Blogging about life and raising our five kids with Down syndrome.

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

Monday, November 23, 2009

Impending something

My blog post title today says it all. I don't feel a sense of impending doom, but it's close to that. All is not at rest in my soul.

I'm very worried about this trip to Boston. It seems every time we see a new specialist we find out things are worse than we thought, and I expect the Boston testing will end up no different.

Let me put this into perspective a bit. Achalasia, which is the name for a lack of paristalsis in the esophagus along with a malfunctioning LES and the subsequent stretching of the esophagus that comes with it, is rare. Fewer than 1/100,000 adults have it. Of those who have it a small percentage need surgery for it. The rest manage with dietary changes and endoscopic dilations. I have found a few blogs, and a list serve for people with Achalasia. There are a couple of parents of teens on there.

But the type of Achalasia Angela has, Cricopharyngeal Achalasia, is even more rare. This happens at the TOP of the esophagus, causing food to get stuck right in front of the airway. ( The fact that Angela is still alive is pretty much a miracle. The fact that her lungs haven't been severely damaged by daily aspiration is nothing less than a miracle.) I have found MENTION of C.A. in reports online. And I have found mention of it in studies. I have found descriptions of it on medical journal sites. I have yet to find someone who actually HAS it. Those of you who know me, know that if it's to be found, I will find it! I have spent countless hours rooting around the internet and I'm just not finding these people!

I have not gone into all the details here about the possibilities for Angela. I'm just not claiming those things for her, and I prefer to live believing a less-invasive option will help her. Until the doctor tells me something different, I'm going to keep my rose-colored glasses on.

And yet, how can I ignore the possibilities? So, I'll lay it all out here on the table.

First, if the problem is found to be *just* the UES (upper esophageal sphincter ), then while we're in Boston she'll have either a myotomy (removal of that muscle) or she'll have it dilated and injected with botox to help it relax. This is the outcome we're praying for!

If the problem is found to be with the vagus nerve, then we have bigger problems. The risk of Angela developing Barretts Esophagus (cancer of the esophagus) is extremely high. The fact that Angela's esophagus is just completely slack and really does *nothing* is of huge concern. If myotomy (separation of the muscle) isn't an option for her, eventually her esophagus will need to be removed.

Yes, you read that right.

Some people who have their esophagus removed have it replaced with a section of their own small intestine. Some just have their esophagus shortened, stretching the fundus, or top part of the stomach up to meet it. But in Angela's case, it would be her ENTIRE esophagus. And, given her history of poor wound healing, this is just NOT something she could tolerate. Not just the removal part, but the putting back together of various bits and pieces! Her scarring abilities are crappy, to say the least. Her surgeon here, who I absolutely adore, said he'd never want to touch her again unless there were no other option. Her last round of surgeries 6 years ago, which should have been a one-week stay in the hospital, landed her there for four and a half months!

So, when you remove the esophagus, how do you eat? Well...that all depends upon how things are put back together! Some people, once everything is reconnected, are able to eat and are pain free for the first time in their lives! It can be a real blessing to have it removed or shortened. Some people end up using a gtube instead. In Angela's case, since using her own intestine really isn't an option, she would end up on a gtube. THAT doesn't scare me, since she's had a gtube before. And really, she's old enough she could do most of the gtube stuff herself now. The problem, once again, goes back to wound healing. When she did have her gtube before, the stoma kept breaking down and stretching out. Pretty soon it couldn't hold a tube in place and every couple of days we'd have to take it out and let it shrink down a bit. More than anything it was just a pain in the ass to deal with. But, maybe now that she's older, things would be different??? I don't know.

Have you ever seen Angela eat? Have you seen her enjoy every last morsel of a plate of french fries? Have you seen her chug a root beer? Have you seen her at Applebees (or any restaurant for that matter) and seen her in heaven with food? It's not something I want to take away from her.

So, that's what I laid awake thinking about all night. All these things coming up. What will they find, and how will they fix it? I'm also dreading that Dean isn't going along to Boston. I don't want to be alone if they give me bad news. It will be up to me to stay composed with Angela sitting there, and I just know I won't be able to do that.

............sigh.............

6 comments:

cremcd said...

We'll be sending all our good vibes your way, hopefully for the best possible outcome. ~Courtney

Julie said...

Leah, I've been following your blog for quite some time, and also see you post on another forum. I don't think I've introduced myself. My name is Julie. I'm a sahm of 10. Gus, my youngest, has Ds. My family and I just love to read your blog! Angela is such an inspiration to us, as are you! I will keep you and especially Angela in our prayers. She is such an amazing kid!

Tamara said...

Hey, gf, you know we're praying here too. I just don't know what else to say. You've all been through a lot, and we'll just pray that if things are as bad as you think they may be, that those doctors in Boston work a miracle or two -

Leah said...

Awww Thanks everyone for your thoughts and prayers. I really do appreciate them. I think I must be hormonal or something. The only time I get all gloom and doom is when I am. Otherwise I'm usually the one to find the humor in the situation. I'm just having trouble shaking this feeling of impending something.

My name is Sarah said...

Leah this is Joyce. Golly I can't imagine what you are going through right now. I am so thinking about you and Angela. Hopefully they will know just what to do.

Warpedwoman said...

I know I have commented before, but I only do so to give you hope. I too have no peristalsis at all - none! All the awful things that you can hear about can worry you (or me) (and of course they do exist however much we wish to believe otherwise) BUT - there are options and good ones too. I hope that dilation will be suitable for Angela although, you never know - the botox may be simple and work for her. I had to have surgery and with my scar complictations I had to make sure I had a good surgeon. Since then, I eat in a way that looks normal to the rest of the world and is mostly easy enough for me and I have had no aspiration since the surgery and that was a very regular thing. Like Angela, how I avoided lung infection, I don't know.
Anyway, concentrate on the positves of the outcome and hope for the easy route - I will keep everything crossed for you.