The week that Dean left town

Audrey and Abel had their surgery on April 7th.

On Saturday I took Angela shopping for her prom dress.

On Sunday Dean went down to Arizona. His parents own a condo there and that's where they spend their winters. Dean was helping them pack up then help them navigate the airport and fly home.

On Monday morning Angela got up from school very pale and running a low-grade fever. She's had a bit of "Monday-itis" several Mondays in a row, only she is truly not feeling well. (Its very odd and makes me wonder if there is something in the house she's reacting to.) Anyway, I kept her home and she ended up sleeping all morning. About 10:00 Axel's teacher called and said he wasn't feeling well, and neither was his interpreter. Around noon Angela woke up saying her head and neck hurt really bad, and she didn't want to turn her head at all. She also didn't want to wear her glasses, I think because of the migraine. It was time for us to visit Dr. Mary.

On the short drive over Angela started acting very strange. She was moving her hand in front of her as if she was wiping off a window, "My eyes is foggy." she kept saying. I took this to mean her vision was blurry. Then she started talking NONSENSE and doing a weird shaking with her head. I asked what she was doing. "I don't know. I'm shaking my head but I don't know why."

When I got her out of the van we started walking to the clinic door when she dropped to the pavement. I tried telling Axel to run in and get someone to help but he didn't understand what I wanted. After a few seconds she was able to stand back up though I was supporting a lot of her weight. I waived at the receptionist and said, "We're going straight to a room!" and kept walking.

In the exam room we got her up on the table so she could at least lie down. Her temp was still 101 with Tylenol and still she complained about her head and neck. We did some blood work and a strep test, which was clear. However, the doctor looked in Axel's throat and he had a real mess going on in there. He tested positive for strep. Given Angela's history the doctor wanted me to take her to the hospital, and she called ahead to let them know we were coming.

She was in bad shape when we got there. Her blood pressure was down to 60/40 and she was extremely dehydrated. (The ER doctor later told our ped. that we should have been transported by ambulance but her blood pressure wasn't that low in the clinic.)

We ruled out all kinds of things, including leukemia since her white count was at 28,000, in order to arrive at doing a spinal tap to test for Meningitis. Although three doctors had listened to her chest by this point, and all said she sounded clear, the ER doctor decided to do a chest X-ray to rule out a sneaky case of pneumonia.

And he wins the prize!

Unfortunately that hospital had no beds open so Angela was transported by ambulance to the partner hospital. Angela is all about medical stuff, and ambulances, etc so she was thrilled to get a ride!!! When I arrived there a short time later two doctors were in her room. That's when I was informed she not only had pneumonia, but a quarter size "pocket" of something they could not identify. It did not look like a mass, but some type of fluid. They asked if she had been exposed to anyone with TB. They also asked how long Audrey had been home. It was one year last month. Both doctors exchanged looks then wordlessly left the room. When they returned a few minutes later they were fully gowned, had face shields, gloves and booties. They didn't *think* she had TB, but it was possible and they weren't taking any chances.

So let me recap a bit: Angela is admitted to the hospital at what ended up being 2:00 in the morning. I had four kids at home, one of whom tested positive for strep. Dean was out of town. Luckly of all the kids to be hospitalized it was Angela this time. The one who always says, "Just drop me off at the door" and is disappointed when she doesn't get admitted!

As it turned out all the kids ended up testing positive for strep. I had my pre-op physical and begged my doctor for antibiotics so I wouldn't have to cancel my upcoming surgery. Dean came home in the early morning hours on Friday. On Saturday Angela was very disappointed to discover she had missed her prom, and on Sunday she came home.

The final verdict of this hospital stay is this was a bad episode of aspiration pneumonia. Angela's swallow has gotten MUCH worse, and we need to get her back to Boston to visit Dr. Nurko. He is the only doctor in the country who will treat Angela and her Cricopharyngeal Achalasia. The pulmonologist here, who works with people who have achalasia of the LES, doesn't want to touch her because she is high risk. The Pulmo here, who trained under Dr. Nurko and now has a manometry clinic here said no, Angela needs to back to Boston. ASAP.

Insurance says no.

I spent the past week both recovering from surgery and arguing with the insurance provider about getting Angela back to Boston. Guess what I'll be doing more of this week?

Angela conducts the Boston Pops!

We call it the Angela phenomenon. Everywhere she goes, things just HAPPEN. When she was 2 1/2 she was in a national newspaper magazine that went to every house in the country that subscribed to the Sunday paper, her picture the full size of the page. She has been in our local state newspaper a couple of times, mentioned on the radio a time or two, and two appearances on the Jumbotron in Time Square. Then there are all the times when she's just GOTTEN something. I know most people wouldn't consider being the recipient of a Make A Wish "lucky", but Angela sure felt special! Anyway, it's very strange, but it keeps happening to her. 

Today was another one of those days.

We were just getting ready to be discharged from Boston Children's when the Child Life person said the Boston Pops would be playing downstairs, and would we like to come? So we went, injured animals in tow.

When we got there, we were asked if Angela wanted to be their guest conductor! We sat through the show, including Emmy Rossum, star of Phantom of the Opera who came to Boston just for this performance.

We sang some more songs, the it was Angela's turn. As always, Angela put on a good show in her striped pajama pants and "Boston" t-shirt! LOL Conductor Keith Lockhart took her hand and helped her to her place on stage.

She took her conductors stick, and turned to the orchestra, "Ready guys? 1....2....3 Hit it!" and they played.

At the end she took a grand bow....twice. ROFL

Yet another "Once in a lifetime" moment for Angela.

Some pictures from Boston

I sure don't like the pictures my cell phone takes. Unfortunately I didn't think to bring my cameral along! Anyway, my friend Kathleen picked us up and brought us to the Boston Commons (I think that's where we were! LOL) and we really had a nice time. Here's some pictures from the night.

The horse-drawn carriages. This was around 6:30 at night, and the horses looked exhausted.

The tree in Boston Commons. I could only get part of it!

Looking down Boston Commons (I think! LOL) The pictures don't do it justice.

"Cheers" Need I say more???

The Art of Manipulation

A couple days before we left for Boston, Angela was given a stuffed dog that she named Ruby (just like our real Rubee) and she decided Ruby should come to the hospital with her. Then, on Tuesday night my friend Kathleen gave her another dog that looks a lot like our Zurri, so Angela gave it that name and decided Zurri should come to the hospital too.

Some time last night both dogs were injured. According to Angela they'd fallen of the jungle gym and each broken a leg. (I made the mistake of calling them "arms" and was quickly reprimanded because, "Dogs don't have arms! DUH!"

So we head to the hospital, and in the pre-op room Angela informed every nurse, anesthesiologist, nurse's aid, and her GI doctor that her dogs had broken legs. When she was given her bracelet she insisted that her dogs get one too...and they did!

When surgery was done, and Tyler and I were escorted back to the recovery area, there was Angela and her dogs. And, much to our surprise, each dog was supporting a REAL cast. One red, and the other green in honor of Christmas.

She always wakes up from anesthesia with a smile! (but shivering like crazy, which she did for the next several hours.)

And look! Two casted legs! (and these are real hard casts!)

Later, when we got up to the hospital room Angela was insisting that her dogs should have IV's like hers. Yeah...whatever! After awhile Angela was getting agitated and Tyler and I were hungry so we gave her some alone time while we went to find the cafeteria. When we came back, we found each dog did, indeed, have a complete IV set up.

And yet, their care was still lacking! They didn't have EKG leads on like Angela did! The next thing I know a nurse walks in and starts putting "stickies" on them. Here she is telling the nurse EXACTLY where to put the leads.

To say that Angela has the art of manipulation down is an understatement!

Yep, it was tight!

So today's surgery was an endoscopy with balloon dialation of the cricopharyngeal muscle. When the doctor got the scope in there, the area was VERY tight, and he had a hard time even getting the scope in. He dilated it with the largest balloon they have, and we'll just have to wait and see how it works.

She's been drinking liquids today but doesn't want to eat anything yet. That's ok, because it's soft foods only until tomorrow, then we'll see how regular foods go.

If they go down fine she can stick with them. If they don't then she needs to stay on a soft food only diet indefinitely while we figure out what to do next.

All these years she's done a pretty good job of protecting her airway, but it's only a matter of time before she ends up getting very sick from aspirating food. When I told him how well the soft diet went a couple of months ago, he laughed. He said his main goal is to give her a good quality of life, which is to be able to enjoy food pain-free.

For tonight she is crabby and demanding. She's perseverating on wheelchairs for her stuffed animals and driving all the nurses nuts with that. I have a couple cute pictures to post but I left the cord for my camera at the hotel so I'll have to do that later.

Thank you everyone for your prayers and kind words this week. They mean the world to us.

Meeting MORE friends!

I tell ya what! Kathleen is THE sweetest person!!!! I met her online at The Doodle Zoo (a dog forum) a couple years ago. When I posted that we'd be in Boston and was anyone around with therapy doodles, Kathleen said she'd love to help us see some of Boston. Kathleen planned a great day for us, getting passes for the Children's museum, and while Angela and I were doing that Kathleen would take Tyler to the Aquarium.

Unfortunately the tests Angela had done this morning were under sedation, so by the time we got back from the hospital at 1:00, she fell sound asleep and slept until a little after 4. But Kathleen came and picked up Tyler so he could see some of Boston! He had a great time at the aquarium. I had given him enough money to get himself a souvenir, but he bought a gift for me instead. He is the sweetest kid! He brought me this beautiful glass dolphin. I know just where I'll put it when we get home.

When they were done at the aquarium they came back to the hotel and picked us up, and we all went to dinner together in Boston. We had a great time, and saw some really nice evening sights. The weather was beautiful for walking around (which is great since I hear tomorrow its going to turn ugly!) and we just had a really nice time! I have pictures on my cell phone that I'll post tomorrow.

Thanks so much Kathleen, for your wonderful hospitality!

Teaching

When Angela was born, a team of flight nurses quickly prepared her to be airlifted to another hospital. It was during that chaos that the attending pediatrician told her dad, and my parents, that she suspected Angela had Down syndrome.

A few minutes later, her dad and my parents came into the room I'd just been wheeled into after having my belly closed after the emergency c-section. My parents stood at the foot of the bed, and Angela's dad came to my bedside. "Is she ok?" I asked.

"She's fine." he said. But as he said it I glanced at my parents and saw a single tear rolling down my dad's cheek. Never in my life had I seen my dad cry before.

"No she's NOT! What's wrong!" I said.

Her dad struggled with the words to tell me, "Well, they think she might have a little bit of Down syndrome."

Just as I was saying, "There is no such thing as a little bit!" in walked the flight team, with Angela in an isolette. They removed her and placed her in my arms. I couldn't see her face, because they were bagging her to keep her breathing. All I could think to do was turn her hand over and look at her palm. At that single crease that held the truth of her diagnosis. There it was, in all it's glory, deep and plain as day, leaving no room to question if it was a true transverse palmar crease or not. It was, I saw it.

There is one picture of me that day, holding a very tiny Angela (just 4 lbs) with a temperature strip stuck to my forehead and a very dazed look on my face. I don't look like I was crying, and I honestly don't remember if I was. I'm looking into the camera like a deer in headlights. I wonder who took that picture? It wasn't my parents, or Angela's dad. Maybe a nurse? I don't know.....

Anyway, I only held her for a minute before they took her away, loaded her onto a helicopter, and flew into tornado weather to another hospital where the NICU was. Angela's dad followed in the car, and my parents were left standing there with me. Not sure what to say. How do you comfort your daughter in a moment such as this?

"Now it all makes sense." my mom said. "All those kids with special needs you were always bringing home. All those babies you noticed that you didn't know had Down syndrome because you were just a kid, but I knew. Yes, it all makes sense."

She went on. "She's here to teach. She's going to teach everyone along the way that what they thought, and what is supposed to be, is wrong. It doesn't matter who they are, she's going to teach them."

Angela has held true to that prophecy.

First, she taught me about control. I don't have it. You don't have it. None of us have it. We're not in control of our destiny. He is. We can try to manipulate it all we want, and yes, we have free will, but our destiny has already been determined. Angela was destined to be my child, and I was destined to be her mother.

Over the years Angela has taught teachers and educators in general to think outside the box that is "Down syndrome". That not all kids with DS are alike, that there is no such thing as, "Oh they're all so sweet!" Yeah...WHATEVER!

She has taught me advocacy. I *thought* I knew what the word meant, and I thought I was pretty good at it with my boys. But when Angela came along I discovered what the word really meant, and that I had a lot to learn. By the time Angela was 3, I was changing the way our small town school district approached special education, and by the time she was 5 I was changing yet another. And when she was 7 I learned I needed to advocate not only for her, but for me, as a mother, too.

But the biggest area of teaching Angela has been involved in is the medical system. She has broken all their rules. The rules say, "When you have a stroke, it looks like X on the scans." But in Angela's case, the X isn't there, but all the symptoms area. The rules say, "When you have a seizure, it looks like X on an EEG." But Angela broke that rule too, and after two years of my questioning seizure activity, and two years of normal EEG's, her neurologist finally got to see one for himself. "Treat the symptoms, not the EEG" is the rule to follow, not the other way around.

Angela taught her primary surgeon that the rules of wound healing were meant to be broken, and that Angela has her very own timeline.

Angela has taught many doctors that the old phrase "When you hear hoofbeats, think horses, not zebras" does not apply to her...... EVER. She is full of zebras! In fact, I'm pretty sure she's breeding them somewhere. I have learned that when a doctor says something like, "Well, in very rare cases....." That I should start researching those cases, because that's where we're going to end up.

But I have to say, the most amazing thing Angela has taught all around her is JOY. Pure, unadulterated JOY. You can see it when she sits down to an order of french fries, and you can see it when she hugs someone she loves. It is infectious. She melts into you like a warm knife in soft butter, cutting straight to your heart. She delights in thew world around her like nobody I have ever met. Today, after she threatened to arrest her doctor, I watched her, heavily sedated, grin from ear to ear as a nurse appeared out of nowhere with a set of toy handcuffs. The smile was pure JOY.

Yes, I get frustrated with her, just like every mother does. Yes, I get tired of the constant repetition of her favorite game (jail!) or her favorite topic each day. Yes, I want to pull my hair out when she swears or sticks out her tongue for the 500th time that day. But when and opportunity arrises to watch Angela experience something I know she's going to enjoy, I'll go to the ends of the earth to make sure it happens. Because seeing her delight in something like watching a cousin's wrestling match or basketball game, or a dog show, or riding a roller coaster....anything that makes her clap her hands in excitement....does something to my heart that nothing else even come close to duplicating. She has taught me to seek joy in other areas as well.

So, as my mother prophesied 13 1/2 years ago, Angela has indeed taught people a thing or two along the way.

Update

So, the testing today showed definite problems with Angela's swallow. All the muscles in the pharyngeal area are very uncoordinated, and they just kind of do their own thing, none of them in unison. The fact she hasn't been aspirating constantly is nothing short of a miracle! However, watching her face, (hard blinks, watering eyes, closing her eyes completely as she swallows, and also patting her chest, caughing) and watching the monitor at the same time you can see what's happening in there and when.

But after her swallow, once the food gets past the pharyngeal area, her peristalisis looks pretty good! A little on the weak side, but well coordinated. It's the upper stuff that's the problem.

When she drinks liquids there are some spasms happening in the area of the epiglottis. When she drinks viscous liquids (so thickened slightly) there is a spot just BELOW the cricopharyngeal muscle that suddenly tightens.

With solids, as she's CHEWING, that same area just below the cricopharyngeal muscle spasms and tightens, and as she swallows the solid bolus it closes almost completely, yet her epiglotis appears to open. That means food or liquid that has stacked up there can dump right into her lungs! She was able to tell us when she had food stuck, which corresponded with what the doctor was seeing on the computer sensor.

Tomorrow we're going to try dilitation. It may not work, but it's at the bottom of the "least invasive" scale, and something pretty easy to try. If it doesn't work, we'll have to figure out what, if anything, is our next step. Even though she has remained free from aspiration pneumonia for the past 3 years, everyone is stunned. Solid food sits right in front of her airway and liquids splash around the solids and into her airway. It is NOT safe to eat or drink this way, so going back to a gtube, for the rest of her life, is a very real possibility right now.

Meeting Friends! Pt 2

Last night my friend Lisa and her family drove all the way over here to meet us. We're SO excited to finally get to meet them. I've "known" Lisa from the downsyn forum since shortly after her little guy Daniel was born. He's 4, and his birthday is the day after Angela's. I thought I took more pictures last night, but I guess not! I'm sad I don't have pictures of Angela and Elaina, or Lisa's husband Dan, because Angela wanted him to come home with us. LOL

Checking out the room

Love the beds in our hotel. I've slept like a ROCK!

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!

We're here!

I've been up since 3:45 this morning, and I got Tyler and Angela up at 4:00. Our flights were mostly fine, except for the last 45 minutes. There were no snacks served on either flight. I can understand the flight to Milwaukee, since you barely have time to get in the air before you're descending again, but from there to Boston? Plenty of time! And, a note to AirTran, my kid is COUNTING on those snacks getting served since that is kind of like being at a restaurant! Needless to say, she was not a happy camper, and might possibly have taught the little girl behind us a few new words!

So we landed, and since we hadn't yet eaten in the 7 hours we'd been up, AND Angela hadn't had her morning meds yet (needs to have them with food.) Well, lets just say that is a bad recipe and all of Boston Airport's Burger King got to hear about it. Thankfully once all of us were fed, and Angela had her meds, things improved very quickly!

We've been in our hotel room about 45 minutes, and both Tyler and Angela are out cold. Time for me to take a nap too! Toodles!

reduced to tears

Angela went to her dad's tonight, which gives me some time to pack for Boston. Among the items on the packing list, are two large envelopes that came home from school today. One from her homeroom class, and the other from her friends in the DCD classroom.

Angela isn't going to see these until the morning of surgery. Something to help get her mind off the anxiety. Know what I mean? But that doesn't mean I couldn't take a peek myself.

And here I sit, reduced to a puddle of tears from the words written by all of her friends. I won't be able to read them out loud to her without bawling. Good thing I'm bringing Tyler along!

Just a couple more days

The next couple of days are going to be BUSY!!!

First, I have a ton of cleaning to do. Fortunately I really only need to "pick up" because the "cleaning" part is going to get done by someone else. ;-)

I have to go in for some blood work tomorrow morning. So as of right now I'm fasting, which means eventhough I ate dinner, and I can have water, I'm starving and craving a chai latte. LOL

Angela social worker is coming tomorrow afternoon to do the paperwork for Angela's 2010 waiver funding. The paper work is so necessary but such a pain! I can't forget to praise God for it's availability to us though! Especially with the very scary stuff happening in the world of healthcare right now. Some of the changes being proposed for people with disabilities are beyond scary!

I also have to study for a test that I need to take either tomorrow night or Saturday morning. At the same time getting all the laundry done, and packing for Boston!

Saturday afternoon we'll be having ThankChristmas at my sister's. Then come home and (hopefully) get to be early, since we have to be at the airport at 4:30 Sunday morning.

We'll see if I get all this stuff done!

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.............

Scheduling issues

As you know, we've been waiting and waiting for notification of Angela's appointment in Boston, which finally came yesterday. We can't really be picky about the dates!

Guess what? We'll be in Boston when I'm supposed to be taking finals! GAH! Needless to say, I have some rearranging of my finals schedule to figure out!

Got our dates!

Angela and I will be in Boston from December 13-18th. That'll be here before we know it! I wish Dean could come along. We just can't swing airfare for him with Christmas here. Well, that and he doesn't think he can take any time off work. There is that I guess.

So, we're headed to Boston!

More waiting

So the GI office in Boston is open today. But the people I need to talk to are out....until Monday!

....sigh....

Ok, so I am the least patient person I know. Yesterday I called Boston Children's and was told, "The Dr. PROMISED me he'd have the appointment dates on my desk TONIGHT before he leaves for a national conference. I'll be able to call you tonight or tomorrow morning with your appointment date."

Then, early this morning, I had a dream that our appointment is next week on Thursday. I woke with adrenaline-induced shakes, then realized I'd been dreaming. (I hate that!)

But I never got a call last night. And now it's today, and it's no longer morning in Boston. So, I called! And they're CLOSED today because of the holiday. Why didn't the secretary tell me that yesterday (my kid is in school, why isn't the clinic open???) So, I wonder if I'll EVER get called?

Still no news

We still don't have an appointment date at Boston Children's. I was really hoping to hear something on Friday, but it has come and gone, hasn't it?

Apparently the doctors there bring each case before a committee (which means a couple times per week) At that time the review the case, come up with a treatment plan, and prioritize the patients based on the current medical status. I do know the letter written by the specialist here in MN said that Angela needed to be seen on an emergency basis.

At least tomorrow (which is really "today" according to my clock) will find my very busy and without time to sit and wait for a phone call! Angela has an orthodontist appointment, then I'll drop her off and school. From there I have school for me (and take a huge anatomy test!), and teaching canine obedience later in the evening.

Speaking of anatomy...I've been studying for this test, and finally gave up about an hour ago. I guess if I don't know it at this point, I just plain don't know it! I also have to re-take one section from the previous test, and to be honest, I don't have A CLUE what was on it. LOL I sure hope my guessing skills are right on!