It's Official.

Harlie has moderate hearing loss.

There. I said it.

I am definitely bummed. Disappointed. Frustrated. Ugh.

I see the good things. We know. Therefore we can address it appropriately. And it certainly explains our difficulties with her behavior. And they do make hearing aids. And it's moderate loss - not complete loss. I am thankful for all those things. Trust me.

But c'mon! When am I going to stop learning about stuff that doesn't work right? And now I have to learn a whole new body part and all the details that go into how it is supposed to work, and why it doesn't work with Harlie. And there will be more appointments, more specialists, and more equipment. Super.

Yes, I'm whining.

For some reason, I really believed that she would get the tube, and that her ABR test would say she was perfectly fine. I really did.

But I guess that was more hope than belief.

So, here were/are my questions...

How could she pass the newborn hearing test with moderate loss? The newborn screening test is supposed to catch that.

Did they do the test - or was that incorrect information? Those first weeks were very busy and very crazy. Maybe it just got lost in all the flurry of tests and surgeries?

If they did it, was it done correctly? If so, did she have good hearing then, and it progressively got worse? Why? Could it continue to get worse leading to complete hearing loss?

Is this loss conductive or sensorineural? Or a little of both?

Most of my questions will never be answered. Nothing new, really. That seems to be the case with so many things about Harlie.

The audiologist that performed the test will get me a full report soon. Then I can read more about the specifics of her hearing loss. I think she said that she showed some sensorineural loss (which is damage to the nerves and is a permanent loss). But, I can't remember exactly what she said. We covered a lot of information in a short period of time. The report will really help me process all of this.

The good thing is that she said a regular hearing aid worn on her right ear should help her a lot. We'll have to get her a BAHA on her left - and we have to wait till she gets bigger for that. They anchor it into the skull, so the skull has to be a certain thickness.

So, I read this and wow, is it easy to get depressed over this!

But I won't. I can't. I need some time to process it, of course. Learning that your child has a disability is never easy. No matter how many things I've gotten over, I don't think it gets any easier. But, she's been receiving speech therapy since she was 14 months old. And we started using sign language at 16 months old. So, it's not like we haven't been addressing her language and communication challenges.

I will say that my faith in her intelligence is restored. With all of the issues we've had with her not cooperating, following instructions, etc. I was starting to doubt her capabilities! But, the fact that she's had three wonderful speech therapists in her three years and we didn't realize that she had hearing loss, speaks for her intelligence. She's been able to figure things out using other means, keeping the fact that she couldn't hear under the radar.

I think part of my struggle with this news is that I thought all along that she had one good ear. I can't tell you how many times I have thanked God that she could hear. Even after her ABR test last summer (which also showed moderate hearing loss - but the audiologist said that if she had an ear infection, that could affect the results and she did have an infection and a granuloma had grown in her ear canal - so OF COURSE that was the reason for her poor hearing results). If I had gotten her another ABR test after her ear healed and a new tube was placed, we would have known sooner. She could have gotten a hearing aid MONTHS ago and who knows where we'd be now.

I don't know what she hears when we speak. And that bothers me. What do we sound like to her? Do we sound like the teacher in Charlie Brown? What does the world sound like? What does music sound like? Does she even hear the narrator in Curious George (her absolute favorite tv show)? So, now I don't know what she sounds like, and she probably doesn't know what I sound like. That makes me so sad!!!!

Hopefully a hearing aid will help all of this. Maybe that will make me stop being a big baby about this. I just hate that I didn't know. And when given a clue - I didn't believe it. Granted, I think I had good reason not to believe it. But, still.

Here's my post from a few weeks back when she had the regular old hearing test that showed moderate hearing loss (that I still didn't really believe) with a video to show what it might be like for her.

Oh my. I just looked up my old post from when she had the ABR done last summer. It said she had MILD hearing loss. And that was WITH a blockage in her ear canal! What does that mean???? Does that mean that her hearing loss has been progressive?

Oh no. I gotta stop thinking about this for a while.

Another early appointment

Just a very quick one tonight since I have to get up at an un-Godly hour. We have to be in DC (at least 2 hours away) at 5:45am!!! So, we'll be loading a tired little girl up at 3:30am and hittin' the road.

Tomorrow morning is Harlie's ear tube placement and Auditory Brainstem Response Test. Hopefully this will answer her hearing capability questions - for good.

Life with Harlie has been very challenging the past few months (heh - okay, the last few years) not knowing what she can hear or not hear. Is she ignoring us? What does she understand?!?! She seems to be challenging me at every opportunity. And she is wearing me down.

All I have to say is if this tube helps her hear - and her ABR test comes back with normal hearing in her right ear - well, play time's over, Sister!

I'll let you know how it goes...

Goodnight!
~Christy

More good news...

We have a date for Harlie's ear tube placement and ABR test!

It is November 20. Which, of course, is a Friday - a day I said I wanted to avoid being in DC. Oh well. When you don't have a choice, you take what you get. I feel lucky that it's this soon. I know that her ENT worked really hard at fitting her in his schedule - and then worked really hard trying to coordinate it with the audiologist performing the ABR test.

I am very anxious to get some concrete answers as far as her hearing goes. It has been frustrating not knowing what she can or cannot understand. Not knowing what your toddler can hear/understand has made parenting her more difficult than I can put into words.

And now that this has been brought to the top of the priority list - we are going to start the process to get her a BAHA for her left side - at least. Her ENT told me that getting her that aid will really open up her world. So, that's what we're going to do.

Anyway, just wanted to write a quick one while I had a sec.

More later!
~Christy

CT Scans

So, Friday was a long day. Just as I expected.

Plans were changed at the last minute, so we didn't have to leave until 7am (vs. 4am). At first I was glad. But, then I thought about it a little more, and realized I preferred the earlier time. The sooner we get there, the sooner we get to leave was my thinking. And we were in DC on a Friday. Not that any day is a good day to be in DC traffic, but Fridays seem particularly awful.

At first we went to CT Scan check-in, but were told we needed to go to Surgery (since she was getting general anesthesia). When we got to Surgery, they told us to go to CT Scan. Heh.

Then I told her about the anesthesia and she said she didn't have any paperwork on her so it would take a little while. Hmmmm. It took over an hour till we were finally called back to register! I will admit that I was a little mad. Okay, a lot mad. After as many conversations as I had with various nurses in the days prior to the big day, I didn't understand why the paperwork wasn't ready.

Until a nurse came in to do Harlie's history and physical.

She told me that earlier that morning she was going through her patient files and saw that Harlie was there for a CT scan (not realizing that she was going to get general anesthesia). She said that she thought that was a mistake since they don't do CT scans. She took a quick look at her file and said "thank goodness I don't have to do the paperwork on this one" and went on about her day. HAH! It was actually pretty funny. I can imagine that from a paperwork perspective, Harlie's a bit of a workload. Well, I don't really have to imagine. I have many 3-ring binders to prove it!

Anyway, here's Harlie and Brandy while we were just hanging out waiting.



Harlie was fine and playful until we got in the exam room and the nurse brought in the hospital gowns. Harlie took one look at the gown and started crying. But, it was a very brave cry. Just a tear or two with absolutely no sound. It broke my heart. For all she knows she's checking in for a week or two. She has no idea. What a way to live! But, as you can see, she seemed to get over it pretty quickly.







I love this one. She looks so curious about what the nurse was doing. I have a feeling she's going to be dangerous with all her knowledge when she gets older...



I'm hoping a few of these quick hospital visits and procedures will make her feel less scared when she sees her next gown. Which, with any luck, will be very soon (for a new ear tube).

Her ENT was able to look in her ear while she was out. He said that her tube has come out of her ear drum. So, another will need to be placed. He said that since this will be her third, he's going to put in a different kind of tube that's designed to stay in longer. He said there was no granuloma or blockage in her ear canal. Bummer. But he said that a lot of kids with craniofacial issues can't pop their ears. And since her tube has come out of her ear drum, maybe that could be contributing to her hearing problems.

The plan now is to schedule an OR time for an ear tube placement, and then immediately after, do an ABR test. At that point, we'll know exactly what we're dealing with as far as her hearing goes.

Oh, she had a lumbar puncture so they could inject dye for some contrast scans. Since she can't have an MRI, they did some other scans to hopefully give them as much information as possible. They told us to run and get lunch quick, because it would only take about 15 minutes till she would be done. So much work for 15 minutes of scans!!! Anyway, we hurried back and waited. And waited. Then the doc came out to tell me that it took longer than normal to get the dye in her spine "due to her anatomy." I'm sure.

Then at 4pm they let us take her home. Ahhh, 4pm traffic on Friday in DC. The best. We finally got home after 7:30pm. A long day. But now we can proceed with lots of things. And that's good.

She will see her plastic surgeon in Norfolk on Tuesday. She will see her orthopedic surgeon on November 3rd. And hopefully soon, she'll see her ENT for her new ear tube. And, with any luck, after each appointment I'll have an idea of when her surgeries will be. Which, I like, since not knowing kind of stinks.

More later!
Christy

Speech Evaluation

Today we had Harlie's speech evaluation with her speech therapist. It was more of a formality than anything else, as clearly her speech therapist knows that Harlie needs and qualifies for private speech therapy. But, we have to go through the required process since she's three now and not in early intervention anymore. So, basically we're starting anew through our insurance than through the county.

However, Harlie was not cooperative, so it became much more than just a formality. A therapist came in with Michele (her speech therapist) to "test" or evaluate her. She asked her to do some basic things, like "Harlie, show me your shoes." Which she wouldn't do. Although she clearly knows what shoes are. Then she asked her to follow basic commands with blocks (like put them in the box, take them out of the box). Even that wasn't easy to get her to do. Then she showed her a flip chart with pictures on it. And she asked her "which boy is eating?", "which dog is the biggest dog?" and things like that.

The therapist who was testing her asked our therapist if Harlie's behavior could have been because Harlie was unfamiliar with her. So, Michele asked her some questions. This time she asked her while signing at the same time. And that helped a little. But the questions got more difficult. Like, there were five or six pictures on the page of various things (shoes, flowers, etc.) and the question was "what do you watch?" and the answer would be the TV. Well, she signed that question, and Harlie got it right. But, then she showed her a picture of a dog chasing a car and asked her "where is the dog's tail?" Well, she didn't get that one right. Or the next several ones.

She did terrible. It was hard to tell if she honestly didn't know or if she just didn't care. It really was terrible. And I hate the way that seeing her "test" like that makes me feel. I've always heard how smart Harlie is. And I've always believed that as well. So, to see her act the way she did was shocking. And I wasn't the only one who felt that way.

About halfway through the eval, Michele asked me about Harlie's past hearing tests. Here's the summary:

She passed a newborn hearing test shortly after her birth - in her right ear only. She has no canal on her left, thus no hearing on her left is possible at this time (and we don't know what's going on inside her ear, so potential for hearing in the future is unknown).

She had many ear infections on her right, so she had a tube inserted a long time ago.

Last summer (2008) she had an ABR (auditory brainstem response) test. It's performed when the patient is sedated and they look at brain wave activity. Well, the results of that ABR were not good. They said she had moderate hearing loss on the right side and complete on the left. I was shocked (about the right side, not the left) and didn't believe it to tell you the truth. And then the audiologist said that if she had an ear infection or something that could affect the results. Oh, and she didn't look in her ear, either, so there you go. I was not impressed.

About a month or so later, she was seen by her ENT (at a different facility than where she had the ABR test done). And her ENT said that her ear tube had come out of her ear drum and was stuck in her canal with wax and a granuloma had started to grow around it!!! So her canal was completely blocked.

Well, that explained the bad results from the ABR. So, her ENT removed the blockage, and the granuloma and after it healed, he put in another tube. And I went back to thinking that her hearing was just fine on her right side.

However, today It was pretty obvious that she was displaying signs that hearing was an issue.

Michele said that Harlie is so smart that maybe she's been getting by using other ways to figure things out - other than hearing, I mean. And she does understand sign language when she looks at you. And the results of this evaluation today made me really look at things in a new light. I think she hears the phone ringing. But then when we got home today, she didn't look when the phone rang. It could be that she doesn't care because she's not going to answer it, or talk on it. But I know that I've seen her look at her monitor when it beeps.

Michele also asked her, "Which boy is taking a bath?" Harlie saw her sign bath and immediately pointed at the right picture. She also asked her "Which boy is running?" She didn't get that one right. But, she doesn't run herself. Could that have affected if she knows what it is? Have I ever made a point to teach her what running is? Am I supposed to? I didn't have to "teach" Murphy that. I bet I'm going to over-think this.

I've just always thought she was ignoring me. But, now looking back, if she doesn't hear well, that might explain some things. In preschool, she hasn't wanted to participate in circle time. If they are talking about the day, or reading a book, and she can't hear them very well, of course she's not going to be interested.

And if I'm scolding her for something, or trying to get her to do something she doesn't want to do, she will NOT look at me to avoid my signing. It is VERY frustrating. And Michele said that children who can't hear will purposefully avoid watching signs. Which leads us to a behavior problem that I have NO idea how to address.

However, to a child with language problems, asking them a question can be very stressful. It's just not the same as asking a typically developing child a question. Language does not come easy to them. Tonight for example... I told Harlie to pick a book and I would read it. She brought me one and I asked her (while signing) "Do you want me to read this book?" I want her to nod her head or sign yes. She won't do it. She doesn't respond at all. Could it be that she doesn't understand she's supposed to answer? When I ask her if she needs to go potty, she'll shake her head no. So, I think she understands a question. Could it be that she's thinking, "um, yes mommy, duh, you told me to pick a book and you would read it." What is going on in that mind of hers?!?!?!

The bottom line is that she needs to be tested soon. Very soon. We need to properly address whatever is going on. I also need to contact her ENT and see if he can look in her ear to see if there's a blockage. That is my hope.

Overall, today was a difficult day. It is very hard to see your child not answer questions correctly. And basic stuff that she should know. And it is very scary to think that this is another thing that we have to address now. Which means more appointments, more tests, more results, more learning and possibly more action. And I can't help but think that it's my fault that she doesn't know more. Should I have done more? Done something differently? But how? Seriously! How can/could I do more?? And it's so overwhelming. And hard. So, so hard to have a child with so many challenges. Indescribably hard. Murphy and Cooper just do things naturally. And the learning just happens. I don't have to think about it. I don't have to ask professionals for help and advice all the time.

I was thinking today that everything I feel is in extreme - nothing is middle of the road. I feel happiness to an extreme. Or I feel devastation (heart broken even) to an extreme. It's an exhausting way to experience life. And today wore me out. I'll pick myself back up tomorrow. Don't worry.

Thanks,
Christy

Happy Harlie

Today went great. Harlie is her happy self. As if yesterday never happened. She is doing great with putting things in her mouth.

Speech Therapy

Beth, our speech therapist, seemed really pleased. She showed me some mouth exercises to do on her. They are basically the same ones we did prior to her jaw surgery. Now that Harlie can open her mouth - wider than she ever has - we can see a lot more of her tongue than ever before. It is fascinating, really. I find myself trying to look inside whenever I can.

Keep in mind that Harlie has never licked her lips or moved her tongue, really. For her whole life, her tongue has been rolled toward the back of her throat. She's never used it to move food around, or feel toys. So, as a muscle, it is VERY weak.

So, Beth did some tongue tapping to see if she could trigger a tongue reflex. Basically when you put something in your mouth, the tongue should find it. The first time she did it, Harlie's tongue didn't move. Just as Beth expected, considering she has never really been able to move her tongue. The second time she did it, Harlie moved her tongue towards the object in her mouth. Beth said sometimes that can take a month of therapy - and Harlie did it the second time!

The best part is that she is so incredibly tolerant. After 9 weeks of no stimulus, you would think that she would be way over-sensitive, but she's not at all. No gagging or anything. And tonight, I went on ahead and got out the tooth brush, and she went right to it, as if there was never a break. We are all very excited about all of this!!!

Auditory Brain Stem Response Test (ABR)

Basically, her ABR study yesterday offered us NO insight whatsoever. It was a big, fat waste of time and effort. In the most basic of summaries - the audiologist said that Harlie has mild hearing loss in her RIGHT ear (her good ear). I was very unpleasantly surprised at this. She said that she has TINY ear canals and had to use the infant sized supplies to test her. But, after she said all this she said that a blocked ear tube or fluid in the ear could cause the delay in her hearing response - but she didn't look inside to see if that was the case. WHAT?!?! Are you kidding me?

Then she said that she got no response at all on the left ear. I asked if that means that she does not have an ear drum or inner ear in there and she said she didn't know. WHAT?! Really, it's not even worth going into more detail. She didn't know anything other than that a bone anchored hearing aid (BAHA) could work for her. But they don't actually anchor it until after they turn 5, so before that kids have to wear a headband.

Whatever. I'll clearly have to re-test her somewhere else. So, we still don't know anything. Not that I'm THAT surprised, really.

Okay, well, it is late. I'll have to save the rest for tomorrow.

Take care,
Christy

Just a few more hours...

Since the hospital here in Norfolk doesn't have wireless, I thought I'd give a quick update before I go to bed.

We have to be at the hospital at 5:30am. So, we drove down tonight and are staying at my friend Melissa's for the night. She will keep Murphy for us tomorrow while we're at the hospital with Harlie. Harlie's scheduled OR time is 7am.

She will be getting her wires removed and will also have an Auditory Brain Stem Response test (ABR). That will tell us if she has an inner ear in her left ear. I haven't thought much about that, really. I guess because I have pretty much been expecting to learn that she doesn't have the ability to hear. Not that I'm being negative, just realistic. Even if she does have an inner ear, surgery to give her a canal to enable her to hear is still very new to the medical field. So, that decision would not be an easy one. Although, it is my understanding that if she does have the ability to hear (even without a canal) we could give her a bone anchored hearing aid. So, I think there are some options, but no sense in getting all caught up in that until we know more.

I will say that it will be very nice to finally know, one way or the other. It is hard to believe that we've made it this long without knowing. I remember right after she was born - I wanted all my questions answered immediately and I just didn't think I could take the waiting. But here we are 22 months later and we have waited just fine. I surprise myself at the amount of patience I have been able to learn!

Well, I better get going. Although I have to get up in less than an hour and a half (at midnight) to turn off her night feed and start Pedialyte, then get up again at 3am to turn off the Pedialyte. Not to mention that I feel like I'm 5 and tomorrow is CHRISTMAS!!! I just can't wait to see her smile without the wires!!!

I'll update as soon as I can! Take care,
Christy

Moving along...

Things are still improving. Slowly. But improving. The only thing that is getting worse is that Harlie is stuck to me like glue. She wants to be attached to me 24/7. It’s not good enough that I’m on the floor playing with her, she needs to be in my lap. And sometimes, that’s not good enough, she needs to be wrapped around me. Either way, she must be touching me or she cries.

For the most part her incisions look pretty good. Except for one under her jaw (there are three under there). But I spoke with Dr. Magee’s nurse today and she said as long as it doesn’t look infected then it is fine. I don’t think it’s infected, it’s just bleeding through a little bit. Her trach collar irritates those incisions at night and during her nap. But, there’s nothing I can do about it. She has to have the humidification.

I thought I was going to have to take her out in public today. I didn’t know if I was ready for that. Murphy had a doctor’s appointment and Brandy couldn’t work today so that meant that I would have to take both kids for his appointment. But it turns out I didn’t have to worry about it. The office called to reschedule the appointment (doc was stuck in the OR). Murphy will be seeing the same doc that did Harlie’s nissen. Murphy has to have a relatively minor surgical procedure. But I’ll know more about that next week.

Dr. Magee’s nurse did say to give her a few more days and then I can take her out all I want. She said it might do Harlie some good to get out of the house. Which makes sense. Plus, it will be better when her hair isn’t coated with Bacitracin. The greasy look isn’t really a good one. Ever.

Harlie still doesn’t want to walk. Her occupational therapist came yesterday and she said that maybe Harlie was feeling top heavy due to all the swelling. The bruising seems to be going away a lot faster than the swelling. I certainly notice that more anyway. For some reason only the right side of her mouth can go up when she smiles. Her left side still isn’t moving. Which is strange to me considering all the major work was done on her right (that’s why her right eye was swollen shut).

The derma bond (glue) is starting to peel from the incision between her eyes. I want to pull it. But I must resist the temptation. That incision is where they put the screw that holds the wires. When the wires come out, they will leave the screw in there (it’s titanium).

We have a date for the wires to come out, August 5th. Which is exactly 9 weeks from her surgery and 8 weeks from now (not that I’m counting or anything). They are also going to do the ABR (auditory brain stem response test) on her left ear. They were supposed to do it during this past surgery, but it was cancelled for some reason. So we have to wait 8 more weeks to find out if she has an inner ear (which could mean the ability to hear down the road). Both procedures will happen while she’s under anesthesia and will be a day surgery thing – in and out in one day. Woohoo! Haven’t done one of those before!

We also have a date for her third heart catheterization. That will be August 19th. The last one was last June and did not show good results. HOWEVER, that was BEFORE her lung surgery that changed everything! So, we are all hoping that her better lung function will mean better heart function and results.

I suppose I will try to squeeze in an appointment to see her ENT in DC between the wire removal and heart cath. I am assuming that’s who will tell us if her jaw surgery was a success, and if so, what kind of decann plan (getting the trach out) he will have.

Well, Mike and Marcy had their baby yesterday. He’s a tiny one – 3 pounds, 10 ounces. But he’s doing great and breathing on his own. Marcy is doing well, all things considered. And Lindsay had her baby, she’s adorable. Hopefully I’ll get to see them all tomorrow. Well, that’s it for tonight.

Take care,
Christy

Went to the zoo

Well, on Friday we went to see an ENT in Newport News (his main office is at CHKD in Norfolk). The reason why we went to see him is because I was told by Harlie's ENT in DC that we should also see someone who is part of her craniofacial team about ear reconstruction. He was recommended by Dr. Magee. I liked how he opened, "So, I see you need some more doctors." I love funny people. He spent a lot of time with us explaining everything. Basically, it comes down to this:

1. We need to test to see if she has the inner workings and ability to hear on her left side. The test is called an Auditory Brain Stem Response (ABR) test.

2. If the test shows complete hearing loss, then all we will need to focus on is ear reconstruction.

3. If the test shows that she has some hearing capability, then we need to determine how much and what we want to do about it.

He does not recommend ear canal construction (actually giving her a canal). He said that the path is never "normal" and the risk to hitting a nerve and causing facial paralysis is too high.

He prefers a Bone Anchored Hearing Aid (BAHA). The hearing aid is drilled into the bone above the ear.

As far as the actual ear building - he always sends parents to see a guy in Northern VA to talk about a prosthetic ear first before considering reconstructive surgery. He used to be a CIA disguise specialist. Pretty interesting, huh? But, we have plenty of time before we'll be looking into all that.

We also discussed her future jaw reconstruction surgery. He explained why it is so complicated. Especially with her missing some bones on the right side of her jaw. But, I will not think about this until I speak to Dr. Magee since he's the one that will be doing that.

So, as of right now, her ABR test is scheduled for October 23rd at the children's hospital in Norfolk. We will get the results immediately, which will be nice. Hopefully she wouldn't even have to stay overnight. But, with Harlie and anesthesia, I won't count on it.

So, enough medical stuff for now.

On Saturday, we (Tom, Murphy, Harlie and I) went to DC to the zoo. The Children's Hospital's Heart Institute sponsored an event there.

On our way up, I sat in the back between Murphy and Harlie. I do not recommend this. Especially for long trips. It took only a matter of minutes till I had regressed into a child myself.

"Murphy, stop touching my seat belt."

"Sorry, Mama." lots of laughter.

"Murphy, stop touching my seat belt."

"Sorry, Mama." lots of laughter.

"MURPHY! I SAID STOP TOUCHING MY SEATBELT!"

"Sorry, Mama." lots of laughter.

"YOU TWO, KNOCK IT OFF!" (that was Tom)

And while all that was happening, Harlie kept taking off her HME (the barrel thing that covers her trach). Literally, every 2 seconds. So, I keep saying, "No, Harlie!" To which she just smiles and laughs. And I have to put it back. Over and over again. I really don't know how I am ever going to discipline that child.

But we made it up there in one piece. And the weather was perfect. It was so nice to get Harlie outside. She kept on swinging her legs off the end of the stroller. It was really cute. And it was really nice to see our social worker (Kristen) and some of Harlie's nurses, and doctors. Especially since she is doing so well now. And it was good to meet other heart families as well.

Well, they had a raffle and they gave a lot of goodies away. And if you can believe this, we won the grand prize - a week's vacation in Myrtle Beach. So, now we HAVE to take a vacation! We are already looking forward to it. Funny, because I was really bummed we couldn't take a vacation this past summer.

So, then we leave. Get to our car, and we have a flat tire. Tom said there isn't just one nail in it - there are two! Funny how things balance out sometimes.

Well, that's it for tonight. Talk to you soon!

Take care,
Christy