One step at a time…

Yesterday, we moved one step closer to some answers we’ve been asking since Landon was born- it was time for her first CT scan. When she was born, we had several doctors tell us she needed to have one done. While I lay there two days post partum, a flurry of doctors who traveled in packs of four would cruise in and out and proclaim a list of tests she needed (or was having) and I vividly remember the CT scan conversation like it was nothing. “Ct scan needs to be soon! Schedule it now!”  After discussing it ad nausea at the Clark School and with other supportive “special” parents… I knew we would wait.  There was no reason to do it then- she was too little, it was too much radiation for a 6 lb baby and her father and I knew she didn’t need it yet. Surgeries wouldn’t occur for years, but doctors can be so pushy sometimes and not always with necessity. Alas, we waited and I’m so glad we did.

And yesterday… Landon killed it. She was amazingly still, put her “brave face” on, and was in and out in 10 minutes at the most.  Truly, it was shockingly easy in the end. To prepare, we actually learned what the word “still” meant, and played “statue’ where she practiced freezing while her step-sister tried to make her laugh. I walked her through what it would be like- a machine that looks like a huge donut would be above her little head and she would get to lie down to play the statue game. I made sure she knew she could bring Lenny the lovey and that I’d be right by her side.

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The night before and the morning of the scan we also talked about who we thought was brave. “Moana! Moana is super brave when she walks toward the fire monster and places her face on her face. Moana saves her island and is brave with the water. Also Hei Hei is really funny. Momma, I’m brave like Moana!” Thank the world that Moana exists and my girl can esteem to be just like her. Seriously, thank you Disney for this one- parents definitely approve- but I digress. As we talked through who we thought is brave, she of course mentioned Anna her sweet sister, and I told Landon I thought that she was the bravest kid I’d ever met. She beamed and moved her eyes from side to side. She stood a little bit taller I like to think after that conversation.

So… why do a CT scan now? Well, in conjunction with a sleep study we’ll do June 3rd, Landon’s ENT wants to be able to give us some clearer direction on what’s next for her so we can strategize and schedule her surgeries appropriately. The CT scan will tell the team what’s going on inside- the structure of her inner ear in particular.

Here is even more detail in case you’re wondering:

The scan will allow the otolaryngologists (ENTs) to “see” into the middle and inner ears to understand the internal anatomy. From the scan, they will grade the her ears’ anatomy on a 10 point scale to determine if canalplasty (opening the ear canals) is an option for improving her hearing loss.  This 10 point scale is called the Jahrsdoerfer or “J” scale and was developed by Dr. Robert Jahrsdoerfer.  The “J” scale is broken down on the following point system:

Stapes bone 2
Oval window open 1
Middle ear space 1
Facial nerve 1
Malleus-incus complex 1
Mastoid pneumatization 1
Incus-stapes connection 1
Round window 1
External ear 1
Total Possible Score 10

If an individual rates a 7 or higher on the scale (70% out of 100% chance of having hearing restored to normal or near normal range), he/she will most-likely be a candidate for canalplasty.  Usually, if the individual rates a 6 or below, canalplasty is unlikely an option.  However, in some cases some individuals may still be candidates even with a 6 rating depending on the actual anatomy of the ear.

Because Landon has Treacher Collins Syndrome and not just microtia atresia, she is most likely not a candidate for a canalplasty because the anatomy of someone with TCS is usually far too small to qualify. The critical importance of following these specialists’ advice on this is that a canalplasty poses a significant risk for damaging the cochlea. So, we’ll know where and what her surgical future may look like with the designation on the scale. We’ve not yet decided if we’re doing microtia repair and building new ears, but if there’s a good chance for a canalplasty that’s a large piece of this microtia puzzle.

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Next up will be the sleep study. That night they will cover her little body in nodes and watch her sleep while analyzing her air flow. Given the (probably abnormal) amount of sleep stalking I’ve done since she was born, I’m fairly certain she has some form of obstructive sleep apnea (OSA). It is also very common for anyone with TCS because of their traditionally small jaws (called micrognathia) and therefore the tongue rests far in the back of her throat and obstructs air flow. We will know after this study the severity of the apnea and then be able to discuss (using actual data!) whether she needs a tonsillectomy, adenoidectomy (or both) or the full monte… a jaw distraction or “mandibular” distraction surgery. That would involve making the lower jaw larger by lengthening the lower jaw bone and relieving airway obstruction. That last surgery there… that to me is the most terrifying surgery in all of the TCS related surgeries. It’s invasive, it’s extensive, it’s painful.

But… knowing more of her surgical future can allow us to prepare for the what, when and begin the conversation of where. We’ll have more questions following these answers, but have studies completed to be able to get those other opinions. The absolute hardest part in this is not moving ten steps ahead and being consumed by the what-ifs. What if she has jaw distraction surgery? Who is good enough to perform that surgery? Do we use someone in Charleston? In NYC? In Philly? Do we take a chance with a canalplasty if she scores a 6 on the J-Scale test? How many opinions do you get before you make a decision?

The weight of the future decisions has settled somewhere deep in my chest. The emotional recall is quick so if we talk about it, there’s a very good chance I’ll end up in tears. Don’t think I don’t want to talk about it, and don’t assume you’ve caused the tears,  but there you go… that’s your fair warning. I currently feel like I’m emotionally stuck together by some duct tape and bailing wire. The only way to live, however, is as if these concerns and future decisions are not looming. I have to show up every day for my family and be source of love and fortitude. I have to show up at work and be on my game for everyone I support with work alongside. I believe that’s how I can show Landon what bravery is like outside of Disneyland. Life is about showing up. Isn’t that the old sports adage thrown around in pre-game huddles and on inspirational posters in offices from the 90’s? And it’s still true today- that and one day at a time.

There you go my friends and tribe members… that’s the latest on Landon’s medical life and why there are a few more pictures lately in doctors rooms or hospitals on my Instagram. Thank you for your love and notes of encouragement. We have certainly managed to have a lot of fun lately as well and some of those pictures are below. I’ll certainly try a little harder to keep this thing updated as this blog always serves as a wonderful space to exercise my thoughts and (hopefully) help those going through similar things with their little ones.

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Sending you love-

xoxo,

Eloise

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4 thoughts on “One step at a time…

  1. So touching. My nephew went out to CA in December to meet with Dr. Lewin and have his microtia surgery. It went awesome and his new ear looks so real. Let me know if you decide to go that route and I can get you in touch with my sister-in-law if you’d like! Best of luck!!

  2. Girl you are a rockstar!!! Landon too!!! Thinking about you and sending positive thoughts and prayers!!! Love and miss yall!! 😘😘

  3. That smile, just kills me. So beautiful just like her mom (non creepy way 🙂 I’m 22 and have had 57 brain and spinal cord surgeries and many, many mri’s and ct’s. I can definitely say that I could not lay that still for a scan until I was older than she is. Great job Landon! I just found your blog and you truly are an amazing, awesome and gorgeous little girl.

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