Rare Disease Awareness - Post 2

Blisters.

If we break down the meaning of the 2 words Epidermolysis Bullosa we get epidermis (which is the outermost layer of the skin) and bullous which is characterized by blistering. So, simply translated, EB means skin blisters.

Graham has a mutation in the COL 7A1 gene which produces collagen. Collagen is the main protein found within our skin and other connective tissues within our body that holds the layers together. Without collagen, the layers of the skin blister and tear very easily, hence the term EB.

Yesterday I discussed the preventative wrapping routine that Carl and I perform after each of Graham’s baths. Although Graham has very little skin exposed, blisters still occur daily. Despite our very best bandaging efforts, our very delicate and deliberate handling of him, and trying to be proactive with choosing soft clothing, toys, etc. the blisters still come. I would be lying if I said it isn’t defeating some days. We can be as cautious and careful as possible but we can’t stop the blisters from rearing their fluid-filled heads.

Some blisters are quite small and minuscule and some are quite large. For example, Graham has had blisters that wrap around his whole foot and ankle. Some are filled with clear watery fluid and others are dark red and filled with blood. What it’s filled with depends on how deep in the skin the blister formed.

As soon as we see a blister we need to lance it. If we don’t pop the blisters they could spread and become larger in size. This requires us to constantly do thorough inspections of Graham’s skin, especially at every diaper change when we remove all his clothes and check for any affected areas of the skin. Once the blister is lanced and drained with a small hypodermic needle we then apply medical-grade Vaseline to the area and cover it if need be.

The blisters may keep coming, but so do the infectious smiles from our sweet boy. He is our tiny hero and we love him so deeply, deeper than any blister he will ever get. We shall keep marching on with hope in our hearts, popping one blister at a time until we find a cure.

"I survived because the fire within me burned brighter than the fire around me."

#thisisEB