Chances are that before you came across this blog, or another blog for an EB child, you had never heard of it. I had never heard of it. Most doctors have never heard of it.
You can read about all the clinical definitions and medical jargon hubalu at sites like this, or this.
But I was, and am, an EB mom. EB is not a definition to me, it was a real life bleeding, blistering, painful, 11 month ordeal that caused my daughter a short lifetime of pain and tears. It took her away from me, and I hate it more than I ever thought I could hate anything.
Leah had EB because, unbeknownst to us, my husband and I are healthy carriers of the gene. There is a 25% chance that any child we create will have EB .... Junctional EB.
With that being said, this is what EB is:
Think about it this way: it's a genetic (incurable) skin (the largest organ) disease. In the worst cases (like Leah's) it causes damage to both the external (skin) and internal (airway, mucousal lining, GI tract). Most people's skin is held together with elements within the dermis that act as anchors, literally holding the skin together. You know when you walk a lot in new shoes, and you get a blister from the friction? Well imagine that your entire body was able to blister like that, except it does it in response to the lightest touch, or the elastic of a diaper, or a tag from a onesie.
How do you pick up a baby? Under their arms, right?
Not with an EB baby; the skin there is so sensitive it will instantly break down.
How do you change a baby? By doing the double ankle hold and lifting up their bottom half, right?
Yeah, not gonna fly with an EB baby.
This is how I changed Leah: I would undo Leah's diapers in her bassinet, hose her down with a squirt bottle (cause you can't wipe an EB baby) and carry her; naked, to a clean already laid out, pre-elastic-removed diaper on her changing table. Oh and I would slather the back of the diaper in vaseline so it wouldn't chaff her back - oh and then I'd cover her thighs in a layer of diaper cream to protect them.
Every diaper, everyday.
Then there are all the other aspects of EB......
Leah's fingernail grew up, rather than out. To stop her from hurting herself, we had to keep mittens on her hands until all the nails fell off. And when they fell off, they left behind painful raw fingertips that never healed.
EB causes corneal abrasions ...... a condition any eye doctor will tell you is incredibly painful. As in, a Marine will easily cry over a minimal abrasion. Both of Leah's eyes had significant abrasions that took a lot of time and effort to heal. She had to be kept in a dark room. She had to be around cool-mist humidifiers at all times. We had to keep her hands away from her face. We had to put drops in her eyes throughout the day.
EB can effect the dental enamel. At 4 months old Leah had her 2 preciously cute bottom teeth come through. Then they seemed to recede back into her gums ...... when she teethed she seemed to be enormous pain, so much so that she could barely close her mouth. The day before Leah passed she had 8 teeth removed due to abnormalities.
Leah's airway swelled to the point of requiring an emergency tracheostomy. Leah breathed through a tube in her throat for the last 5 months of her life. The tube needed to be suctioned several times an hour, the collar holding the tube in place needed to be changed daily. The collar was padded to avoid causing blisters. We had to change the actual trach every other week. Have you ever stared into a small hole in your childs throat, and then carefully place a small piece of plastic in that hole so that they could continue to breath, continue to live? I have.
Leah's bandages were changed almost every single day. Leah was bathed almost every single day. A bath included: soaking and meticulously cutting off old bandages, squirting her down, and getting her as clean as possible. Then we'd change her trach collar, and bandage her back up. It always took well over an hour to do all this.
You get the idea? It's exhausting .... but I didn't care, everything I did was to relieve her pain.
But I couldn't; no matter what I did. And that's what hurts the most. When you're beyond tired, beyond stressed out, sitting in front of your child, looking in their eyes and you know they are in pain; despite everything you've done; they are still in pain.
That is EB.
Hate it yet?