What is EB?

When Brody was first born and we were trying to explain to people what he had, what he looked like, prognosis, etc, it really was hard to do this. We could describe how his wounds looked all we wanted, but until someone actually saw him or saw a picture I don't think they really understood. In the beginning people would always ask us, "Well, he'll grow out of this won't he?" ummm, no. Or, "How long are you going to have to bandage him?" which all we could say to this was we really don't know, which we still don't. I mean, I expect him to be bandaged to some degree his entire life, the severity of his type can't be determined by any kind of medical test. We, he, will just have to take it on a day by day basis and see what needs to be bandaged and what doesn't. There are many paths this disease can take: wheel chair needed for mobility, trach tube for breathing, feeding tube for eating (which is usually because of a nutrition thing but for Brody it was because he was aspirating), eye problems, teeth problems, fingers and toes can grow together (webbing), fingers lose mobility and slowly close inward towards the hand and stay like this (mitten deformity), the list goes on. Brody's left foot I guess is a combination of the last two things. Chuck calls it his left nub. Basically, the skin grew in so quickly that it grew completely over his toes. He has toes, you just can't see them.

I may not be completely correct, but from everything that I have read about EB, there are three main types: Simplex (least severe), Dystrophic, and Junctional (most severe). The difference in these types, besides level of severity is where on the skin (between which layers the protein needed to hold the skin together is missing) blisters/wounds can form and some forms affect the inside of the body more than others.Then, there a many subtypes for each one. Brody's is called Recessive Dystrophic Epidermolysis Bullosa-Hallopea Siemens. Which, of the Dystrophic subtypes, this is the most severe. Brody's skin is missing the protein called Collagen 7 that is basically the glue that holds the top two layers of skin together. Any type of friction against his skin causes it to separate and will either form painful blisters (which must be popped as soon as possible) or just cause his skin to come off altogether. When Brody was born, he was missing 1 to 2 layers of skin on his hands from the wrist to the fingers, his right leg from about half way up the shin to the toes, and then his left leg from mid thigh to the toes. The best way to describe is this is that it looked like he had 2nd and 3rd degree burns in these areas. You could even see his veins in some areas (as opposed to brain which is what a newscaster quoted me as saying GOOD GOSH!). His left leg was the worst by far and still remains his worst area. On his hands and right leg, most of that skin has grown back. The skin in these areas is very purplish deep pinkish looking in color and is still very thin. Basically, it looks scarred. Much of the skin on his left foot/leg has grown in but it isn't attached as well as the skin on the other extremities and comes off very easily and exposes very painful and very raw areas. I have a feeling this leg is going to be like this for quite some time, if it every actually heals. His underarms are also very sensitive, no matter how careful we are that we do not pick him up using the underarms, they still have raw areas.

I've decided to post a couple of pictures of Brody's wounds, not to frigthen, offend, or gross out anyone. But instead so people will know what I have described above actually looks like. Like I said earlier, describing what it looks like to someone is one thing but for them to actually see it and understand it is another. So, I apologize now for the pictures. If you do not wish to see them then don't go scroll down any further.

These were his wounds when he was first born.

These are wounds to his abdomen from where they kept trying to put monitors on him and they kept coming off.

This is a picture that I took a few months back. His hands still look pretty good.

Feeding tube

So after the swallow study showed that Brody had been aspirating when he eats, Dr. Lucky and others from the EB team decided it was best to admit him into the hospital that day to get a g-tube instead of sending him home in case something happened before they could do the surgery. This was on Friday, March 30th and he did not have the surgery until late Wednesday afternoon, April 4th, and then he was dischared from the hospital on Easter Sunday, April 8th. So we were in the hospital for a total of 9 days. The reason we had to wait this long is because both doctors that would be involved with the surgery were out of the country until Tuesday, April 3rd and then we had to wait for an opening in both their schedules as well as the OR schedule. And then we had to wait a good 3 days after the surgery to make sure the tube was working properly and Brody was adjusting to being feed by the tube.

Using the feeding tube to feed him isn't really that big of a deal and once you do it a couple of times and get over the initial fear of the whole thing, it's pretty simple really. Now, the fact that the balloon holding it inside his stomach could deflate and it could come out or if it gets caught on something or tugged hard enough that it could come out, still freaks me out a little. But they showed us what to do if that does ever happen.

Brody will be going back at the end of June beginning of July for follow up appointments with the ENT, ortho, dental, and possibly the EB clinic. Either at that appointment or sometime before, he will also be getting what is called a mickey button on his tube. Right now he has about 4 inches of tube coming out of the hole. When he gets the button, it will lay flush with his skin and then I guess we will attach something to it to simulate what it is like right now. I will be glad when he gets the button because every time he sees it he tries to grab it and tug on it. EEEEK!

Here is a picture of him right after the surgery in the recovery room.

Spring Break 2012

Even though we couldn't afford to go away some where for spring break, I still wanted to do a few fun things with Makenna. On Sunday, March 25 we went to the zoo. It was a beautiful day...not too hot, not too sunny, the perfect weather for Brody. On Monday, Brody had a follow up with the heart doctor. We found out that the hole in heart had closed and also the artery that wasn't carrying enough blood to his lunghs was fully functional now. YIPPIE! From there we went to Incredible Daves. After about an hour Makenna came up and said, "Mom, can we go home now, I'm just worned out."  :-) On Tuesday, we just stayed home and did a little housework and packed for our Cincy trip. On Wednesday we came up a day early for Brody's dermatology/ortho /ent appointments and took Makenna to the Newport Aquarium. We had a good time. That place is so big and so neat. Big Mike the alligator was back and he is GIGANTIC! From there we went onto the hotel a little early so Makenna could swim some before it was too late. Thursday we head to Cincy Children's Hospital for what we thought were going to be routine checkups. We had to get Brody measured for new braces: 2 for his right leg, one during the day and one at night. The one at night will keep his leg straight. He is also getting one for his left foot because it points almost straight down, like a ballerina stance. His derm appoinment went well. Dr. Lucky is always so happy with what she sees when she sees him. We don't have to go back to EB Clinic for 6 months!!! Too bad ortho, ENT, and the surgeon (read on) want us back in 3 months. POO! Makenna had to get her blood drawn for the genetics testing. Although she teared up just a little, she didn't cry at all. Brave girl...The ENT doctor decided to do a swallow study on Brody the next morning to see why he coughs when he takes a bottle. Everyone expected to see strictures in his esophogus from the EB. But instead, he has been aspirating into his lungs when he eats and has been doing this for quite some time. That drew an immediate admittance into the hospital for Brody to have a scope of his throat done and also a feeding tube put in. Both doctors that were to be included in the surgery were out of town so we had to wait until late Wednesday afternoon before the surgery took place. So, here I sit at the hospital, a week later, so beyond ready to go home. I haven't seen Makenna since last Friday, I haven't slept in my own bed since last Tuesday, I've missed this whole week of work (without pay I might add EEEEK!), and I'm just over being here.

I didn't take all that many pictures of our spring break adventures, but here are a few.