We really haven’t fallen off the planet! Life has just been happening. So this is going to be backing up and jumping ahead all in one post. Just trying to keep you on your toes!
Back in March of 2014, E had a test done at the ENT. It’s called an ABR. I don’t know what it stands for and don’t really care. It measures how the brain responds to sounds rather than how the person responds to sounds. So, in order for this to work properly the patient has to be asleep or sedated. She’s right on the cusp age-wise of when they would require her to be sedated in an OR or just getting her to sleep in the office so we decided to go ahead with the latter and try it out. I was not looking forward to this. She has never fallen asleep in anyone’s arms without being SUPER tired. This appointment was early in the morning and I knew she wasn’t going to be tired. They did give her a little bit of medicine to make her sleepy but it wasn’t much more than Benadryl. They put us in a darkened room and I was tasked with getting her to sleep. NOT an easy task for a child that never settles. I finally managed to get her to sleep and then when the nurses came in the light from the hallway landed right on E’s face and woke her up. I thought we were done for sure but I managed to get her back to sleep again by nothing short of a miracle. The were able to get the electrodes placed and everything and kept her asleep. We got through the left side test (the side with an ear) but she woke up again when they tried to move the electrodes to the right and there was no getting her back to sleep a third time, although not for lack of trying. So we never got a reading of the right side (without the ear).
The was taken during the test.
I took a picture of the computer screen while the test was happening. I just thought it was interesting.
So, we found out that the hearing on the left side with a tube now is almost normal. There is an obvious hearing deficit though so after a few more meetings with the Dr. we decided to go ahead and get her fitted for a hearing aid. Jumping ahead to July - E got fitted for a BAHA (Bone-Achored Hearing Aid). It’s unilateral right now but after she’s gone through a few more hearing tests they might decide go with bilateral devices. Her hearing is definitely better with the aid but it could still be better. She is doing well with the BAHA. It’s attached to a headband right now with a plastic part that is placed on the patch of skin right behind where her right ear would be. This is really just a very sophisticated microphone that amplifies sound and conducts the sound through bone into the middle ear. The Dr. is thinking that although hearing tests close to normal on the left side that since her ear canal is so tiny and malformed that the sound isn’t being conducted through it very well. It’s still too soon to get a definitive picture of where her hearing is at. Especially since she lacks the skills to communicate anything to us.
She has been in speech therapy since July 2014 and that has been going VERY well. Tammi, her speech therapist, has made several comments on how far she’s come in such a short amount of time and that the BAHA has definitely improved things. E can now make a ‘p’ and a ’t’ sound, which are both things she could not do when she started. There is still some question as to whether she is physically capable of making some sounds like ‘g’ or ‘k’. One thing Tammi has also helped out with is getting E set up with the craniofacial/cleft team at the children’s hospital close to us. We feel like Dr. Fearon (who did her cleft repair last year) did a great job but we felt like his office staff was AWFUL. No one kept us informed of anything; we’d call and call and call and no one would ever return messages; and we just didn’t get any help from anyone. We were just left with lots of questions and no one to answer them. He wasn’t very helpful in getting us set up with other specialists either and I felt like I had to do all the communicating between Dr’s and that just wasn’t very efficient, especially when you can’t get the main Dr to call you back! So, I told Tammi all of this and she has been such a help! She specializes in children with hearing impairments so she didn’t really have experience with cleft kiddos but that didn’t stop her. She researched a bunch on her own and found this cleft clinic and called them to talk to their speech pathologists and they told her to have us make an appointment. This all happened within a couple of weeks of our first contact with Tammi. So we set up an appointment and Tammi even came with us all the way down there so that she could learn and hear firsthand what everyone had to say. We didn’t learn anything new but at least we felt like there were doctors and therapists finally willing to listen to us!
We took both girls with us and no one believed us when we told them E was older! They both did really well.
They put this headband on E to hold back her hair so they could take some pictures of all her features. She didn’t want to give it back and had lots of fun putting it on and taking it off.
We had lots of waiting time in between specialities to play with all our toys.
Or sit in our stroller and blow spit bubbles.
The next appointment was to do a palate study which involves a video x-ray of E “speaking” to see how the palate moves or doesn’t move. That was scheduled for January of 2015. The hope was that she’d have several sounds conquered before then by working with Tammi but if she didn't we’d at least get a clear picture of how her palate is working or is not working. The study went well after we were able to calm E down. The machines really freaked her out at first. We found that her palate was not closing very well, which is something that is needed to keep air escaping out of your nose when you say certain sounds. The Dr felt like she needed another surgery to lengthen the palate and give it at least a better chance of being able to close better. That was scheduled for May 2015.
The palate lengthening procedure went well. She was in the hospital for one night and then sent home. This time around the stitches were mostly concentrated in the center of the back of her palate and down to her uvula, which also got sewn together after splitting apart after the last surgery. She is all healed up now and did really well with the soft food diet for 6 weeks. We haven’t noticed a huge difference in her speech or abilities yet but the Dr said it can take up to 6 months for the healing process to be complete and that progress will be slow. The important thing right now is to keep working and get those muscles moving. They haven’t been used in the proper places so it’s gonna take some time for E to figure out how to use them properly. Every once in a while she’ll say a perfect ‘b’ sound at speech therapy, usually when she’s distracted and not really concentrating on the speech itself, and surprise the heck out of me and Tammi. We usually can’t get her to repeat it but that’s ok because at least we know she’s capable of it and we’ll just keep working towards consistency. We have another palate study (video x-ray) scheduled for October of 2015. Hopefully that will show an improvement in the palate function. If not, another surgery may be in her future. . .
At the time of the palate revision I asked for an ENT to check her ear tube and do another ABR test since we only got results on the left side during the first ABR. The ENT we saw was really against doing the ABR and had talked me out of it at the pre-op visit after doing some hearing testing at their facility at that time. After I got home though and started going through the test results of previous hearing tests I realized that this pre-op testing had only muddied the waters more and called them back to tell them we WOULD be doing the ABR after all. I was not going to take no for an answer. The only way to get this done was to have her out under general anesthesia and I wasn’t about to do that at a separate time when we had the perfect opportunity only days away. Thankfully the audiologists agreed to do it even though the ENT was never on board with the idea. And, as it turns out the ABR came back saying the exact opposite of what the pre-op tests had told the ENT so I’m glad I pushed to get it done. The result for the left side were the same as the previous ABR, which is almost completely normal hearing. The problem there is mostly the narrow canal which doesn’t allow the sound in as efficiently as it would in a normal sized canal. The right side showed hearing loss but that the ear organs were functional. The hearing loss on that side is due to the atresia or not having an ear canal at all. So we are on the right track with the BAHA and we’ll continue to use that. She is not a good candidate for a hearing aid not the left side. The BAHA is sufficient for both sides right now. Overall, she is considered to have a moderate to moderately severe hearing loss. She also got another tube put in the left ear and the previous one was nowhere to be found. I’m not sure how it even fell out considering the narrowness of her ear canal but it wasn’t there.
Like I said before, Tammi specializes in helping kids with hearing deficits so she has also been teaching E a lot of sign language (which I’m learning as well since I’m with her at all of her appointments). E remembers the signs and is eager to use them. It amazes me at how much she remembers sometimes! She is trying very hard and is usually very willing to go along with whatever we ask her to do speech-wise. I know she definitely wants to communicate. She has already made so much progress and is now making ‘p’ and ’t' sounds pretty regularly along with correct ‘m’ and ’n’ sounds most of the time (those are usually really hard ones for kids with hearing deficits because they sound so similar) and trying many others.
We’ve also been working really hard on getting her to make choices. That has been a struggle from day one. If you asked her what she wanted to eat and gave her a couple of choices she will only repeat the question you had asked her. She would do this for pretty much everything you said to her. We’re starting to see her make some choices though and that is wonderful. I’m not sure they are really the choices she wants to make yet since she usually says she wants the item I’ve suggested last, but at least she’s not repeating the whole question back to me. We hope, in time, that she will be able to figure this out. This is such a huge part of each person and their personality that it’s hard to watch her NOT do these things. It’s kind of disturbing and makes me wonder if there is another underlying problem we haven’t found yet. She is just so behind developmentally that it’s hard to know if it’s a delay thing or a deficit thing. Only time will tell. That’s pretty much become our motto with her.
She also had her 4-year-old well check in October of 2014 and here are her stats:
Height: 36 inches (1 year ago: 34 inches)
Weight: 28 lbs. (1 year ago: 23 lbs)
She has been followed by a nephrologist as well after finding evidence of hydronephrosis in December of 2013. It seems to have resolved itself completely and will have one more followup with lab work and an ultrasound in June of 2016. If everything remains clear we won’t need to see him again. Yay! One less Dr in our future!
