October is National Pregnancy and Infant Loss Awareness Month and the last week in October is also EB awareness week-so I thought I would talk about our Rainbow Baby, Butterfly Boy. As most of you know our story, Eli was conceived through my sister's donor eggs after losing four previous pregnancies over the course of seven years including the stillbirth of our daughter Riley. My pregnancy with Eli was the first successful pregnancy and the most rewarding 9 months of my entire life. As each month passed we grew more confident that he was going to be our Rainbow Baby and that the storms of our lives would be over. We checked into the hospital at 39 weeks, 1 day and thoughts we would be checking out with our perfect newborn baby boy a few days later. It was the best day of our lives-we were about to become parents-real life parents-not just a mommy and daddy of angel babies-for the first time.....what could possibly be better?
But it hasn't been all rainbows and butterflies the past two months. When Eli was born with Epidermolysis Bullosa (EB) our world was once again shattered. Our perfect Rainbow Baby was born with skin as fragile as the skin of a butterfly. You could only imagine the thoughts and emotions that we have experienced over the past two months as we have learned to adjust our expectations and accept Eli as our Butterfly Boy.
How did this happen? That was the biggest question that we had. We have never heard of this disease before. No one in either of our families have this disease. My sister and husband are both healthy people. My sister has a healthy, beautiful five year old daughter. Eli was perfect on every ultrasound and NST that I had throughout my pregnancy. I was so diligent with making sure all of my medical issues were under control so that they didn't affect him while he was growing in me. Surely God would not have allowed us to conceive a child through my sister's eggs if my husband and sister are both carriers if he would not have allowed us to carry a child with Down Syndrome conceived through my own eggs. And Surely God would not have created a spontaneous mutation in Eli's genes causing him to be the first one to have this disease. But there was only one way Eli could have gotten EB and it was one or the other.
A week ago we found out that Eli has Recessive Dystrophic EB. He has two different mutations in his Collagen 7-one from my sister and one from my husband-causing him to have a reduction or lack of protein which binds together his bottom layer of skin to his middle layer of skin. Recessive Dystrophic Epidermoylisis Bullosa (RDEB) is the second most severe form of EB. This form of EB not only affects the skin on the outside of Eli's body, but also affects the mucus membranes of the esophagus and digestive tract.
Here are a few websites to better explain RDEB:
So how is Eli affected by EB? He was born with missing skin on half of the bottoms of both feet and the entire tops of both feet along with both big toes. When he was being cleaned in the operation room after my c-section, the nurse wiped the skin off of his right hand. Due to suctioning Eli's mouth, he immediately got blisters on his tongue and gums. His mouth blisters initially caused him problems with eating but now he does not seem to be bothered too much by them. They come and go fairly quickly and always pop on their own. He has to use a special bottle called the Haberman where we can control the flow of his milk so he does not have to suck as hard. We also always put a layer of vaseline on the bottle nipples to create an extra barrier and less friction when he is sucking. Every couple of days Eli will get blood blisters that just pop up on his lips and a day or two later they pop themselves and he will have several days where his lips look great.
While in the NICU for three weeks, Eli's little body would spontaneously develop blisters on the sides of his chest. Since he has been home, his chest and back areas are especially sensitive to clothing. There were days the first week he was home that we would find huge blisters on his back, sides of his belly, and armpits from the friction created from his clothing, the seams of the clothing, or picking him up by the chest area. These areas are all healing but are definitely showing signs of scarring. If we think for one second that it would be okay not to wrap his armpits and torso we are sadly very mistaken and find several new, smaller blisters not even an hour after the dressing change.
We have been experimenting with several different ways to wrap Eli's feet and hands. We've tried the way the hospital taught us-some days we saw improvements and other days the products we were using seemed to be more damaging to his skin. We then tried the way another EB mom told us she wrapped her son's feet and saw some significant progress. We even made our own concoction cream which seemed to be working. But the product we used to cover his feet left big pimply looking marks all over his feet. So then we started trying some products we were sent by an EB organization in a care package and have starting seeing even more improvements with the color of his skin looking less purple and the texture of his skin less pimply. However, one thing that we have noticed is that we can never get too excited about the progress we are seeing because the moment we do, the next day everything has gone to hell in a hand basket. His feet get mostly blood blisters on them from the pressure of holding him too tightly or from him kicking while screaming his lungs out. At the end of last week his feet were looking rough-but they made some really great progress in just a few days with the new products we are using. His right foot especially was looking amazingly better than it ever had been. But during our dressing change today I did not have his foot dressing completely secured before starting to wrap his calf area because I was going to secure the foot with the gauze I was using on the calf. The nurse was holding onto the foot though while I was trying to get under the leg and his dressing started slipping off of him. It ended up falling completely off before I got done wrapping the calf and when the nurse was holding Eli's ankle so I could finish wrapping the calf, I looked over at his foot and watched a huge blood blister form up the side of the ankle and foot and over top of the foot. It was devastating to me as his mother to see his "good" foot turn into one giant, painful blood blister. The nurse felt horrible too and Eli screamed bloody murder until we were completely done covering his foot back up. This is how fragile Eli's skin is-it is never fully healed and in an instant everything can go to shit. Eli's toes often develop blood blisters between the big toe and index toe (is it called the index toe like the index finger??). Since putting strips of Mepilex Transfer (just a wound care product name) in between the toes that has been a lot less dead skin which means less messing between the toes which means less trauma to the toes which means less blisters between the toes! His big toes are hideous and the one on his right foot is fusing to the bottom of his foot-the more I try to clip the skin the more trauma and blisters that I cause. Toenail wise-I know his big toes have no nails and some of the others only have the nail stub while some of the others have some fugly nails...yes, fugly. I know-I just said my child has fugly toenails. I promise-I still love him regardless of this! Now the hands-they are just like the feet. Some days they look amazing. Other days, they look like someone beat our child with a club. His right hand is definitely scarred where the skin was rubbed off at birth and his left wrist is scarred from where a nurse put a pulse ox directly on his skin in the hospital-that's a big no, no. We put strips of Mepilex transfer between his fingers to keep them separated but this hasn't helped with the dead skin issues like it has the toes. His fingers, like his feet, blister so easily from the slighted touch. I feel horrible that my child can't have his hands unwrapped so he can start to play with toys and he has never been able to hold onto my finger for longer than a few seconds a day while we are unwrapping and rewrapping. One of the things I looked forward to the most was having my son's little fingers wrapped around mine....and we don't get to experience that....and now I'm crying thinking about how unfair EB really is! I absolutely hate EB. I worry all the time that Eli won't get to be the rough and tough boy that little boys are meant to be. When we wrap up his hands, to make myself feel better I guess, I always tell Eli we are putting his boxing glove on him since his hands get wrapped up into a fist. We have had to put rolled gauze in his hand and in between his thumb and index finger because he does try to keep his thumb tucked in and his thumb was fusing to his hand. Fingernail wise-he got a blood blister under one of his middle fingers almost 2 weeks ago and the nail still is barely hanging on. Whatever nails he loses he will not grow back.
The future is really unknown for Eli. Some EB kids learn to walk and other's never can walk because their feet and legs do not heal enough to put pressure on their feet. Even though Eli doesn't have trouble eating right now, it doesn't mean that he won't have problems once he starts eating solid foods. Most EB kids end up with g-tubes for feeding or have multiple dilitations of their esophagus to stretch it out so it doesn't close and cause them to choke when eating. Eli seems to have a "big mouth" right now-when he screams it's a full open mouth torturing scream-so I don't know if he will look like most EB kids with small mouths that can't open very wide and crooked teeth from the blisters on their gums. Sometimes EB takes kids in childhood, sometimes in early adulthood, and sometimes they live a normal lifespan. It's the scariest thing to think about losing another child-and especially a child I carried a full 9 months, gave birth to, and have fallen completely in love with. I honestly don't think I could handle losing Eli at a young age. Someone would have to put me in a straight-jacket. I say that as I am sitting at work-and I am a crisis worker in a hospital who assesses and admits people to our psychiatric unit!
Dealing with EB is very difficult. I wrote in my last blog about my breakdown that I had the first week Eli was home-when I was screaming at God for giving me an imperfect child. Eli's skin got so much worse the first week he was home that I literally wanted to crawl into a hole and scream and cry and curse the world. But we have come to see Eli as our son with EB instead of just seeing him as his disease. We love cuddling with him. The best feeling in the entire world is seeing him smile. It is a good reinforcer that we are not horrible parents that cause our son pain every day and that he is a happy baby. I can't wait to hear him laugh out loud for the first time-he is starting to make some funny sounds and I know the laughing is just around the corner. As much as EB scares the living daylights out of us-we won't let this disease steal our happiness or our son's happiness. We won't allow this disease to prevent Eli from being the best he can be, learning how to do things that other babies and kids can do, or being involved in any activity that he wants to be involved in as he gets older. He will learn what his limits are and will figure out how much pain he is willing to endure to do the things he wants to do as he gets older. I am 100% positive that he will be the toughest kid on the playground one day. And I am 100% positive that this boy will have a profound impact on our community and people who follow our story from around the world. He is not only OUR Rainbow Baby, Butterfly Boy-he is all of ours!