Married in Sequined Slippers (Part I)

What is Epidermolysis Bullosa?

Some would describe Epidermolysis Bullosa as horrific. Some use words like “Painful”, “devastating”, “horrible”, “dreadful” or “insurmountable”. It’s also been described as “The worst disease you’ve never heard of”.

But see, I don’t like the word disease. It tends to give people the impression of it having contagium, much like the plague or leprocy. Individuals tend to shy away from a disease and become more accepting of disorders. “Disorder” implies that something went wrong in the genetic coding of someone, or simply, something went a little askew in utero, which is exactly the case with EB.

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, healthy eating or Holy water.

EB starts inside of the womb with painful blisters and raw wounds. 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 life-long. There is no cure. It cannot be outgrown. It is within the genetic coding and thus, is permanent.

In some forms, life expectancy is only a few months. In others, left expectancy is about 30. The less-known, but more common cases have a normal life expectancy. Though there are treatments for EB, as I stated above, 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 closes up. Due to being 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. 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.

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.

Each October, the last week of the month is known as International EB Awareness Week and became officially so in 2006. The aim behind EB Awareness Week is to not only inform the general public about what EB is, but also to inform doctors of how vastly different EB is from any other illness; that whatever heathcare instinct one may have when they see a blister or abrasion must be thrown out the window when caring for someone with EB. Epidermolysis Bullosa is a completely different nemesis.

Look for Part II later tonight…


About gespurr

Emily was born in Southwestern Louisiana and has moved over 20 times in her life through nine different states. Most of her life was spent in the Twin Cities of Minnesota, where she met her husband and had her only child. Both she and her husband are also only children. She graduated from Stillwater (MN) High School in 1992 and from the University of Wisconsin in 1997 with a BS in Journalism. Three years later, she met her husband, George, and they married in 2002. Their daughter, Kathryn, was born early in 2004. She relocated with her family back to Arkansas in 2005 after being away for 30 years. She currently works as a customer service representative for an insurance company and lives in North Little Rock. When not taking care of her daughter she is either cooking, working, cleaning house, sewing, gardening, knitting, crocheting hiking, traveling or spending time with her husband.

Posted on October 29, 2012, in Current Events, Epidermolysis Bullosa (EB), Family & Marriage, Parenting and tagged , , , , , , , , , . Bookmark the permalink. Leave a comment.

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