Blog · October 17, 2015

Side Effects of RDEB there was one question that I was asked more than any other in regards to my son’s condition is why he and others with RDEB look so pale and why they don’t seem to age per se, they always look very young. Even when Nicky was interviewed by FOX11 earlier last year, the most pressing question that came to me from the reporter and later by some of the followers of Nicky’s page is why Nicky looked so young. “He does not look 18 at all, why is that?”

There are many answers.

For one, most RDEB patients have some sort of iron deficiency. This is because iron is lost from the extensive deep wounds and serious bleeding that occurs, and loss of iron has the side effect of making the skin pale. Normally after Nicky gest an iron infusion he gets rosy cheeks, so I can always tell how good his iron levels are just from looking at his face.

For two, because of the amount of scars that occur everywhere on the body, the skin loses its elasticity. This explains why fingers often get “trapped” in the hand, the skin is not flexible and does not allow the normal growth of the fingers, and as the bones try to grow, they curl up in this tough, tough skin. This also makes the skin of the face tighter, hence making their face look ageless, keeping the mouth small. Inside the mouth the tongue webs to the bottom of the mouth and skin also webs into the mouth, making it difficult to talk well, or eat for that matter.

Scarring is an understatement in RDEB. I have scars from my pregnancies and c-sections, but none of my scars look as horrible as Nicky’s. The skin gets incredibly tough, leather-like on some areas. It’s still susceptible to get blisters though, make no mistake. These blisters do occur and they are very painful to poke. The skin in those areas are then open wounds that take forever to heal.

Of course, the relentless attack on his body by nasty wounds that take sometimes years to heal, means all the calories he intakes go directly to wound healing, so puberty is often delayed, compounding to the “young” look. Nicky is nearly 19 and is just now showing “some” pre-puberty signs. His little brother, who just turned 12, has more advanced pre-puberty signs than he does.

These are just a couple of side effects of EB. Just missing this little protein, Collagen Type VII, does so much overall damage, and that is because it affects the skin, which is the largest organ on our bodies.

So, next time you see a man/woman with RDEB that looks like a young teen… now you know why.

Love & Light,