Tuesday, October 25, 2011

Raising EB awareness--the first few weeks

  My goal this week is to post every day or two on different reasons of why raising EB Awareness is important to Brian and I. Today, I'm starting with:   The new parent

  Every parent has their own story of the birth of their child...and every mother loves telling them. Funny, sweet, memorable. Daylon's birth story is not one I speak of often. It's not just because it's not a funny story, but because how we felt during that time brings up very personal feelings and I don't know that there are enough words to describe how we felt. For the sake of EB awareness week, I'm going to share our story with all of you.
   After we had the twins, we contemplated being done with having children. Four always seemed like a good number for us. As we prayed for an answer, we knew that there was one more child to join our family. The pregnancy ran like a normal pregnancy for me- complete misery. :) But after having three other pregnancies, I was okay with things. We were just so excited to meet our little boy...a brother that Caleb desperately needed to stabilize all the girly in our house.  At 38 weeks my uterus measured 8 weeks further along than I was and since my past single births were 9lbs 11 oz and 10lbs 3 oz, they were preparing for another big baby. Everyone in the operating room was making a guess at the baby's size and laughing. Feeling like a seasoned veteran, I wasn't the least bit nervous and just laughed as people threw out ridiculous answers like, "14lbs!". As my OB cut my belly and reached in for Daylon, I remember him saying, "Ok! Let's see what we got!".  I couldn't see over the curtain (and my mirror only let me see the back of the baby), but I heard the room grow quiet. The only thing I heard was Brian say, "What happened to his face?" . Once the doctor turned him around, I was shocked that his face was covered in blood and the skin on the tip of his nose was missing. I held him for a moment and kept asking, "What happened?" While the nurses did admit that they had never seen anything like it, they were sure he had just scratched too hard in utero. Brian followed them back to the nursery and when he visited me in the recovery room, he brought pictures of his weird, long finger nails. I remember thinking that they looked like Edward Scissor hand's or Freddy Kruger's nails. When they brought Daylon to the recovery room to visit with me, the stay was short. He got to meet his big sister and two grandmas, but his lips turned blue and he had trouble breathing. He was taken away overnight for observation. In the following months, we would learn that this was normal Daylon behavior.
   The next morning Daylon was brought back to our room and because our pediatrician was in his last week of recovering from hip replacement surgery, a pediatrician new to us looked Daylon over. I told her that I was concerned about his nose and his nails that were now looking to lift off of his fingers, but she told me that he probably rotted his nails by sucking on them and their length was more than likely due to being further along in pregnancy than we thought. I just kept thinking, "How can that possibly be?". By that afternoon, when I changed his diaper there were blood blisters all over his bottom and my heart just sank. Why is this happening? One of our biggest blessings that stay was to have the same nurse almost every day we were there. She watched these blisters form on his bottom with me and agreed that something wasn't right.  I called the "stand in" Pediatrician and she told me it was probably just a rash. She then said that she couldn't believe that our nurse was encouraging a paranoid mom. I was so upset with her. By the next day, the blisters were on his elbows, feet, back and covered his squirming legs. I told my OB, the anesthesiologist, our visitors and every nurse that entered that room begging for help and they all said the same thing, "I don't know what that is. You should ask the pediatrician."
  You know that dream that everyone has where you're running and running and someones chasing you or you're trying to get to someone and you never get to where you want to be? Then you wake up all sick and anxious? That is EXACTLY what these days felt like. I was begging for help, in one of the best hospitals in CA, and it was like no one could hear me. I felt like I was going crazy. He was not my first kid and I was not some paranoid mom. Something was wrong with my baby. I knew he was off recovering, but I called our Pediatricians office and asked that he come see Daylon.
   While the hospital doctors didn't know what was wrong with Daylon, they did know something was off. I had to lay on the hospital bed while the doctors looked over every inch of my body looking for an infection or STD that I may have passed on to my new son. It was so humiliating ...but at least I was kind of being heard.
   Early the next morning, the nurse came in to remove the little round band-aid that had been placed on Daylon's thigh his first day of life after he got one of the Hep vaccines. When she pulled the band-aid off, the skin under it came off too. Daylon screamed in pain and the nurse and I were horrified. I remember how sick I felt. How I just wanted to go run and hide somewhere. Thankfully, about 30 minutes later our wonderful pediatrician, Dr. Blumberg walked into the room. I was sitting on the edge of the bed preparing to walk Daylon back to his bed (I'm still recovering from the C-section) when he entered. I remember the second I saw him, I was so relieved that there was finally someone here who would listen to me that I started bawling. I pleaded with him to "please help my baby!" Then continued, "He looks like someone dragged him through the Amazon and gave him some foreign disease!". He looked him over and said he wasn't sure what he had, but he would go get help. He returned about 10 minutes later with a NICU doctor who examined Daylon over and diagnosed him with Impetigo. The diagnosis just didn't sit well with me. I had seen Impetigo several times working at a preschool in previous year and it never looked like that to me. Dr. Blumberg looked at the NICU doctor and said, "I was afraid he had Epidermolysis Bullosa." ....and that was the first time I had ever heard that name.
  We were discharged from the hospital the following day with antibiotic cream to apply on the blisters several times a day. As the next 24 hours passed, the blisters not only didn't go away, but grew in size and multiplied. Around his mouth and chin was covered in little blisters and there were even blisters growing in his mouth that were painful enough to have him quit eating. The morning after he was discharged I sat on the couch of my parents house (thankfully, they had us spend the night so they could help with the other 4 kids) with Daylon trying to change his outfit for the 10th or so time (as the blisters would pop from movement, he would get wet and need to be changed). He was screaming in pain, I was crying and Brian was pacing the living room. I had this sinking feeling in my soul that he was very sick and dying. We decided then and there that he was going back to the doctors. Brian video taped Daylon's body before we left. Excuse the bounciness, we were a bit anxious as you may have guessed.

  The following video may be too graphic for a few viewers-

   Thankfully, it was Dr. Blumberg's first day back and he got us in right away. He told us that he thought he had Epidermolysis Bullosa, a rare genetic disease that he had only seen once decades earlier as an intern in Los Angeles. He sent us to see a dermatologist immediately after. The dermatologist (which judging from their clientele in the lobby was a botox-y kind of dermatologist) gathered his fellow doctors and they examined Daylon and then left the room. Two of the doctors returned a short while later and said that they believed it was something genetic, but didn't know what, so they were having him admitted at the local children's hospital for testing. Infectious disease among others examined Daylon and once again, I was checked and then questioned... and then Brian was questioned. I really think they were sure I was going to suddenly confess, "Oh yeah! And then there was that 6 week stint during my pregnancy when I prostituted myself out...". The idea that two people would wait until marriage and stay faithful to each other was apparently too much for them to believe. Anyway,almost a full week passed. Sixteen...that's right...sixteen doctors later, we met Dr. Metz, a pediatric dermatologist who took a punch test of Daylon's skin.
  She gave us the DEBRA website and told us to only look at this website. We were warned to stay off the Internet because it could drive us crazy and scare us as we waited for the results. It was difficult, but I was obedient. I printed off the list of the close to 20 different types of EB and went through each symptom guessing what he might have. Even just reading on Debra scared me. Reading symptoms that could come, discovering that our touch was doing these horrible thing to his body, learning that several of these subtypes carried shorter lifespan...is was so much to process. Honestly, though; if you mix this sort of news with the hormone changes a woman experiences shortly after giving birth, it's absolutely horrible for her. You carry this baby for 9 months knowing that it will all be worth it when you hold your beautiful baby and you start to plan what your life will be like with this new baby: pick out cute outfits, imagine those giggles from blowing raspberries, rubber duckies and bath time, etc, etc and in a second the dream is not only not possible, but your baby is sick and you don't know how to help or who to ask for advice because no one you know has heard of what your child has. I seriously remember watching a Pampers commercial with this crawling baby and the whole time I just thought, "How will he crawl?". I really began to worry when our fingerprints appeared on his head through his blanket from lifting his newborn body.
   At 3 weeks old, we were called to the doctor's to receive the results. We were told that he had Junctional Herlitz form of EB. I couldn't remember which one that was. Junctional meant worse than we were hoping, but still not horrible, right? She gave us a bunch of print outs and answered our questions including "Is there a cure?" and then we left. We were in the elevator on the way down when I started to read the print outs. I scanned for Junctional Herlitz and began to read, "Junctional Herlitz EB is a very severe form of EB. These infants often die in early infancy..." My head spun, my heart raced and I began to think I would pass out. Brian and I made it to the van, snapped in Daylon's infant seat, shut the doors and cried together. The idea of giving birth and burying your child within months made me sick. We felt so alone in the world. We have wonderful family and friends who love and support us, but we knew no one who had a child with EB, no one who knew what EB was.  We didn't know how to diaper him, how to bandage him, how to bathe him, how to dress or hold him without blistering him. We had to come up with it all on our own and Daylon paid for our mistakes.

    My hope, is that no other family has to go with their child misdiagnosed and feeling alone and lost. If more doctors know of EB, then they can diagnose properly. The more people who know about EB, then the more people that have the opportunity to comfort a family with a newly diagnosed baby. Please spread EB awareness. Please share what you know about the disease with someone who doesn't (it's probably most people you know).
   We are so thankful to have all of you wonderful people in our lives. Thank you for being our friends and our support group. We love you.
  Check back soon for Reason #2: EB kids have feelings too


Cheryl said...


Thank you so much for sharing your families journey. I hope to help to spread the word.

<3 Cheryl

Anonymous said...

Jennifer, I read your post with tear filled eyes. Even though my precious Kelly had a different diagnosis and did pass within 6 months of her birth, I remember having the same feeling of loneliness and fear. Nobody had heard of Trisomy 18 and there was no cure as it was a chromosome anomaly and not a disease. I pray that someday EB will be a disease of the past.....something cureable or even PREVENTABLE...wouldn't that be a miracle? God Bless you and your family as you continue through your EB journey. I admire you so!


Aviles Family said...

Wow! Jenn that is about all I can say. The more I see of this disease the more horrified I am that these sweet babies have to suffer so. I can't imagine what you guys felt. Thank you for sharing.
Love you!

Anonymous said...

I have been following this blog since it was shared on facebook last year by a dear friend of mine. Not a day goes by that I do not pray for Daylon and his improvement. He is the same age as my son (born the same month July 2009). There is a group on facebook called Moms Who Drink and Swear (MWDAS for short) Nicole Knepper is an amazing mom and she started the group mostly for humor, but she is all about raising awareness! So I shared the link with her and asked her to post it to the group page to get some awareness of EB out to the world. I hope that is ok.

Anonymous said...

Jenn, it's how all we EB moms feel at first (and later on). I'm sending hugs to you and your husband and many good wishes to Daylon!


Anonymous said...

I'm new to your blog. I've been reading it from the beginning and decided to read the newest entry, and WOW. I cannot imagine how you must have felt, but it hurts just to try to imagine. My baby has his 6 month appointment on Monday, and I'm going to ask my pediatrician if he's ever heard of EB. I'm excited to see what he will say. I'm looking forward to the conversation! My thoughts and prayers are with you and your family!

Shawna L.
Washington, MO

Jennifer Edling said...

Thank you all so much for your amazing support!
CetinCM- yes! Of course that's ok! Thank you!
Shawna L- Thank you for taking the challenge!

jeanine said...

Oh Jennifer! How scary it must have been! I can't even imagine being in your shoes!

The Garcias said...

I was referred here from a friend who has been following your blog for while and who posted on facebook about it. I am so happy that I got here too! I never knew such a disease existed. My heart aches so much for you and your baby in those early days and even now. It is painful for me to hear his little cries on that video for 33 seconds. I can't imagine being his mother and not knowing how to console him at that time. I also read your post about the reactions that people give you guys. They are absolutely horrible. That is just one reason I am going to be referring people to your blog as well. Also, some of those people are just dumb or rude. I will keep your family, and especially Daylon, in my prayers!