When we reviewed Clementine’s medical, it was pretty boring. In a really good way.
Down syndrome, a minor heart issue and developing relatively well.
Once we’d committed and were well into the wait, we got word of her breathing issues. Asthma? Pneumonia? Bronchitis? She was hospitalized for the entire month of April.
Then in September we got the news that she was being moved to Beijing for open-heart surgery.
Turns out, it was none of the above. And it would be months after we got home before we would really know anything.
In late January, we took Clementine to our local children’s hospital for tubes to be placed. During the procedure, our ENT wanted to perform a bronchoscopy to see if he could see a reason for her noisy breathing – despite being on a round of oral steroids as well as two daily inhalers. About 30 minutes into the wait for her to get out of surgery, I was paged in the waiting room. The nurse hustled me into a little room where the doctor was already waiting, eyes like saucers. “We’ve discovered the reason behind your daughter’s labored breathing. I’ve only seen this a few times in my career, and there is only one doctor that can treat it in the entire state. She has a condition called complete tracheal rings.”
He proceeded to show me a series of pretty scary images of Clementine’s trachea – so. so. small. He explained the risks of her getting a common cold, or much worse, the flu. He said that if she were to be intubated by someone who didn’t know exactly what they were doing, it could go very badly.
He said her trachea is 3mm. About 1/10th of an inch. Her trachea is the size of a preemie’s.
I don’t share a whole lot about our kids’ special needs because in many ways, I want to protect their stories. I share when I feel lead, but honestly, our kids are usually just that, our kids. Not a special need. But I want to share about Clementine’s special need, as well as these images for several reasons. The first, and most important one, is so y’all can pray for our girl. I want you to be able to see exactly what she has going on on the inside, so you can pray specifically, with her little anatomy in mind, for her upcoming surgery and the hours, days and weeks afterward.
I also want to share because there is almost nothing out there about this particular special need. As a mama of eight kids with special needs, with thousands of friends and acquaintances in several online communities who parent special needs kids, who manages a website dedicated to special needs kids from China, you’d think I’d have found someone. Somewhere. But no. I have been truly surprised by how little there is out there written by parent of kiddos with complete tracheal rings.
It is a rare condition, more often seen in kids with Down syndrome, but clearly unusual enough that my ENT, in his 20+ years in practice, had only seen a few times. Unusual enough that the only doctor in our state that has been trained to do the corrective surgery has only completed a handful of surgeries.
The surgery is called a slide tracheoplasty and will require Clementine’s chest to be opened just like open heart surgery. Her trachea will be sliced in half and split again, and then slid back on itself, held together by one long suture. Essentially becoming twice the width but half the length. A cardio-thorasic surgeon will be working with the ENT and pulmonary specialist and Clementine will be on cardiopulmonary bypass during the estimated six hour procedure.
The cross-section of a normal trachea should look like a capital D. The rounded part is hard, it’s the part you can feel when you touch your throat on the outside. The smooth, straight part of the cross-section, the part that backs up to the esophagus, is muscular and soft and stretches as a person grows.
The trachea at birth is about 4mm and by adulthood it has grown 4 – 7 times that size to accommodate adequate breathing (13mm – 27mm for men and 10mm – 23mm for women).
Clementine’s trachea – because almost all of her tracheal rings are complete rings (meaning they lack the smooth, muscular portion) – is not only too small, but will likely never grow any larger to accommodate her increasing need for oxygen. During her recent evaluation asked the pulmonary specialist what would eventually happen to a child with uncorrected complete tracheal rings. He looked at me and said, “They would asphyxiate.”
Usually children with complete tracheal rings have additional anatomical anomalies but our girl is special in that way too – she continues to refuse to fit the mold. She’s anatomically pretty perfect, other than her trachea, which means her surgery will not require any additional corrections. No heart defect, no pulmonary artery sling… which is a very good thing.
In many ways having complete tracheal rings manifests like a cardiac condition – we were told at her diagnosis to watch for bluing in her fingers and around her mouth, decreased appetite, frequent illnesses and fatigue upon exertion. The worst we’ve experienced has been brought on by just a simple cold, something that would keep an average child out of the mix for a day or two, knocks our girl out for weeks. And weeks. She just can’t seem to recover. Thankfully, right now we have been in a wonderfully peaceful ‘well’ season, that has us keeping her home almost exclusively, lots of antibac wipes and gel when we do venture out, and (I can’t even tell you how much) rigorous hand washing.
It was during her last long illness in late spring, after a few middle-of-the-night-she’s-not-breathing scares, that we decided it was time to follow up with our local surgeon to begin the difficult task of determining whether or not to pursue the surgical repair.
After meeting with the local ENT and cardio-thoracic team here, and discussing our options with our wonderful pediatrician, we decided to take Clementine to Cincinnati for further evaluation and a second opinion. Cincinnati Children’s is the home of the tracheal slide surgery – they operate on children from around the world with complete tracheal rings and have completed over 160 surgeries in the last 14 years.
While the seemingly-endless evaluations and sedated bronchoscopy were exhausting for Clementine and anxiety producing for her mama, it was such a gift to be able to take her to some of the very best airway and ENT doctors in the country. To have them discuss her with such compassion and wisdom. And to know that the decision on whether or not she needed the surgery was in very best hands.
At the recommendation of all of Clementine’s doctors, we have decided to pursue the surgery. We will return to Cincinnati in just a few days – on the 30th she will have a thorough pre-op evaluation completed and, as long as all goes well and Clementine isn’t feverish or otherwise ill, her surgery will take place on the 31st. We are expecting a hospital stay of 1 – 3 weeks, depending on how her trachea heals.
Please pray for her in these coming days.
That she would stay healthy and be in the best possible health going into surgery.
That she would not be afraid and that I would be able to meet her needs and comfort her.
That the surgeons would be at their best and the surgery would be without incident.
That she would be extubated easily and would not need to spend extra time in the ICU.
That she would recover well from the chest incision and that we would be able to control her pain well with medication.
That her trachea would heal perfectly and not require balloons or stints in the coming months.
That all my other babies here at home would be well, have fun and behave themselves while we’re away.
And, since I shared all those pictures of her on the inside, here’s one of her on the outside.
Cute as ever, and trying to learn how to pedal a trike, thankyouverymuch. We had a mini photoshoot yesterday, sort of a pre-surgery-and-mama’s-getting-emotional kind of thing, hopefully I can get them posted before it’s time to go.
I will do my best to keep everyone updated on how she is doing, probably posting most frequently on Instagram.
He sustained her, despite her health, and brought her home in the mightiest of ways. And I fully trust Him, His unrelenting love and His perfect plan for this child.
The LORD your God is with you, He is mighty to save. He will take great delight in you, He will quiet you with His love, He will rejoice over you with singing. – Zephaniah 3:17