Just Kidding!

A photo from Ben’s adoption file

I meant to write a final wrap-up of our visit to Cincinnati, but got right back into real life. Meaning, no quiet time in a dark hotel room while Ben naps. It really was quite the trip, finishing off with a fever for Addie and a detour through snowy rural Ohio (or maybe it was Kentucky?) because of a closed road. No lie, GPS and GoogleMaps told me the detour would take 2 days and 4 hours and involved a ferry. What?!?!? I pulled over, had a quick cry, studied the map, turned off the ridiculousness of my navigation devices and off we went. Addie suggested we listen to “We Know The Way” from the Moana soundtrack since we were clearly doing some serious wayfinding. We blasted it and felt very inspired and empowered. We also saw an abandoned waterpark, which was really creepy. All in all, it only added about a little over an hour to our trip. Addie and Ben were so incredibly good during the long twelve hours of travel after a long week of appointments and unexpected illnesses.

Our final appointment at Cincinnati Children’s was fairly uneventful. Ben had a FEES study, which involved a small telescope being passed through his nose (he was awake) in order for the ENT doctor and speech therapist to get a better look at his swallowing while eating and drinking. Interestingly, he had some intermittent movement of his “paralyzed” vocal cord. The doctor had no definite explanation for the regained function, other than being pleasantly surprised. When Ben took a sip of juice, there was some wild sloshing (my words) that could lead to aspiration, but overall it seems he is learning how to better protect his airways while drinking. He still needs his liquids thickened, must use a straw and his positioning should be carefully monitored – no tipping his head back, one sip at a time, etc. – but he is making progress!

We were told the airways team would meet in a week or so and call with their final recommendations.

A few parting words included:

“Roughly 60 kids are born with complete tracheal rings each year.”

“It is truly a rare condition.”

“90% of kids need the surgery.”

“We are cautiously optimistic he’s the 10%.”

“Let us know if he starts to turn blue.”

and

“See you in 6 months!”

Except not really.

Two weeks post-scope and Ben was not sounding great. He never sounds great, but the “washing machine” sound while breathing just wasn’t letting up. The nurse said she’d expect this up to a few days post-procedure, but not for this long. A few phone conversations later, a course of steroids was sent to our local pharmacy and I was told Dr. Rutter wants to see us back in three months. Ben also needs another sleep study determine if they will include a DISE (drug-induced sleep endoscopy) procedure during the next OR visit.

So we are back in the scheduling game. I found out this morning that the sleep study at our local children’s hospital is set for March 29th. Hopefully Cincinnati Children’s will be able to schedule soon so we can start to figure out travel and child care. The thought of doing this again in just 10-12 weeks is a little daunting. But, we were entrusted to be the parents of this little boy and went halfway around the world to get him and call him our own. When you factor that in, I suppose Cincinnati is considered fairly convenient.

The boy who breathes like a pig and other exciting news

Yesterday was a much needed day of rest. We hit up the Disney store, stocked up on snacks at Target, napped, and then headed across town for dinner with my cousin and her family. It’s so fun to see our kids just pick up where they left off two years ago. My dad is also in town for work, so it’s been really helpful to have another adult to distract all of us and help out in the evenings.

And it was a good thing we had a day of rest. Because at 9pm last night, Ben started barfing. It continued until 10:30 or so. I’d never seen his little body so limp. I was hoping it was just carsickness, but by 3am and 4am, while he was heaving and whimpering in pain, I began to panic he wouldn’t be well enough for anesthesia.  All that time off of school, turning down playdates, and avoiding public areas to keep him healthy and then this?!?!

Thankfully, Ben’s lungs were clear, his temp was normal and his stomach was empty, so anesthesia gave the thumbs up.  The little stinker even mustered the energy to wave to nearby nurses as he rolled down the hall on a stretcher. Then the doors to the OR opened, he blew me a kiss, and that was that. He’s sort of a pro at medical interventions by now.

Here’s what we learned today:

– Two-thirds of Ben’s trachea is composed of complete tracheal rings. Meaning, two-thirds of his windpipe is restricted and unable to expand as he breathes. We originally believed he might have one or two complete rings. Nope!

– A 3mm tracheal tube fit through the restricted portion of his trachea. This is the size of tube used to intubate a neonate.

– They also found he has a tracheal bronchus, which means the airway (bronchus) to one of his lungs comes directly off of his trachea. The doctor explained to me that pigs have this type of airway, so don’t be concerned if someone refers to this as “pig bronchus” in the future. Ben has similar anatomy to a pig. Because at this point, why not?

– Most Important Info of the Day: Dr. Rutter believes Ben could possibly be in the 10% of children who do not need surgery! He is taking a cautiously optimistic approach and will see us back in 4-6 months for another evaluation. Trips to Cincinnati will be our new norm until a decision is made one way or the other. I was warned this could take years to decide.

– But what about all the symptoms??? The team is going to treat Ben’s current symptoms of strider and otherwise noisy breathing as a baseline. They told me  he will still needs steroids to make it through a typical cold. His breathing will still sound like a washing machine when he’s running around. We will still see some retraction when he’s having a particularly rough patch. But his current “bad” is not bad enough to warrant a very, very risky surgery.

So tonight, we went out to celebrate with burgers and milkshakes. Poor Ben had to eat goldfish and a banana, but was totally back to his goofy self.

Tomorrow we return for a FEES (fiberoptic endoscopic evaluation of swallowing ) study. A small telescope will be passed through Ben’s nose and he will be be given various foods to eat. Should be interesting!

 

 

 

Cincinnati or bust

The day we met Ben he had incredibly noisy breathing and a deep, croup-like cough. When I asked his nanny about the cough, she said he clears his lungs every morning with lots of coughing due to his heart condition. She also advised we not let him have more than thirty minutes of exercise. Nanny and New Mommy both knew this boy needed to be kept under close tabs. We made our promises to love him forever. And thus began our journey…

Twelve months later, our list of medical issues has grown quite long. Aspiration, paralyzed vocal cord, profound unilateral hearing loss, talk of congenital infectious diseases (since ruled out), crazy amounts of strep, rare urinary bacteria, mild sleep apnea, and so on. But his repaired heart looked great! And his rattly breathing was written off as gunky vocal cord noise, a hefty kid with Down syndrome, and reactive airways.

I was not satisfied with the answers. We spent to many nights jumping out of bed to see if Ben was still breathing during horrendous coughing spells. His nostrils flared too much for my liking when he ran across the room.  I bought my own pulse-ox. I took videos of his coughing and breathing. I cried next to his bed. Our wonderful pediatrician always took my mom instincts seriously. The local specialists? Not so much.

So, when our ENT sat me down in the waiting room after Ben’s tonsillectomy to tell me they had trouble intubating him, I was not surprised.  When he told me they found several complete tracheal rings, making a portion of Ben’s trachea the same size as a newborn baby’s airway, I was shaken, but also kind of relieved. We finally had an answer.

So what the heck are complete tracheal rings? I’ll let Cincinnati Children’s explain:

The trachea (windpipe) is made up of cartilage, which is also called tracheal rings.  Normally, tracheal rings are “C” shaped.   Complete tracheal rings are “O” shaped.  The “O”-shaped rings are always smaller than the normal “C” shape and may make it harder to breathe due to a narrower trachea.

Complete tracheal rings are a rare condition present at birth.  It is usually associated with other vascular (blood vessel), heart, or lung abnormalities.

Children with complete tracheal rings have noisy breathing which gets worse over time.  Typically, their breathing sounds like a “washing machine”  because of the noise made when they breathe in and out.

Some children may have to work harder to breathe, causing a sucking in around their ribs and chest (called retractions).  Some children may also have trouble with feeding.  Colds or respiratory illness can also make breathing worse.

So here we are, with the poster child for this condition. Which is why I’ve spent the past three months making phone calls, talking to insurance, asking for letters, looking at my calendar, praying for peace, and arranging details. Ben has been on medical house-arrest to prevent sickness as while we await our trip. His IEP was even changed from classroom to home instruction. We’ve been washing our hands a million times a day. And we are soooo close to more answers.

On Sunday morning, we leave for Ben’s week-long evaluation with the aerodigestive team at Cincinnati Children’s Hospital. As our local ENT put it, we are headed to the Mecca of Airways. And because everyone needs an 8-year-old assistant, Addie will join Ben and I with the promise to be my helper, do her homework and “maybe play on the iPad a little.”

 

 

 

 

Annnnd, we’re back!

I started this blog many years ago when stay-at-home-momming + blogging was a definite thing. This blog (named Really Really Much by Addie) saw us through the the addition of two little girls, a move to Seattle, then back east to Virginia Beach. After a twelve week stint on bed rest and one measly shout-out to newborn Jack, the blog updates ended and many real life adventures began.

During a four year blogging hiatus, life involved numerous doctor appointments, specialist visits, physical, occupational and speech therapy. Jack was diagnosed with hemiparesis (in his case, mild weakness affecting the right side of his body) and Childhood Apraxia of Speech. Parenting Jack opened our eyes and hearts to the possibility of special needs adoption. Enter Benjamin aka “Yang Yang.” It turns out, there was a three-year-old little boy with Down syndrome waiting for us to see him and call him our own. And in January 2016, after ten months of anticipation, we traveled to China to bring home our son. And what a year it’s been.

This blog has been resurrected to update our friends and family as we navigate Ben’s newest diagnosis of complete tracheal rings. This condition is so rare I’ve only been able to connect with ONE mom who has walked this road. Besides sweet little Clementine’s story (which I’ve read at least a dozen times) and a recent video on our hospital’s website, there are no other personal stories out there.  My hope is to document our journey so there is just a tiny bit more information available the next time a scared mom googles “complete tracheal rings.”