Hopkins. Hopkins is one of the best Cochlear Implant hospitals in the country. Our consult is just the beginning of this next turn in our journey, and who knows where it will take us. We're not even sure if Christian would be considered a candidate.

Once again, we're waiting and trying to be patient.

Lucky Number 26

Sunday night, around 8pm our phone rang. The Caller ID tells me that it's our Geneticist from Children's Hospital. My stomach rolls, I look at Chuck, and pick it up. My first thought is "Whenever a doctor calls you this late, it can't be good".

We have a 10 minute or so conversation. I ask questions. I'm stunned.

I hang up. I cry.

Ok, I sob.

I struggle to get the words out to Chuck. He tells me to get it together because he wants to know what the heck is going on.

"It's Genetic. It's Connexin 26".

Thank God that I have a practical, put-together, ENGINEER for a husband. Chuck has this amazing ability to get to the root of a situation, remove the emotion and get to the facts...which, for everyone who knows me, knows that's something that I can't do on my own. Maybe it's because I'm a Mediterranean woman who wears her heart on her sleave. Whatever it is, it's just the way I am. And Chuck balances me out perfectly.

We sat down and I explained to him what the doc explained to me. As the facts were leaving my lips, I was slowly starting to realize that the news is not bad at all. In fact, it's good.

In very simplified terms, Connexin 26 is a protein that helps the inner ear function. When the protein is disrupted, the functioning of the inner ear is disrupted...resulting in a hearing loss.

It turns out, that Chuck and I are hearing carriers of the recessive mutated Connexin 26 gene. 1 in 35 people in the population carry the recessive mutation, and are called "Hearing Carriers". Chuck and I are hearing carriers. One of our parents on each side is a Hearing Carrier too...

Think back to your biology days doing Punnet Squares. Chuck and I each carry a BIG R (which is the dominant hearing gene) and a Little r(the Recessive mutated gene). We each passed down our Little r's to Christian.

It turns out that as Hearing Carriers, we have a 25% chance in each pregnancy to pass down the recessive gene to our babies, resulting in a hearing loss. Talk about odds...

Over the past several months, Christian has underwent a myriad of testing from renal-bladder ultrasounds, EKGs, and complete opthamological testing to rule out any other syndromes that may present themselves with the hearing loss. Due to the Connexin 26 diagnosis, we now can almost certainly rule out other syndromes, which is FANTASTIC NEWS. With Connexin 26 deafness, it usually is not progressive. We also can be pretty confident that his hearing loss is NOT due to a malformed cochlea. Which is even more wonderful news, as it makes the possibility of a Cochlear Implant for our kid even more real. Alot is known about Connexin 26, which is fortunate.

This diagnosis FINALLY puts the missing piece in this puzzle of Christian's hearing loss together. As his Mommy, I can FINALLY stop wondering if his Deafness was something that I caused during the pregnancy. I can't tell you how many times I wondered if one glass of wine did it, or when I was sick on-site at my last Convention that I directed. I am FINALLY free of the guilt. Sure, it's alot to take in. Sure, it absolutely SUCKS. However, as always, it could be worse. If this is all we have to deal with, we're lucky.

Genetic Testing....my thoughts...

I've been putting off posting an update about last week's Genetic testing at Children's. I haven't had that much time to really process it. I've been avoiding thinking about it. It wasn't a pleasant experience (although what part of this whole journey has been?), and it was definitely the hardest thing for me to deal with since the actual diagnosis. I mean, OF COURSE I want to hopefully find the root of Christian's loss since it is such a mystery to our family. OF COURSE I want to try to identify any other syndromes present (hopefully we can rule them all out). OF COURSE I want Christian to know about his loss for his future family. But, do I really want to know if I passed a gene onto my child that would make his life A LOT different and harder than mine? Do I really want to find out that my other children will be or most likely be or have a 25% chance of being deaf? I don't think I can deal with that yet.

I know it's completely normal to have all these guilty feelings. "They" say it's part of the grieving process. "They" say not to worry about these feelings, that it's completely natural. "They" even say that these feelings may never go away. "They" also say that sometimes there is a reason for everything and this is all part of God's plan. I'm not sure I believe everything "They" say....I can't imagine feeling this way forever.

Some folks have asked us about this test and our plans for more Children. Chuck and I want more children. Period. Regardless of these results, I hope that we will be as lucky as we were with having Christian. A friend of mine who is expecting her first baby asked me how hard it was parenting a deaf child, and how it must be such a stretch....in all honesty, I can't imagine it any other way. And other than having a slightly collicky baby in the beginning, Christian is an easy baby. He has the most loving, silly, and sweet personality. So, the possibility of us having more deaf children isn't a concern.

It all goes back to the "Did I CAUSE THIS?" question. I know I'm going to need more time to deal with these feelings. The bottom line is that no parent wants their kid's life to be difficult. Every parent wants their kid to have better than what they had. Our philosophy is that hearing or not hearing, we will do whatever it takes for Christian to have anything and everything that we ever had and more.

Quick Update

I completely forgot to post an update from the eye doctor appointment we had earlier this week. We saw Dr. O'Neil, who is a wonderful I might ad, and he checked out the monkey's eyes, and we got a clean bill of health. I feel so bad for Christian. He's been through SO MUCH TESTING, yet he is such a trooper. He makes me proud!

Waiting...again....

I just finished putting some calls into several Genetic Counselors. The earliest we can be seen is April...of 2008. Should I be surprised? No. I mean, did have to wait almost 6 months for a sedated ABR, and Christian finally got his aids at 9 MONTHS OLD. Waiting I guess is part of this game. And I hate it!

Some friends and family have asked us why we even wanted to do the genetic testing. The answers to this test are not going to influence our decision to have more children. For that, it doesn't even matter to us. Chuck and I just want to know if Christian's loss comes from a wonky recessive gene that we have, or if it was a spontaneous occurance. IDing the source of his loss will also help us rule out any other syndromes that sometimes come with SNHL.

So, we're just going to play the waiting game...something that we are all too familiar with !