Brave as a Princess

We often tell Ella how brave she is. A few months ago she decided that she didn’t like being brave. Apparently she put 2 and 2 together and realized that being brave meant that something bad and painful was about to happen. Unfortunately, she was right. Whenever we would tell her she was brave she would say, “No I’m not!” or “I don’t want to be brave.” Too bad kid…you are the bravest little girl I know. And that was proven last week.

Ella and I just returned from her annual visit to Cincinnati for the EB clinic. We started taking her shortly before she turned 1. We go every year and our purpose has always been to establish a record of where Ella has been, so that when a problem does arise, we know what Ella’s “normal” is and how things have changed. Before we left on Wednesday I told Joe that this is the first time we’ve taken the trip where I have real questions about real problems that have come up. This was the first time I was going for more than just a record of her health…I needed medical advice.

We arrived Wednesday morning and spent the day having a nice lunch and going to a movie…which Ella slept through. We got settled at the hotel and stayed in for the rest of the night. I knew it was going to be a long day on Thursday, and I knew that we were in a new place that would cause some excitement, so I did what any parent in my situation would do…I gave her a dose of Benadryl. The child was asleep by 8 pm and all was right with the world. We woke up early the next morning to be sure and make it to our 9:30 dentist appointment. Ella is very protective of her mouth, so she was not keen on the idea of a stranger putting her fingers in her mouth. Luckily the dentist was named Katie, so we had a good conversation about how she was a nice lady because she had the same name as mommy. Ella opened her mouth wide so Dentist Katie could get a good look.

The best part was after the appointment the dentist went and found 2 princess stickers to give Ella. One of them was of Aurora (Sleeping Beauty) and it said, “Brave as a Princess.” How appropriate! After that Ella was ok with being called brave because, after all, princesses were brave…her sticker said so.

We had a break after the dentist, so we went and grabbed lunch then headed up to where the clinic is held. The clinic is wonderful and terrible all at the same time. It’s wonderful because we get to see everyone at one time. Every doctor that we see knows EB, so there isn’t any time wasted on explaining what the disease is, and every doctor knows Ella…they have read the profile I submitted and always come in prepared with comments and questions. It’s terrible because we are confined to a room for a couple of hours (give or take) and by the end of it Ella is very tired. Thank heavens for the Child Life Specialists who bring toys, videos, coloring pages ,etc to keep her occupied.

We made it through the clinic (Ella was a total champ) and headed back to the hotel. We stopped at Longhorn Steak House where each of us downed a steak…we deserved it. Ella was asleep within minutes of getting back to the hotel.

Friday was the day I was most worried about. We only had 3 appointments, but all 3 were medical tests and 2 of them were medical tests that Ella has never had before. I wasn’t sure how cooperative she would be. Did I mention that my kid is as brave as a princess? She was awesome!

First, she had her ECHO. This was a first for her, and walking into a dark room is pretty scary. The technician brought in a Child Life Specialist who walked through all of the equipment with Ella. She showed her each piece and explained what it was going to do. And she even let Ella touch the sonogram wand. That helped a lot! She also brought in a special princess DVD for Ella to watch during the procedure.

Once the tech got started, Ella was a champ. She was nervous about taking off her chest bandages, and I was nervous because her chest is really rough right now and I had visions of the sonogram wand causing all sorts of additional problems. Fortunately Ella just laid there and watched Tangled and the guy was able to check everything he needed. He went to great lengths to ensure that he didn’t cause further problems. He even got special sterile gel and covered the wand with plastic to make sure she didn’t get any germs. I am SO happy to report that we left with the same boo boos we came with…no new ones. And only a few tears were shed in the process. The best news is that according to the tech, her heart looks good.

Next we stopped and had a great chat with Geri, the EB nurse through Debra. We love Geri and unfortunately, don’t get enough opportunity to talk. It was so nice having a few minutes to catch up.

At this point Ella wasn’t allowed to eat anymore. Yeah, that was not fun. Ella still eats almost every 2 hours, even if it’s just a little snack, so the fact that she couldn’t eat for 4 hours was really hard on her. We went and had her dexa scan (bone density scan). She’s had one before and the scan takes all of 2 minutes, so that was no big deal. Next came the dreaded Barium Swallow…insert scary music here.

I have dreaded a barium swallow since Ella was a baby. I didn’t dread the results, but from all that I heard the drink that they have to drink is terrible and the last time we had to take Ella to get an x-ray she flipped out and ended up getting a huge boo boo on her back. Basically, with a barium swallow the person has to drink this white drink (I’ve heard it tastes a lot like chalk…yummy) and lay on a table while an x-ray videos the drink going down through her esophagus and into her stomach. EB also affects the esophagus, so this test checks for any closings in the throat that may be causing swallowing problems.

The assistant in the x-ray was an angel! When we first arrived she was talking to Ella and asked her who her favorite princess was. After Ella said the obvious, Cinderella, the girl went and found a Cinderella DVD for Ella to watch during the procedure. This made everything go much smoother. Then she got Ella her special drink and let her choose which flavor she wanted (she chose grape). Since Ella was so hungry she was more than willing to drink the stuff, regardless of how it tasted. The only time she freaked out a bit was when they brought the x-ray down and she was in this little tent type thing with the x-ray right over her face and body.

Let me take a moment to say that medical technology is absolutely incredible! Here we were…Ella was laying on an x-ray table watching Cinderella while she was drinking this thick white drink…meanwhile, the doctor, his assistant and I are watching a TV screen where we can see the drink go completely down her throat and into her stomach, then empty into her intestines. No joke, I watched the whole thing!

After we watched the drink they gave her a graham cracker with some white paste stuff on it, and we watched her chew it up and swallow it down. It was so fascinating!

I’m no doctor, and the doctor that was there didn’t give me much information on how her throat looked, but I have seen pictures of several EB throats and in my humble opinion, Ella’s looked pretty good (I realized I just jinxed her). There was one very minor narrowing at the bottom, and a little place about half way through (that I couldn't even see but the doctor assured me it was there), but for the most part she looked good. The doctor even commented on how great the food went down and it didn’t get stopped by anything. This is GREAT news! He’s going to show the video to the Dr. who works with all the EB kids (he was out of the office for the clinic) so I’ll know more after he evaluates everything.

That was our last visit, so Ella and I headed to Cracker Barrel for a late lunch, then back to the room for a quick swim and a night in front of the TV. We spent Saturday at the zoo where I pretty much let Ella ride anything she wanted and eat anything she wanted. I figured that she had put up with a lot, and if she wanted me to pay $5 for a 10 minute Dora show, then she deserved it. We came home this afternoon (Sunday) and are so happy to be back!

So here’s a short rundown on where we are:

v Wounds, specifically her neck wound that isn’t healing – the doctor isn’t too concerned. She said it doesn’t look infected, which is great, but they took a wound culture just to check it out. She also gave me some new wound dressings to try.

v Hands – at this point we’re not doing anything. Her connected fingers really bother Ella and she talks about them constantly, so we’ll see how we feel in the future.

v Teeth – she has several cavities (which I had noticed a while ago) so we’re going back in August. They’re going to completely put Ella under (in the OR) and fill her cavities, do a thorough cleaning on her teeth and take x-rays. This may sound drastic, but I am so relieved to not have to hold her down during any mouth procedure. The thought of that makes me cringe. During this time they’ll also take any blood for testing (another relief…no visit to a clinic that freaks out when they see her) and they will likely give her an iron infusion (since her iron has been low for years and she's taking loads of supplements and it's still only creeping up). If there are any other procedures that need to be done they’ll try to do it at this time so that it’s all done at once. Ella will spend the night in the hospital.

v Weight – this is the biggest area of concern. She’s always been small, but in the past year she hasn’t gained any weight. This is a big problem. Joe, Metti and I have noticed how skinny she is, but the child eats great…I mean she seriously eats (she ate 5 pieces of pizza after we got home from the zoo…no joke). The first thing to look at was her throat to see if there were any strictures causing her to not eat. The second step is to pump her full of as many calories as we can get in her. This means that she has to start drinking Pediasure (disgusting…she hates the stuff) and we have to start sprinkling her food with this stuff called Duocal (basically powdered fat…sounds good doesn’t it). If neither of these work then she’ll likely have to get a g-tube (feeding tube in her stomach). Don’t freak out…it’s really not as bad as it seems. It’s very common among EB kids and it was never a question of IF Ella would need a g-tube. The question was more WHEN she would need one.

I think that’s about it for an overall summary. I say it every time, but it really is such a relief to have Ella seen in Cincinnati. Yes, it’s a plane trip, hotel stay, etc, but for us it’s worth it just to see a team of doctors who know EB.

We are both so glad to be home, and Ella was especially excited to see her daddy! They missed each other a lot! Tomorrow it’s back to our regular routine.