Blogging about life in Minnesota, raising our five 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!"

Wednesday, October 21, 2009

Boston and Cricopharyngeal Achalasia

Have you ever dropped the ball on something? How about, if you're the parent of a child with a complex medical history, have you ever just gotten so tired of the constant unanswered questions that you just stop???

Well, last spring, when we discovered how bad Angela's achalasia really is, the doctors at Mayo suggested we see Dr. Sam Nurko at Boston children's. In order to do that, it had to be approved by the state of MN. The state denied the request, saying the University of MN can do the testing here, and that the doctor here had done her training under Dr. Nurko in Boston. IF the dr. at the U of M writes a letter recommending we go to Boston, then we can go.

Insert big fat sigh here

I already knew the U of M had the testing equipment. I knew because I had called and talked to one of the program nurses who told me they'd *just* gotten it, and were being trained how to use it. Yeah, that's a comforting thought! NOT! I had images in my head of a doctor with a big head, all excited they have new equipment and look! A new patient with a very rare disorder! WHOO HOO! Jackpot!

And so I just stopped pursuing it. Angela has been like this for 12 years and we didn't know it, and while she's had some discomfort here and there, she's ok.

But this past weekend was NOT ok. Angela had a lot of trouble with various foods. Monday morning before school was exceptionally bad. She'd had a breakfast pizza, and it took her forever, with lots of retching and discomfort, and about 2 hours of drinking tons of water to get it to move through her esophagus. I felt like a heal. But Mondays are crazy busy for me with school, dog school, and other stuff.

Yesterday rolled around, and I realized I had NO EXCUSE for not calling the U of M. Especially when you consider they usually schedule out weeks to months in advance. I couldn't put this off any longer. So I called, and got the Ped. GI nurse, and said, "I'm not sure who we need to see there. Whoever trained under Dr. Nurko. But to be honest, I don't want to see ANYONE there, I just want a letter saying we need to see Dr. Nurko."

The nurse on the other end of the line chuckled. "Yes, we're very familiar with your daughter's case. (apparently it's been discussed among the three doctors) Dr. G. will be more than happy to write the letter so you can get to Boston. Our motility clinic isn't even up and running yet, and won't be for quite some time. We're still learning to use all the equipment, and have to be proficient with it before we can even think about testing patients with it. But the state of Minnesota says we have to physically SEE Angela, so how about if you come in tomorrow at 2:30 for a quick appointment, and we'll send you on. "

Are you serious? I have stalled for THREE months...why???? OMG! I could kick myself.

Once we have that letter, things should move very quickly. Dr. Nurko in Boston has already reviewed Angela's records, and has a plan of treatment for when we finally get there.

1) Do a new endoscopy, and at that time place the probe for the esophageal manometry testing. Because she'll have general anesthesia in her system, the probe will need to stay in 24 hours before they can start the actual testing. This is to make sure all the anesthesia is out of her system so it doesn't skew the test results. Hanging around with the probe in the esophagus carries it's own risks, but I'm not too worried about it.

2) do the actual manometry testing. This will tell us if the problem is A) due to nerve damage or B) due to a problem with the Upper Esophageal Sphincter. The outcome of the test determines our next step.


If the problem is found to be a problem with the sphincter muscle, they will go back in for another endoscopy, surgically separate the muscle, and also inject it with Botox to make it relax. This would need to be repeated about ever 2 years, and we would ALWAYS go to Dr. Nurko to do it.

If the problem is due to nerve damage, there isn't anything we can do. We will come home, and we will continue on as we have been. Eventually Angela's esophagus will give out, and she won't be able to eat solid food anymore, and possibly NO FOOD, and be back on a g-tube. So far, the doctors are surprised this hasn't already happened. As it is, her esophagus is completely slack. There is ZERO tone to it. Take a COOKED spagehtti noodle and hollow it out, now hang it from your jaw line to about where your stomach is. That's about how much tone Angela's esophagus has.

Obviously, we're hoping to find muscle problems! That's about enough info for now, huh? When I told Angela I was picking her up early from school today to see the new Dr. she was all excited that we were going on a plane to Boston today. LOL Whenever it is we go, we plan on being out there for a week. No time to DO anything there, just hanging out in the Children's hospital. I really want Dean to go along, but we really can't afford for him to go. It's not just his airfare, it's also paying someone to stay with the dogs.

So that's the update for today!

4 comments:

My name is Sarah said...

This is Joyce. I think I would have done the exact same thing. We're just conditioned that way after so much time within the medical system. So glad it is working out just as it should be now so you can get to Boston. We'll be hoping for the best outcome.

Kathie Brinkman said...

Well, Dudley could come here.

Warpedwoman said...

Ah...slack noodle...that's mine too! And mine is nerve damage too, but I am at least eating a pretty normal diet now. There is hope Leah!
Thanks for the message on my blog - I am now linked to you. I too blog about all sorts of stuff...so we have that in common also LOL!

Susan said...

I might be able to talk Peter into taking one of the littler dogs. Of course, he may come back less well trained then when you sent him!

You have a ton of friends who will help accommodate those dogs.