a few words

I must admit, life it getting in the way on my blogging. This could be a good thing. Life you know is good! I have been feeling a need to write lately. In fact, the idea has come up a time or two to start writing seriously. Become a true blogger or story writer- I find it quite therapeutic to write. Its something I have been slacking on lately and I feel obligated to some degree to share our adventures. Mostly our adventures with Sophia. She, in fact, is our whole life.

Where to start... Well last I updated we had just transitioned over to Cincinnati Children's to start a new medical journey with Sophia. I am happy to report, we in fact made the right decision to switch hospitals for Sophia. The aero team and CHARGE clinic are without better words to say it- AMAZING. These doctors truly know their stuff. Its rewarding to walk into a place and know they have experience and to know they really have Sophia's best interests at heart. The most amazing thing to me about this group of doctors is that they are a true team. They communicate and share updates and ideas with each other weekly. They do not make any decisions or moves without consulting the TEAM first. It truly makes a significant different when everyone is on the same page; especially when dealing with such medically complex children.

This summer we spent a good month in Cincinnati. Sophia had two major surgeries. One to remove her tonsils and check her airway. We had to stay put for 10 days. We were so so nervous about bleeding and aspiration risks from the tonsil removal. My devote husband didn't close his eyes the night before her surgery. The man loves that little girl. We spent one and half days in the hospital and then the rest of the 10 days at a family friends house recovering. We were told she would be just miserable for about a week. Day two/three of recovery and I am fighting to keep the kid on the couch. Sophia is without a doubt the most resilient child I have ever come across. So for the next 8 or so days, we stayed put and really enjoyed some nice and quiet family time together. Its ironically heartwarming how a major surgery can cause the world to slow down and for ones focus to shift.

We had a 6 week turn around before Sophia's next surgery in July. This is the surgery I was threading the most. My husband and I are perfect opposites. Where he freaks, I am strong. Where I worry, he never wavers. We balance greatly between sanity and loosing our $%*T on most days. We pick each other up and that is what matters most. July's surgery is were Sophia had stents placed in her nose for 6 additional weeks. The ENT team went in and basically gave her a nose job. They removed tissues, chiseled away bone and placed plastic tubes to give Sophia an open nasal passageway. For the first few weeks, her nose was a bloody mess and Tylenol stayed on rotation. We even got some pretty gnarly nose booges out of her nose. I mean I am talking gooey, spaghetti noodle long wads of just plan goodness. I was quite satisfied when I was able to really grab a good one! Though in all seriousness the stents did added a little extra worry. Our biggest concerns were her falling or bumping them. I knew it would be painful for her and I was so afraid it would cause more damage. Luckily my fear of them going into her brain were put to bed by the fact they were looped in and out of the same holes. Plus constant suction through the stents increased the work load. It was work. But like anything, time healed. Her nose didn't look so bloody, suctioning became habit and she never fell on them. Relief. Though when that 6 week deadline arrived, I could not have been more thrilled to have them taken out. Bonus- her cute little nose stayed cute!

However, what transpired from these surgeries this summer, I would have never predicted. The goal was to open Sophia's airways. To give her the best open upper airway we could to maybe give her a fighting chance to get the trach out. One week post-op from her stent surgery and I could already start to notice air sounds coming up and out her mouth and nose. It was quite interesting to hear. I kept my cool. It was a few days before the return of the school year when we got our big surprise....actual VOCAL SOUNDS!! You heard me folks! Sophia is NOW making vocal sounds. She has never made true vocal sounds!! Ever! For 4 years, we have asked ourselves if we will ever hear her speak or say our names. And in good Sophia fashion- she blew us away. She just did it! She started making true vocal noise and surprised us all. Including herself! She was so proud. Now, Sophia has always had a great vocabulary. She can sign hundreds of words and learned them so quickly. She has also mouthed words for a very long time. She even invented this clicking sound she does to make sound for syllables.  But for her to just naturally start to try to say actual words---FLOORED. Literally, it went from a few noise sounds to one day we walked in and she was trying to say "I LOVE YOU" to me. Instant sobs. I couldn't believe my ears! It was the most beautiful sounds in the world. Now to clarify, she is not speaking audible as you and I speak and she is still primarily a signer. But to me and her daddy and her nurse and family- its plain as day what she is trying to verbally say to us. It gets clearer daily. She works so hard. Now granted, she is still speaking through a trach so it sounds muffled. Like shes talking underwater BUT folks she is learning to TALK!! A worry, a want, a fear and a pray all rolled into one came true-- The sound of a sweet sweet girl saying mommy for the first time! Now I am the one without words.

Sophia has been trying to make vocal sounds for a few weeks now. I instantly wanted to share with the world, but we decided to sit on this little secret for a while. We wanted to keep it to ourselves. We wanted to rejoice in this beautiful noise. We wanted to make sure it was going to stick around. We our cautious these days. We now oh to often how things can change. We also wanted to steer clear of questions and feedback for a while. We did not want anything or anyone to lessen our experiences with this wonderful news. We also wanted to be sensitive to Sophia. We did not want to get to excited and at all make Sophia feel pressured to do something more than she was ready to do or to make her feel like we were missing something from her before. We still encourage Sophia to sign and learn new signs. Sign language will also be Sophia's first and maybe her strongest language and for this we will always be proud. Now that we have truly processed our feelings and allowed ourselves times to celebrate these new steps forward. We couldn't resist sharing her wonderful news with you any longer.

I guess it really is just something you have to hear for yourselves...

One year ago... "Hi Mommy video 1" https://youtu.be/DINA5tYYiuo

One week ago... "Hi Mommy"https://youtu.be/fUAGfHufEs

I hope you get goosebumps, I hope you understand the significance, I hope you celebrate with us. I just have so much hope and pride for this little girl.  My little girl.

So even though we struggled and had reservations about Sophia's surgeries this summer, we now know the doctors are truly on the right track. We have been going back every 2 weeks for the last month to keep checking her airway. So far we have had great results. Next steps to be determined soon....

What a week

What a week we had in Cincinnati. So much so that I need a week to recoup. Well as I had mentioned, Sophia was going to Cincinnati to have some diagnostic testing done for the A-team. We started the week with a trip to the OR. She was being put to sleep to have all three doctors evaluate her airway. In their words, they've learned that each doctor sees differently and each knows their field particularly. Also, while under they were going to possibly dilate her nasal passage and put a camera up through her nose and into the stomach. This was to be left for 24hrs under hosptial observation. Well, once in the OR and the doctors started to look around they realized that one, the dialation would not be beneficial for Sophia because her airway in her nose had reduced so much they couldn't pass anything of the left side and the right side was tight. Big problem. Dilating simply wouldn't do. Secondly, They also deduced her tonsils and adnoid tissue was so large that on a scale of 1-4, they rated it a 5! This extra tissue as well as the nasal passage prevented the doctors from being able to pass the camera for observation. The only perk meant we didn't need to stay over night in the hospital. The down fall.. Two more surgeries. Can we ever catch a break? On the whole, the doctors felt that from trachea down her airway looked good and promising. The problem is all upper airway. The next morning we were back at it at 7am for another round of evaluations and test. This time it was a video study in which they put a small camera up Sophia's nose and down into the back of her throat. Not pleasant for most people as I'm  sure you can imagine. Sophia was a trooper to say the least. However, again this procedure was cut short due to a copious amount of secretions blocking visibility as well as the tightness of her airway made  camera angels difficult. The doctors were beyond impressed with Sophia's patience and tenacity. The only perk of these appointments being cut short was we were able to take Sophia to the aquarium. She totally deserved it. The best part was Sophia was selected to be the penguin grand Marshall! She led the penguin parade and got to go with daddy to met the penguins on her own. Totally precious! I will adore that memory forever! The next day we were back again for a couple of swallow study appts. These appts went as predicted. Still aspiration. Still no food by mouth. The next day we had the afternoon before Sophia's overnight sleep study, so treat number two- the zoo! It's sad when a week at the hospital is kinda like vacation. A little hospital, a little fun. It's needed. One would be bitter without a little laughter and fun mixed into all the medical jargon and procedures. Overall, I am still pleasantly pleased and hopeful with cinci. I think we've made the right decision in switching our care to a hospital that has a focus and speciality in Sophia's needs. We just now have to put our faith in their knowledge and decisions to help Sophia continue to grow and strive. As on now, we are in the waiting process before we schedule the next surgery. Until then, my big girl is starting preschool. I know it seems like an off time to start, but we and Sophia live by our own agenda :)

a new journey

I can't believe I haven't written in soooo long. I guess in a way that is a good thing because that means I do not have anything to report. For the past several months, we have been in a holding pattern. I talked last about Sophia possibly needing tonsil surgery and we had received mixed consults from various doctors. This left us confused and unsure as to what  next steps should be taken. With one doctor telling us no and another telling us yes and another doctor we weren't comfortable left, it left us twisted. My husband is huge on research. So he did his. He discovered that Cincinnati Children's Hospital was possibly starting a CHARGE syndrome clinic amongst also reading studies from some prestigious doctors from cincy. So we consulted one more time with our pulmonary doctor at Riley (back in October) and she confirmed that Cincinnati Children's would be a smart move for Sophia. She spoke highly about a program called the Aerodigestive team. The A-team we are calling it. Believe it or not, but it consists of a team of doctors working TOGETHER. Communicating together. What the What? The team is made up of ENT, pulmonary, and GI doctors that specialize in their areas. That in itself had us convinced to make the switch. I have spent months with insurance, communicating with doctors, filling out paperwork, and so many phone calls to try and get us our initial appointments with the A-team. Finally, in January I received a call the insurance was pending. A few more weeks later-approved. Finally, last month (after several more phone calls)- a schedule. I owe a huge thank you to my husband for encouraging us to pursue the switch!

Last week, we made our first trip to Cincinnati Children's Hospital. The first day was a formality really. Sophia met with anethesia first to go over her history before taking her into any ORs. Then the girl had an ECHO and EKG. Poor thing wasn't allowed to eat all day because normally they put small children to sleep during these procedures because they can't sit still. Not Sophia. She's a pro and didn't require it. Lastly. we met with the cardiology doctor to confirm she in fact has a bicuspid aortic valve (we already knew this) that was mild and she was clear for the OR. Day 1 of 7 complete. The next morning we were back again at 8 am for a sedated chest CT scan. This went well. A little upset with some cold /procedure-oriented bedside manner but nothing to get excited about. Sophia went right to sleep with a giggle and giving mommy a tickle:) She came out grumpy. We fed her and then walking down the hallway she lost her fed..ick. I have never been anywhere were people are so eager and willing to help you clean up vomit. I literally had two hands over my shoulders handing me napkins instantly and people throwing down puke pads. Poor girl. But do not fear because Miss Sophia was monkeying around a mere thirty minutes later. After some time to kill, we went to meet our first A-team team-member, ENT. Dr. Choo had me at hello so to say. He walked in an shook John's hand, my hand, and without a beat Sophia reached out and shook his hand. He was impressed. In fact on more than one occasion, he repeated how great she looked and how smart she seemed. What got me, literally crying was when he mentioned is end goal is communication and how he loves when his patients come back years later and say "Hi, Dr. Choo". Oh, how we long for that day. Its emotional. Sophia communicated fantastically with sign but to hear her will make me speechless.
What sealed the deal for my husband was the fact that even though clinically her tonsils looks like they need to be removed. He is a totally picture guy. He wants to know how everything is functioning before he makes any decisions to remove her tonsils and is aware it is in fact a major surgery.  Especially with complicated children like Sophia. The best part though is that Dr. Choo asked us to be apart of the CHARGE syndrome clinic. This means we will have a person contact us regularly to set up all of Sophia's appointments and we will meet them all on the same day. More over- THEY ALL SPECIALIZE IN CHARGE syndrome and research. Worth its weight in gold. Victory.

After the meet and greet with Dr. Choo we went on to meet the pulmonary partner in our A-team. Dr. Wood. He is a seasoned doctor who believes his patients are guilty until proven innocent. He end game is to make his patients tax payers. Dr. Wood went over the chest CT with us. He was pleasantly pleased with the anatomy of her lungs. Nothing alarming or concerning popped up considering her history. He used the world optimistic. Next we looked over Sophia. I didn't realize right away but he covered her trach with his gloved hand for a few minutes. While doing so, he looked over at me and mouthed, ''Oh my, this might be a quicker process than we think". WOOT WOOT! He said again that was very reassuring but everyone's guilty until proven innocent. End of day two.

Next week, we are back to Cincy for 6 more days of investigative procedures. First, they are going to put little miss asleep for the 12 time for a flex bronch. All three doctors from the team will each take their turn looking into Sophia's airway. The admitted their previous faults and realized that each doctor see things differently and more eyes are in fact better than one. After each looks around, they will put a camera up through Sophia's nose and down into her airway. They will leave that over night in the hospital to observe her breathing and reflux and gather more information. The next day we will have two types of swallow studies. We will meet with the A-team the following day and lastly we will have a sleep study. This will be an exhausting week. The doctors are doing all of these diagnostic procedures just to get to know Sophia before they make their game plan. You know what I really like, they have already had two conferences discussing Sophia before anyone laid eyes on her. Amazing. The ultimate goal (like is has always been) is to get the trach removed, her eating, and speaking. I am so far pleasantly pleased and very hopeful for what these new doctors have in store for Sophia. Its the total package really. And no one deserve a better package than Sophia. That kid has a heart of gold and willpower to envy. I mother and see her daily and yet I find myself looking to her in awe. The girl even signed to me the next day after the appointments that she had fun with mommy and daddy at the doctor. smh. what kid. Mine of course. She is one of a kind.

Please pray and send good vibes as we continue to embark on the next leg of our tremendous journey with Sophia Kay.