Elias' first word

You have to listen close as it is right at the beginning and quiet, but his Aural Rehab therapist has confirmed and acknowledged this is/was his first officially spoken word.....the word is BABY. It is proof that progress is slowly but surely being made and while it in no way suggests that Elias will be "talking" anytime soon, this is a reassuring sign

our persistence and aggressive therapy is paying off 

It is becoming more and more challenging to keep this blog updated. It seems as of late the schedule has been so hectic along with "stuff" (like Elias not breathing for example) just hitting us with the old 1-2! The fall isn't shaping up to be any easier, in fact it will be more nuts with the addition a preschool services here at home. We have been exploring other possibilities that might be easier, for instant - audio updates. If anyone has any suggestions we are open.

Since the last update Elias has had several more clusters of breathing episodes. Usually on the weekends it seems. It is as if he knows! Last weekend they began Friday at 4:30 and continued through Sunday Morning. So he has now had periods where he stops breathing 4 out of the past 6 weeks. This is the most frequent we have seen them in quite a while. All we can do is manage and try to keep the stress to a minimum during those times.

Elias has had a few sessions with his new speech therapist. It is a growing process and will take some time for them to learn to work with each other, but there has been some positive things already. We are working to expand his vocabulary with the picture exchange. At times the little stinker will turn it into a game of keep away as he smirks at you. He is a joker so watch out. In his listening therapy yesterday we witnessed something very interesting. She had a plane and a train. She showed and played with each individually with him. With airplane she would make an 'ah' sound before showing the plane. Then in the same manner did a Choo Choo sound with the train. She then presented both but rather than ask which one he wanted she made the ah sound, in hopes he would choose the airplane, and he did. We were not convinced fully that this was not merely a coincidence, so she did it again. Same result. So to try and substantiate what we saw she presented the two again, but this time made the Choo Choo sound. Elias just looked at her and did not do anything. She made the sounds again. After a few times he finally reached begrudgingly and touched the train, as if to say okay okay there is the train can we move on. So we are going to try that experiment over the next few weeks to prove it was not an accident. That he is beginning, ever so slowly to recognize even more sounds and correlated them to the appropriate item. If he continues to prove this theory correct it would be a HUGE step. So here is to praying it was real and effective.

Another issue keeping us from the blog updates is constantly having to chase down and follow-up on things that we are told have been done or should be done by various providers. One example, we are having issues once again with Elias' prevacid an the insurance needing prior authorization. The problem hasn't been so much getting the authorization in as it has been getting to the right person. This is due to the incompetence of the pharmacy providing us with the wrong information on what was needed, not once, not twice, but three times in the past 10 days. On top of that they have contradicted themselves in the information proving that at times they were just saying whatever to placate us and move us on for the moment. Ultimately this has cost them our business and frustrated the doctors office handling the PA, as they caused them 3 times as much work, most of which was not necessary. The part that truly angered us was not only the deception, but the lack of concern or willingness to ensure the information was communicated in a timely manner, as this medicine for Elias is potentially life or death. Why? One of the theories behind the breathing episodes is that reflux temporarily paralyzes the vocal chords, which creates a reaction causing him to stop breathing. He did have a cluster of episodes in the 10 days he has been without. Hard to say for sure, but if that is a physicians theory on the table for the unexplained along with very few others, you tend to take them serious, despite what you believe. Fortunately this morning we have filled the Prevacid and he will be receiving this once again.

This past weekend Katharine's mom and brother came to town for a visit. They will be with us for a week. Katharine's Aunt, Grandmother, and cousin flew up on Friday to surprise everyone. They were here until Sunday. It was a fun time surrounded by family. Elias was having such a blast. He barely stopped the whole weekend. He did have a blip on Saturday night, and was not doing so well, but bounced back by morning. Just reminding us that he is still medically complex is all. He showed off and demonstrated all the wonderful skills he is learning. The bubbles with the picture exchange for communication was by far the crowd favorite. There are some photos that we will work on putting up soon. We will update when we can and who knows maybe we will figure out an easier way. As we said we are open to suggestions if anyone has any.

Creating Total Communication for Elias!

As you know we have been working with Elias to develop a total communication system because of his hearing loss and limited response to amplification. It has been quite a process that has involved 3 audiologists, 2 speech therapists, 2 occupational therapists, 1 assistive technology therapist, 1 aural rehab specialist and 2 doctors –ENT & Developmental Pediatrician. All of these 11 individuals had a different role necessary to create a system that will enable Elias to effectively communicate through various means and methods. It has taken more than a year with this enormous team to finally achieve some success.

What is total communication? This is a system that incorporates various modes of communication. Sign Language, auditory Language, picture exchange, and assistive technology –use of computers, switches & other various devices. We have been steadily working on each of these components to develop Elias’ total communication process. At times this process has been frustrating for all involved, including Elias! With so many hands in the cookie jar and nothing to facilitate bringing them all together there were differing interpretations and implementations of strategies. Some of these were contradictory and went undetected for some time. Reflecting back that has had both positive and negative effects. The positive effects were that tools were given to Elias, but at potentially inappropriate times. Putting the cart before the horse is you will. It was prudent and appropriate when focused on isolated roles, but putting the big picture together it did not always fit. Eventually, these tools would become appropriate. It was just making him understand that he already knew this, it was just being used in a different context from what he was used to. While this is seen in many areas of development with communication it was making things harder for us often times confusing him to a point we would have to regress to build it back in appropriately.

Avoiding the Plague

 

It’s been a week since our last update and honestly not too much has been going on. We have been laying low trying to avoid a very bad bug – we refer to as the plague – that is going around the area. It has been so rampant that at least one entire school was closed because so many of the students, teachers, & staff were out sick. One of Elias therapist was telling us that most people that have been stricken with it have been ill for nearly 3 weeks. Something that intense is VERY bad news for Elias! Of course this is the week he finally decides to get a mundane issue – pink eye. The first time every where going to his pediatrician would be valid and just. The timing just sucked with all the sickness there. Thankfully, we avoided a visit and he just called in a prescription for eye drops. The only other point of interest is that Elias received his own BAHA on Thursday.

A Hearing Moment

Yesterday we had our weekly Aural Rehab Therapy. There has been some excellent progress made, especially noticeable since the settings were altered – something we had questioned for awhile. The therapist had Elias sitting in a high chair and playing with various kids musical instruments. Elias was very engaging and interactive – especially beating on a drum! As Elias plays the therapist would make various sounds - The Ling 6 sounds in addition to what I call a throat trill – a high pitched rolling sound. The Ling 6 sounds are particular sounds that occur at particular speech frequencies or pitches. She would make them randomly looking for auditory response. Over the past 8 months there have been lots of, “was that a response?” Never that definitive ah-ha moment that everyone we have spoken to over the past several years has mentioned.

A Full Week

It is the end of another busy week, exhausting week. We had appointments everyday this week (some days multiple), except for Friday. Most of them

were various therapy appointments, with IVIG and some research lab work thrown in the mix. Health-wise Elias is doing well for the most part. We just finished another 3 week course of vancomycin (second course in 6-1/2 weeks) on Monday for C-diff and already we are seeing the beginning symptoms of a flare up emerging. I imagine Monday morning we will be calling GI for the next plan to try and beat out this very chronic issue. There has been some conversation between Elias GI doctor, His BMT doctor and the chief doctor of infectious disease on alternative treatments. Probiotics have been mentioned in the past, but BMT would rather try other options first. We do not fully understand the reason behind the reluctance, but she is a VERY good FA doctor that we trust explicitly. She is not 100% opposed, but would rather not jump right in if there is an alternative, which apparently does exist. So we will place this in their very capable hands and see what comes next.

Observing His Play

All is well here. We have been bogged down by snow and now the bombardment of rescheduled appointments. We are working on another MRI for Neurology. We had one in September and the doctor wants a follow-up. The exceptional nurse coordinators at the FA clinic are working their magic again with Our ENT – focused on his hearing – in coordinating to do the FMRI we mentioned last October with this upcoming MRI. Especially with the breakthrough we seem to be having with his hearing, it will be very interesting to see just what is happening inside that little boys brain.

A Must Read Update!

This is certainly one post you do not want to miss! We have some information to share about Elias! Yesterday we had a marathon day of appointments at Children’s. One of those with Audiology. Our Aural Rehab Therapist helped us to get an appointment to re-evaluate Elias’ BAHA settings and to conduct a standard hearing test to see if there is any change in response.
The original audiologist that fitted Elias with the BAHA initial is no longer at Children’s, she moved – can we get a big AMEN! You may remember our issues and push-back we received from her when we questioned his lack of response. She was even rude to our speech therapist, telling her that she needed to know her role and stay within it. We have been trying to meet with the new audiologist that took over the BAHA segment. The past few months have not allowed for that until yesterday. We have added him to the team and he offered some interesting perspective. He really took the time to explain things as well as encouraged our questions. In fact he was very patient and prodding for more once we were done. We appreciated that very much. We started with a standard booth test. Here is a very controlled environment for ambient noise, frequency, volume etc – so nothing like the world environment. They placed a bone conduction device that was attached to some wires – allowing more control for testing and response purposes. Elias’ BAHA was not used during this testing.

Babble – Music to the ears

Elias has been doing well overall. His days this week have been filled with walking walking and more walking. We hope everyone had a chance to view the video from this past Saturday. That was a VERY good day for him. Probably the best he has felt in quite some time. He is enjoying going to the backdoor in the kitchen and looking out into the backyard. He figured this out a few days ago. Yesterday, while it was snowing he was just standing there watching the snowflakes fall. You could tell he was intrigued, but not sure what to make of it all.

ENT Hearing Update

 

We met with Elias ear focused ENT this morning. After discussing how Aural Rehab is going and the BAHA hearing aid he is in agreement that there is still little to no response. He is still leaning heavily towards a Central Hearing loss and wants to try and coordinate a research functional brain scan during Elias MRI next week. This new scan colors the portion of your brain than is functioning. While it won’t be diagnostic it will be interesting to see how the auditory portions of the brain respond. If nothing else it can show that everything is intact or if there is an area that the channel is “broken.” We agreed to do this because it will only take 10-15 minutes more during his MRI. The doctor also gave us some positive news overall though. He mentioned that children who are profoundly deaf will babble, but then go silent. Elias is increasing his babbling. While that does not mean he is hearing, it supports the central hearing loss theory. He said that he has high hopes for Elias and his ability to communicate and one day hear. It just may take many years. But we are on the right track and he feels like everything we are doing is exactly what Elias needs. That certainly made us feel a bit more positive about a subject that has plagued us for over 2 years. We will take even the tiniest bit of positive input for this and make it huge.

Please pray for FA patients around the world. There are several families who are really struggling with transplant related issues, cancers, and sadly loss of life. Particularly the Boggs family who’s son Nicholas is at home on hospice. This week has been rough on the FAmily. There have been several somber reminders of how unpredictable and wide range this disease be to harm its patients. Thank you all for your continued support, thoughts and prayers.

Hearing & G.I. Updates

Yesterday was another Aural Rehab Therapy session. Elias was not having anything to do with the BAHA. He was throwing a major tantrum when it was on. We are still trying to decipher why it is he will get that way at times when it is put on. Most of the time he is carefree about wearing it. To call it an intolerance is exaggerating. One thing the therapist brought up was that maybe the settings  needed to be checked. She wants us to set-up an appointment with the audiologist that is now doing the BAHA cases. Specifically to test him in the booth and address that the settings for this are indeed the right ones. I became quite upset over this. They are now suggesting something we brought up multiple times to multiple people and were always dismissed as that not being a potential problem. Now it is? The inconsistency is annoying me to the core. So we are trying to fit that into our already crazy appointment schedule....

Aural Rehabilitation Therapy

Last week was our initial Aural Rehab Therapy (AR). It was more of a goal setting and determination session more than an active therapy. Which was actually a positive because Elias was not having the best of days for some reason. His therapist has been doing this for many years and actually helped start the AR program at Children’s. So her experience will be of value.