Rising Above It All: 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.