First of all, thank you all for your prayers. Evie did great with the procedures and anesthesia and is just a bit grumpy from it all.
GI and pulmonologist both had good news. GI didn’t find any of the problems he suspected except one: she has a tiny stomach that doesn’t have the ability to expand very much. Most stomachs have lots of folds in them so they can expand with a meal, but Evie’s has very few folds. This explains why we’ve never been able to do very large volumes (she can only take about 3 ounces in a feeding).
He also noted that while her Nissen fundoplication is still intact, it is so small that it is basically doing nothing for her, so that explains why she still throws up quite a bit. So I guess the downside of these two tidbits is that we’re still pretty stuck with her feeding issues. There’s not really much we can do except wait for her to grow. Overall we’re very relieved that she doesn’t have any serious GI issues that will require surgery, though. The pulmonologist said Evie’s lungs looked great and are very normal. We’re still waiting back on some cultures, but anatomically, she was very pleased.
Now the more complicated stuff. First the good news: the ENT feels that someday Evie will be able to get the trach out! Yay! Unfortunately, she has quite a few airway obstructions that we didn’t know about, so it will be a long process of taking care of that list.
Here’s the current plan.
- Open up her nose this coming Monday. Her nasal passages were completely blocked off with scar tissue. Her CT scan showed that she had a tiny hole, but they couldn’t find it during the scopes.
With her first two repairs, both sides of her nose were opened separately and this next time they are actually going to take out some of her septum that divides the sides and create one big hole in the back. Since the hole starts off bigger, it will hopefully take longer to close up again. It doesn’t sound like they’re going to do stents to keep it open like I was originally told since the data doesn’t really show better results with stents. Once we’re pretty sure her nose is going to stay open (which could take 1 surgery or 15 surgeries, no way to know) then…
- We’ll get her tonsils removed. Apparently her tonsils are extremely large and block off a good portion of her airway. This is a pretty common procedure for CHARGE kids. After that…
- We will get a consult with a plastic surgeon to address her small jaw. I think we’d been told before that her jaw was small, but no one ever mentioned that it could be an issue. Unfortunately because her jaw is so small, her tongue sits back further in her mouth and falls back into her throat a little, blocking off her airway. This is a little bit of an intense procedure involving cutting both sides of the jaw bone, attaching metal brackets and slowly turning screws to lengthen the jaw. The new bone grows in and then is given 6 weeks to harden before the pins and brackets are removed. It sounds like kids do very well with the procedure and it’s usually harder on the parents. Here’s a good link with some slideshows explaining the procedure more. Then finally after that…
- We will do her tracheal reconstruction surgery and hopefully remove the trach right away. Evie’s tracheal scarring is bad. They said it’s not the worst they’ve ever had, but it is very severe. The opening into her trachea right now is only 3mm wide. That’s about the size of coffee stir straw.
We don’t really have a timeline because Evie will need to heal fully from each procedure before moving to the next one, but it will be at least a few years. The ENT said most CHARGErs have their trachs 5-10 years.
We’re both happy and sad right now. We’re so glad that they think someday she’ll be trach free. That’s wonderful. But it will take us awhile to adjust to the knowledge that she will be having many more surgeries than we had anticipated. We just wish things were easier for her.
We have a few days off now and plan to just rest and enjoy some time as a family. The weather is beautiful here and we’re hoping to get outside a bit with Evie. Next step will be her surgery on Monday with a few days inpatient.
Thank you again everyone. We could feel your prayers keeping us calm and focused this morning.