Happy Valentine's Day!  It has been an exciting day for the Bauer's.  Dallas has been wearing one hearing aid for 2 weeks now (yes, we lost one) and this morning we are finally back to two.  He also got new ear molds, so the aids are fitting nice and snug again.  They did a sound booth test to see if Dallas would turn his head towards sound, and he DID turn his head at 65 decibels (which is a slightly higher volume that normal conversation).  He did not respond to lower sounds, but they aren't sure if he got bored with the test or if he truly couldn't hear the sounds.  Either way, we were all pleased to see him respond to the first set.  This basically tells us that if hearing aids can get him hearing at levels just above conversation, cochlear implants should get him one better.  Lots of "if's" with the cochlear implants, but this is a start.

Last week, Dallas got a helmet to correct his odd head shape.  Since he is still not sitting up, he spends a fair amount of time on the back of his head which doesn't allow for normal growth.  He is tolerating the helmet really well so far.  I think the worst part for him is that it makes him too warm.  He runs warm anyway, so this just brings on the sweat.  We will have to get some lighter clothes on him!  This process could take 5-6 months and he wears the helmet 23 hours per day.

Dakota started a mini-preschool class 2 weeks ago and she is loving her time there.  It is only one day a week for 1.5 hours, but I think it is just what the doctor ordered for all of us.  Dallas gets to spend some time with some new faces as well and I am becoming very educated with the other moms as we discuss child raising topics. :) 

Scott and I started sign language classes last Monday and we both left there knowing the alphabet.  We were excited about that and can ...very slowly...spell words to each other.  The instructor is wonderful and an inspiring person to boot.




Got Camo?






Dallas had a wonderful first Christmas.  Aside from a recent ear infection, he has been a happy and healthy little guy both throughout the holidays and over the last month.  He continues to eat like a normal healthy kid and has not required any feeds through his g-tube.  He is cruising through his clothes like crazy, I put a pants outfit on today and they looked like shorts...he's a weed and it's great.

We have recently stepped up the therapy to try and get his motor/muscle skills closer to where they should be.  He lacks in muscle tone and core strength.  His trunk muscles are floppy and he is still a far cry from being able to sit up on his own.  These therapy sessions are some of the only times I see Dallas upset.  He does not like to work on his muscles!  We'll get there.

Christmas Morning



Umm, is that a swim shirt?



Ok, I am ready to get out now.





Dakota "Cheese, now give me a sucker."




Why doesn't anyone else in this house have to be propped up on a noodle?



You are so funny mom!

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Dallas continues to keep us on our toes as we have been at Children’s for 3 days.  I brought him in on Monday morning around 1am because his heart was racing and he was breathing fast.  When we arrived in the ER, he had a temp of 103.3 and his heart rate was consistently over 200.  This happens when babies get fevers I guess!  He was diagnosed with pneumonia within an hour of arriving at the hospital.  It was visible on his chest x-ray and lab tests showed his body was compromised by a virus/ bacterial infection or both.  Dallas did a heck of a job fighting this off, and it helped that he had great care.  They put him on both oxygen and HiFlo and this helped slow his breathing down.  He earned a reputation of being a little troublemaker after he managed to screw up two different IV’s that were placed in his head.  They gave up after the 2^nd one, and decided he could get meds through his g-tube.  He loves to pull on anything that doesn’t normally belong to him or on him. J  We just got home a few hours ago and Dallas is incredibly happy!  He does not stop talking and smiling.  Other than some antibiotics for the next week, it’s back to normal for Dallas.

In other news, sounds like I am hosting a broadcast television show, we had the "Cochlear Implant panel" last Thursday at the University. This day did not bring much new information, nor was there specific good/bad news. We discussed the device itself, the complex nature of the surgery, and the extensive therapy required afterward. The plan is to have a repeat CT scan in March and possibly schedule the surgery for one ear sometime in late spring, early summer. Dallas's surgeon expressed great concern regarding the potential success of the implant, but did not discourage us from thinking this could be his best shot at hearing.

Dallas has not had a feeding through his G-tube since December 6th. After 10 days of only oral feeds, he had gained 10oz! We are not sure where he is at now, but he will be weighed again in 2 weeks to make sure he continues to gain weight appropriately without any G-tube feeds. He certainly is getting that chubby cheeked, chunky leg, 6-month old baby look! We love it.

Here's a picutre of Dallas right after he pulled out his 2nd IV.  Looks pretty guilty!

The time just flies by, Thanksgiving is over and Christmas is right around the corner.  Dallas has remained healthy through “Round 1” of the holidays, although his sister managed to catch quite a cold.  She has been pretty good at keeping it to herself so far!

Dallas had his 3-week post-op appointment this past Tuesday and his nasal passages have remained open.  This same surgeon that has done all of his airway surgeries, is the same person that has been discussing cochlear implants with us as well.  Up until this appointment, he has expressed guarded optimism (but optimism nonetheless) relative to the surgery.  He has always felt that he would try one ear and see how it went.  Now he is feeling like Dallas may not have the anatomy for this surgery.  He previously said no further CT scans would be needed, but is now mandating another CT before the surgery.  Additionally, this was always planned for 9 months of age, approximately March, and is now being pushed out until Dallas turns 1.  In the end, we certainly don’t want the surgery to take place if there is not a good chance of it being successful, but it was a little disheartening listening to this new found pessimism.  In any event, we are having a group meeting in 2 weeks to discuss the implants further.  The implants have to be approved by a state medical board of some sort and the approval takes 3 months.  A repeat CT scan is being planned for late February.

Last Thursday night we stayed at Children’s for a follow-up sleep study. This was to confirm that Dallas’s oxygen saturations were adequate without air support at night.  GOOD NEWS!  He PASSED!  Of the 9 hours he slept, he only had 14 minutes where his saturations were below the acceptable range.  So, he is officially off air at night for now.  We continue to monitor his levels every night, so that if there is a change we can address it.

Dallas is eating well!  I would say he eats 80% of his food orally, and the other 20% via G-tube.  He has another swallow study next week to see if he can now tolerate thinner liquids.  As of now, he still drinks his bottles at a “honey-thick” consistency.  He weighed 15 lbs this past Friday.

We are still keeping very busy.  We have 2 appointments next week and 3 the following week.   Also next week, I am getting the opportunity to meet two other mom’s that have sons with CHARGE syndrome.  It makes me a little nervous, but I know it will be good to meet other parents who have been through this before.

We are almost 2 weeks post-op and Dallas is doing much better than last week.  It usually takes 2-3 weeks for him to get back to himself relative to sleeping and eating.  He is almost back up to what he was eating orally, and is sleeping comfortably again.  He is such a happy, delightful baby.  You can reliably make him smile or laugh by doing “SOOO BIG,” or by tapping his hands or feet together.  He loves taking baths and getting his diaper changed!  That’s my boy!

Last week, he had his first eye appointment.  I braced myself expecting the worsed, the doctor would for sure tell me he was near blind I was certain.  Thankfully, the optometrist was “unimpressed” by Dallas’s coloboma’s and stated how small they were.  He indicated very little impact they would have on his vision.  He does have stigmatisms in both eyes, which could cause some blurry vision, but this is treatable with glasses down the road if the stigmatisms don’t change by age 1.  What a relief.  I told the optometrist that this was the best appointment we’ve had in 5 months, and he said he was glad he could give me good news, but he seemed disappointed that Dallas’s wouldn’t be a good “case” for him.  C’mon people, seriously.

We heard back on the heart CAT scan which showed that the narrowing was not significant enough to warrant surgery at this time and we’ll continue to have ECHO’s every 6 months to keep an eye on it.  More good news!

We finally received the genetic confirmation that Dallas does in fact have the CHD7 gene mutation that is found in 75% of CHARGE kids.  This doesn’t change anything or tell us anymore that we know already, but it does mean Dallas would have a 50% chance of having a child with CHARGE because he carries the gene. L  The geneticist explained the mutation simply like this; the gene basically stops doing its job early.  Because this gene is required in so many organ systems, these kids are so widely affected when the gene doesn’t complete its’ job.  Explains why many of Dallas's organs were incomplete or are still  incomplete....ears, kidneys, heart, etc.

Yesterday Dallas got his 3^rd set of ear molds (that attach to his hearing aids).  These are the best fit thus far and they aren’t constantly squealing!  The bad part is that he is getting old enough now where he realizes they are on his ears, so he likes to pull them out!  Hence the cheesy hat in the pic below!  It’s not our goal to make him look like little bo peep, but you do what you gotta do.

We are looking forward to showing him off at the Thanksgiving festivities; we have lots of family that has yet to see Dallas in person!  However, you’re always thinking about the germs so our goal is to keep him and his sister healthy throughout the holidays.

Oh, I can’t believe I almost forgot this….he has still been maintaining his stats at night and not requiring air support.  We will be more excited about this once we can prove it to his pulmonologist.  He has a repeat sleep study on December 2^nd.

Surgery days are always difficult, and they only get more difficult each time.  It gets harder on Dallas and certainly harder on us as well.  The older he gets the more aware he is of everything going on around him.  He is scared and confused and it's hard to see him like that.   The morning started with no food of course, and he tolerated that very well.  His surgery started an hour late and it was 3 hours until we heard word that he was recovering.

The recovery period was almost as long as the surgery and Dallas was very unhappy.  He was given 3 doses of a pain medication called "Fentanyl;" none of which gave him any relief.  They then tried a dose of Tylenol before giving him some Morphine.  Finally, after a solid hour or more of crying...he started to calm down.  Then his breathing was concerning and he was very red.  We pushed for G-tube supplies so that we could get some food in his stomach and after a good effort from the post-op group (feeding is uncommon in this area), we were able to get him fed and then he was really out! 

Results:

CT Angio - No results for 2 days.  This was a cat scan of his coartation repair of his heart.

ABR (Auditory Brainstem Response) Hearing Test - Confirmed previous test results, showing Severe to Profound Hearing Loss..(bummer, you also silently hope that a retest will surprise you in a good way)

Ears - Replaced Tubes

Nose - This is confusing to me, but the way I understood it was that the nasal passages towards the front of his nose(where the previous dilation's have taken place) were open and did not need dilation.  However(and there always seems to be a "however"), they found a narrowing near the back of his throat (or bottom of his nasal passages).  This is not good, but not the worst finding either.  They ballooned the narrowed spot which made the opening 25-30% bigger, but are very restricted here by bone and this area is very close to spinal cord nerves making it difficult to do too much.  The surgeon thought 2-3 more of this type of dilation would be needed and no indication as to how soon the next one would be.  We will follow-up with him in 3 weeks.  We discussed different possibilities and potential outcomes related to this new finding, but for now it's best left as we just wait and see how he does.

We are settled into our room now and Dallas has low blood pressures.  They have paged a DR, but hopefully this is just part of him being so zonked out from his big day.

Given an uneventful night we should be going home in the morning.

I've included a pic from a happier day!

Dallas gained another pound!  He is 13lbs 10oz.  He also has had big improvements in his breathing.  Since the last post on Oct.22nd, he has not required air support at night.  I did my own little trial and found out that he did fine without the air.  We discussed this with his pediatrician this week and a lab draw confirmed what we had hoped!  He will still have the air ready for him if/when he should need it.  After surgery this coming Monday, they want him back on it until all the inflammation goes down.

We continue to work with audiology on his hearing aids.  They are a challenge given their large size and high level of sound on his little ears.

Halloween - Dallas had a great first Halloween!  He slept through all of the trick or treaters, but he was festive earlier in the day.  His big sister Dakota LOVED trick or treating this year.  It was so fun to see her go door to door.  Dallas and Dakota enjoyed the day with their cousins as well.