I grew up in a third world country where the exotic butterflies are exquisite! My favorite had wings like brilliant sapphires on thin blankets of black velvet. They would flit by in glorious beauty and no matter how many times I saw them, they still took my breath away. It is hard to describe something so beautiful and splendid, yet still so delicate.
That is how I feel as I write a little bit about our “Butterfly Child”. This is a term that has been coined for children with a rare genetic disorder that results in skin “as fragile as the wings of a butterfly”. Our newly adopted daughter, Abigail, is a “Butterfly Child”; she has Epidermolysis Bullosa.
If you have not heard of it, you aren’t alone. Epidermolysis Bullosa is frequently called “the worst disease you’ve never heard of.” I had no idea what it was until we saw our daughter’s file. It is considered the worst disease because it affects your skin, which is not only the largest organ in your body but also the primary barrier to infection and a part of daily life.
A very simple description of this disease is that the outer layer of the skin (the epidermis) adheres to the deeper layer of skin (the dermis). In patients with EB the layers don’t adhere properly so the outer layer of skin easily blisters and tears because the epidermis is “floating” with no anchor.
EB is varied in it’s presentations and how it effects the individual. There are four main kinds of EB and varying degrees of severity in each kind. With some forms of EB the blistering is not limited to the skin, but also affects the mouth, esophagus, and internal organs as well.
The secondary complications of compromised skin and tissue can be devastating. In some forms of EB portions of the skin are completely missing. Sometimes the image most closely related is that of an active burn victim, which can cause disfigurement, disability, and even early death (some even in the first few months of life).
There are many people with milder forms which, while they can be extremely difficult and painful to live with, are not disfiguring or lethal. Our daughter has a “mild” form, but please do not confuse mild with easy or painless.
Let me be clear that EB is not contagious, it is genetic; there is no way to “catch” EB. Unfortunately there is also no cure for EB. Pain management, wound prevention, and wound care is all that can be done at this time.
For us and our daughter the routine is:
– Daily checking for blisters, tears, and infection.
– Popping the blisters that were found with a sterile needle because the skin does not have the anchoring to contain the blister and thus it grows at an alarming rate.
– Rubbing/patting coconut oil all over her skin which is not only good for her, but is fantastic for bonding!
– Putting Aquifor on her wounds and wrapping the wounds with non-adhesive gauze and coban wrap; anything sticky (ie bandaids) will blister or rip the skin when removed.
– Making sure all her clothing and shoes are soft and do not rub. This includes making sure that there are no seams, tags, lace, sequins, elastic, etc. that might rub and cause blistering.
– Making sure that she weekly gets a chance to swim in salt or chlorinated water or has a vinegar bath to help hydrate while killing infection.
This routine can take as little as 15 minutes all the way to an hour depending on the wounds and care needed.
One of the side effects of EB is that it effects our daughter’s teeth and mouth. She has no tastebuds because of the constant blistering on her tongue; thankfully, this does not keep her from enjoying her food and having her favorites. The EB is involved with the enamel on her teeth, which means cavities are a constant battle. EB also affects the connective tissue in her gums which our dentist has warned may mean she looses her adult teeth early.
Like many people with EB she has no fingernails or toenails. Although she will never get to paint her nails, it is a blessing in disguise because she doesn’t scratch herself.
As a parent you get accustomed to injuries that otherwise might be traumatic. I never thought I would hardly bat an eye when all of the skin was ripped off my daughter’s entire little toe by bumping the edge of the pool. But you can’t keep them in a bubble, which means as a parent, you simply care for the wound, love on them, and help them keep on living.
There are several sites in which you can find information about EB, but I wanted to write about it myself because I have found that although this condition is not “pretty”, those with this condition are truly beautiful! EB is a condition that they deal with, it does not determine WHO they are. This is a disease she will always live with but it is not her life. Our daughter’s skin is fragile but her spirit is so strong!
As I dress my daughter’s wounds each day I sometimes think… she might have been better off with a “quiet, sit around the house” kind of family. But as she jumps up to race outside for the next “adventure” I realize… nope, she deals with the pain because she gets to do the things she loves.
When we first adopted her I had lofty ideas of helping to heal her skin and that she would rarely have any more blisters or wounds. I quickly realized that is not only impossible, but it is not my job as her parent. Our job is to love her, to care for her and do everything we can to enable her to live life to the fullest.
If that means special shoes that don’t rub, so be it.
If that means we buy a membership to our local recreation center so she can swim all year round, then that is what we do.
If it means Daddy carries her on some of our hikes, then it is worth it.
She embraces life with a gusto I admire! She swims, swings, plays with friends, laughs, climbs trees, plays dress up, rides bikes, and on and on. That being said… her skin is fragile! Which means that sometimes something as simple as jumping on the trampoline will result in a large chunk of skin that simply peeled off her leg because her shoe brushed it. Sometimes allowing her to do things means more bandaging afterward, but I can not squelch that beautiful spirit.
I have gotten to know several people with EB and they have more things in common than just this condition…
They embrace life and live with an internal determination and tenacity to hold on to hope each day.
They have an amazing ability to smile and share joy, despite living with daily pain.
I think it is very appropriate that they are called “Butterfly Children” not only because of the fragility of their skin, but because of the beauty they bring to life!
– guest post by Sarah