Blogging about life in Minnesota, raising our six kids with Down syndrome while battling Breast Cancer.

Be the kind of woman that when your feet hit the floor in the morning the devil says, "Oh shit! She's up!"

Monday, December 14, 2009

What we know we don't know

Today started out with an early appointment with the Pre-op team at Boston children's, and meeting with the anesthesiologist who will be doing Wednesday's surgery. Well, the appointment was SUPPOSED to be early, except that when I set my alarm on my Ipod, I forgot about the time change. I set the alarm for 5:45, and since Angela was NPO (aka nothing by mouth) sent Tyler down to sneak breakfast in the lobby. I got out of the shower and happened to glance at a small clock I hadn't noticed before......7:00!!!!!!! We were supposed to BE at the hospital at 7:00! I quickly called and they were really nice about it, just told us to get there as soon as we could.

We made amazing time, and walked in for our appointment at 7:29. That series of appointments took until 10:00, and I debated what to do for the 3 hour wait until the next appointment at 1:00. Whatever it was, I had to keep Angela away from food! We finally decided just to go back to the hotel and play Playstation. Ok...Angela played PS, and Tyler and I napped. ;-)

The next appointment was interesting because what they were planning on doing wasn't going to give them any information about what's going on. It would let them see some of her structures though. And, she did aspirate one swallow of the barium. Interesting, as we haven't seen aspiration in YEARS. So, now we know liquids are still a bit of a problem. For those who are new here, Angela had a gtube until she was 3 1/2 because of her inability to swallow liquids safely.

That done, it was time to EAT. By this time it had been 19 hours since Angela had eaten. Unfortunately the place we found to eat IN the Children's hospital, didn't have kid food! I don't know if there's another cafeteria here (I'll scope that out tomorrow.) but this place was not kid friendly at all. The only thing that had that Angela would touch was bread and Mac & Cheese. Only when we got to the table she wouldn't touch the mac & cheese. Finally I tasted it and discovered why. It had curry in it. Yeah..umm...not something Angela will eat. She ate half of my very tasty turkey wrap instead. (mmmm...I can still taste it, it was awesome!)

3:00 was the appointment we've been waiting for. Our meeting with Dr. Sam Nurko. First of all, what a very nice man! I guess you have to be if you're going to be working in a children's hospital, but still.

I had a whole list of questions with me, and found out the majority of them were irrelevant. Why? Because I haven't found anyone to talk to who has Cricopharyngeal Achalasia (which is caused by problems with the Upper Esophageal Sphincter, or UES) The only people found have Achalasia caused by the LES (Lower esophageal sphincter). While the disorders have similar names, they are completely unrelated. The risks of the two are very different.

So, for Angela, she has no greater risk of Barret's Esophagus (cancer of the esophagus) than anyone else.

Cricopharyngeal Achalasia is usually seen in very young infants as a birth defect of the cricopharyngeal muscle, which they usually outgrow. Or in elderly patients after a stroke or other neurological event. Angela's case is highly unusual BECAUSE of her age. While we believe she's probably had this since she was very little, why hasn't she outgrown it? Which leads the doctor to believe it was caused by whatever "neurological event" Angela has had.

Botox is often used in the achalasia of the LES, but they don't like to use it for Angela's form because it can affect the ability to swallow.

This problem was NOT caused by the nissen! Damage to the vagus nerve would have affected the LES, which is smooth muscle. The UES is striated muscle tissue, and controlled by the special swallowing mechanism, and not any one specific nerve. Very different from what the doctor at Mayo told me.

Tomorrow's manometry testing will tell us alot! First, she'll be sedated, and they'll place the manometry probe nasogastrically (through her nose then and down her throat into her esophagus.) then they'll wake her up. Once awake, I'm not sure exactly what they do, but the sensors will tell us exactly what area isn't functioning right when she eats solid food.

Assuming it shows what we expect it to, she'll have dilitation on Weds. She'll be put to sleep, and they'll go down with an endoscope, then use a balloon device to stretch out that area. Then she'll be woken up, and will stay overnight in the hospital so they can watch her eat and make sure A) the problem is improved and B) she doesn't have some new problem. If the dilitation works, it will last anywhere from a couple of weeks to a few months before it will need to be repeated. There's a "thee strikes" rule with this procedure. Three times and it doesn't work, and it's time to move on to other treatments.

About 50% of the time the dilitation doesn't work, and they need to do a myotomy (remove or separate the muscle) instead. That surgery is a BIG DEAL! And really...I think I'd have her go back to a g-tube before doing that surgery. We really didn't discuss this option any further, because we need tomorrow's testing, AND...I just don't want to go there right now. The biggest problem is this muscle works with the larynx. Remove or alter it and you will loose or seriously damage the voice.

For now, we're looking to tomorrow (Tuesday) once the tests are done, we're headed to the Boston Children's Museum for some fun time, while a friend of mine takes Tyler to the Aquarium. I'm sure both kids will have a lot of fun!


e said...


Prayers for all of you...we call Barrett's the "cops disease" of which larry has...or has the beginnings of...Too much seen and no outlet allowed, kwim???

We're managing it but he's OLD!!! lmao!! I can't believe they'd mention it with a child!

Do keep us posted...hope you find some pizza soon!

e & molly kate

onlywhoiam said...

so much one could say... I'll leave it at thinking of you and ENJOY THE AQUARIUM! It's awesome!

It's snowed here, just a few fluffy inches...