Eleven Days and Counting

We are on day eleven of our hospital stay for Ben’s spinal cord surgery. The average length of stay for his particular type of tethered cord surgery is 4-6 days, but we don’t tend to do things the easy way around here. I’ve posted occasional updates on social media, but will highlight some of the specifics here – mostly so I will remember!

Despite some intubation hiccups (surprise, surprise) Ben’s surgery went well. He was kept intubated and on a ventilator for three nights in the PICU (pediatric ICU) to keep him completely flat so his back would have a chance to heal. Staying intubated is not standard procedure for a tethered cord release, but was done because of Ben’s dangerous airway. A nurse sat a few feet away from the foot of his bed 24/7 and would jump up to give him more sedation meds as soon as he began to stir. And by stir, I mean violently shake his head. At some points there would be three of us holding him down until the meds kicked in. My most helpless moment came when watching Ben’s oxygen plummet from an obstruction in his breathing tube. Then seeing a team of nurses ventilate him by hand and suction out three mucus plugs. Just a day in the life of a PICU nurse, but I could live without ever seeing that again.

Extubation (removing breathing tube) was our next big obstacle because Ben needed to breathe successfully on his own after days of being heavily medicated. His tiny trachea struggles even on good days, so extubation was no joke. The PICU team did a great job of setting him up for success and it went well. Whew.

The next hurdle was getting him through withdrawals. Our poor guy had bad tremors and nausea that took two days to get under control. During the post-ventilator medication game, which involved steroids for his airway, and pain/withdrawal symptom management, we found out Carter fell and ripped open the palm of her hand. Daniel rushed out to meet his mom at Urgent Care. Carter ended up with thirteen stitches and has needed three additional hand-related appointments during our hospital stay.

After four days in intensive care, Ben was moved to the neurosciences floor, where we spent several more days. As we were getting ready to be discharged and applying Ben’s final dressing, we discovered the incision was bulging from a cerebrospinal fluid leak. Ugh, the dreaded leak. The neurosurgeon was called out of a surgery to come check on it and he declared we must go back to the PICU for sedation, a compression wrap and diuretic. I almost fainted (really) when he said “If we have to keep him intubated for two weeks to get this under control then that’s what we will do.” Thankfully, his nurse practitioner was as cool as a cucumber and talked me down from passing out. ENT said it was safe for Ben to be taken off his steroid, which interferes with healing, and thankfully he hasn’t needed it since.

So here we sit. Doing everything to avoid another surgery which would start the entire cycle of airway management and healing again. The fluid on his back has significantly decreased and he came off of sedation today. We hope to be out of here on Monday – only three more nights! Our kids are missing Ben like crazy and we are exhausted. So many people have stepped up to take care of us, for which we are incredibly thankful.

Despite some curve balls, we are what the staff refers to as a “planned stay.” This is so very different from those who are here otherwise. Don’t get me wrong, there is grief in learning the original diagnosis and fearful anticipation of a dreaded surgery. The past twenty-two months have been a roller coaster ride. But, our particular stay in the pediatric intensive care unit means we are a step closer to resolution. One more surgery behind us. Something that was wrong with Ben’s little body was made right. It’s not lost on me that other parents did not have their PICU stay already marked in the calendar. But there is such camaraderie here. Passing each other in the hallway with a nod of solidarity. Being excited for the families going home after six days or six months – seeing the relief on their faces, with a tinge of survivors guilt and a timid wave goodbye. There’s a collective fear in the waiting room with new arrivals anxiously awaiting results, and the heaviest grief with those who just received sad news. We don’t know the details of what brought each other here, or even each other’s names, but we know we are in it together.

Surgery tomorrow, but not the one we expected…

 

This photo included because A) Ben had the best night of his life on the dance floor and B) to remind myself our hospital selves are not our permanent selves.

Complete Tracheal Rings Update: We’ve been to visit the airways team at Cincinnati Children’s twice since my last update and have another date on the books for early January. The doctors feel Ben is on the very edge of needing tracheal reconstruction, but the surgery is so dangerous and the recovery so difficult that they want to continue to wait and watch. So that means we will continue to return to Cincinnati for bronchoscopies every 3-5 months for the foreseeable future. I’m so acutely aware of Ben’s stridorous breathing. It’s nails on a chalkboard to my ears. But, opening his little chest – that’s already been opened for heart surgery and has scar tissue and sternal wires – to reconstruct that little trachea is no small feat. So medical trips are our new norm and I’m learning not to put our life on hold “until we find out what Cinci says.” It’s terribly hard to be at the mercy of the hospital’s schedule. I had to miss Carter’s kindergarten graduation during our May trip (many tears shed over this) and leaving town during the first week back to school in August was not easy. We were told to come back in December but I asked to have it pushed to January. I do have my limits.

In the meantime, and the real reason for a new blog post, is Ben’s recent diagnosis of a tethered spinal cord. A few months ago, we learned Ben’s incontinence is not just part of his developmental delays, but he truly has no control and may actually be in pain when he urinates. This finding led to a spine MRI, which showed a spinal lipoma, small cyst and tethered spinal cord. A normal spinal cord moves freely within the spinal column. As the name suggests, Ben spinal cord is attached (tethered) near the base of his spine and therefore being stretched as he grows. Without surgery to detach the spinal cord, continued strain would lead to progressive neurological and motor impairments, including paralysis. Our neurosurgeon told us this ideally would’ve been repaired as an infant, and unfortunately Ben may not regain bladder function, but here we are with a five-year-old boy just thankful he’s alive.

During our August trip, the airways team and neurosurgeon agreed it’s safe for Ben to have spinal surgery at our local children’s hospital. Special precautions will be taken with Ben’s airways and an ENT will be on-call during surgery and recovery. Ben will likely spend 3 days sedated and lying on his tummy in the PICU to prevent cerebrospinal fluid leakage, then 2-3 more days recovering on the neuro floor. During Ben’s heart surgery in China, his caregivers were not allowed to be with him during his recovery in the ICU. Our little one-year-old Ben was all alone in the hospital! So we count it a privilege to sit by his side during this recovery.

Who knew so many surprises could come in one little package?

I’ll be posting updates on Instagram and hope to get in a blog post or two during our stay.

“Love recognizes no barriers. It jumps hurdles, leaps fences, penetrates walls to arrive at its destination full of hope.” 

                                                                            ― Maya Angelou

 

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!

 

 

 

Manic Monday

 

Today was an absolute whirlwind. We arrived at the hospital at 9am and did not return to our hotel until 5:30pm. Our day included a video swallow study, anesthesia consult, meeting with the pulmonologist and ekg & echo. I am in love with this hospital and the people we met today.  Our day was soooo loooong because each and every person was incredibly thorough, professional and kind. Ben was so tired by the end of that day that he fell asleep during his echo. Easiest echo ever!

There’s not much in the way of results to report at this time. The real information will come on Wednesday when Ben is sedated for a chest CT, flexible bronchoscopy and rigid bronchoscopy.  The pulmonologist we met with today explained that when he started practicing in the 70’s, complete tracheal rings were considered a death sentence. Yikes. He also said Cincinnati Children’s is incredibly aggressive in their approach – they are willing to take on cases considered inoperable. BUT, they are also very conservative and look at all aspects involved, heavily weighing the consequences, before recommending surgical repair. Tracheal reconstruction is no joke. And at the moment, the only information we have is a 3mm neonate tube had to be used when Ben was intubated for his tonsillectomy. The size of a stir straw!

Today’s doctor was also very familiar with healthcare in China. He’s been to Shanghai and Beijing to help train doctors on safe bronchoscopy techniques.  So when he asked me how much I know of Ben’s history and I replied, “Well, I know he’s alive,” he totally understood. Ben is our little miracle and it’s such a privilege to be his family.

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.”