What is EB?
This is the first in a blog series on Epidermolysis Bullosa (EB) as part of EB Awareness Week (October 25-31).
Some would describe EB as horrific. Some use words like “Painful”, “devastating”, “horrible”, “dreadful” or “insurmountable”. In reality, for several who do not know what EB is, there may simply be no words to describe it. EB is not like some disorders that are noticed later in life and then becomes progressively worse. EB is not just something that can be fixed with surgery. EB starts inside of the womb with painful blisters. The pain cannot be eased. Nine times out of ten, the mother is completely unaware that this is happening to their child. It only gets worse from there.
To put it simply, EB is a rare genetic condition affecting an estimated 13,000 people in the United States. For the most part children are impacted, but the disorder is lifelong. In some forms, life expectancy is only a few months. In others, left expectancy is about 30. The less-known cases have a normal life expectancy. Though there are treatments for EB, there is no official cure for EB. By cure, I mean never having any more symptoms of the disorder and ensuring that future generations are never to be impacted by this disorder again.
In all cases, though, friction on the skin, whether it be pushing a grocery cart, walking or even a simple hug, can cause the skin to erupt in painful blisters. In some instances, the skin completely comes off. In almost all cases, EB is not detected until birth. Just delivery can remove the skin from a child with EB. Then, there’s the wiping of the child, diapering, the identification bracelets, handling, hugging, bundling… all these things that happen in the first five minutes of an infant’s life can cause serious damage to a child with EB.
Epidermolysis Bullosa is fatal. The skin must be bandaged constantly to prevent infection and to protect from further pain. Imagine the pain you would feel if you scraped a small section of skin off of your leg. Now, imagine how that would feel if that scrape were your entire leg. Now, imagine what that would be like if your leg just wouldn’t heal and were constantly scraped open over and over again. This is the life of a child with EB.
When the skin of a child with EB tries to heal, sometimes, the healing is ‘overdone’ and new skin forms between fingers, causing then to fuse together. Healing wounds on the mouth causes the mouth opening to become smaller. Sometimes, eyes grow shut and sealed. Often, the esophagus strictures.
In a perpetual state of healing and re-injuring, the body tends to become anemic and sometimes starts to shut down. Sometimes, the pain is just intolerable. EB then potentially becomes terminal.
EB takes not only an emotional toll on every family impacted, but also a financial one. Items necessary for lifetime survival are sometimes not covered by average health insurance. Expenses then have to come out of pocket or other non-profit charities.
I’ve lost count of how many people I have personally known who have lost their battle with EB. Many I’ve cried hours over. Some I just bow my head, knowing there is no more pain for them.
Each October, the last week of the month is known as International EB Awareness Week and became officially so in 2006. Over the next few days, I’ll give links about EB and I will also tell a deeply personal story about how EB entered into my own life and still impacts it today.