EB Awareness Reason #3

  This is my final reason to raise EB Awareness on the final day of EB Awareness week this year. Reason #3- To Cure the Incurable.
 
   I've given a lot of thought as to how I want to go about explaining the importance of this reason for me. There is a large portion of this blog's following that either know Daylon personally and have seen EB and how he's suffered or that follow several EB blogs and have heard all of us parents describe on a regular basis what our children go through living with this disease. It's a horrible disease. Before Daylon, I would have never imagined that such a horrible disease could possibly exist. There have been many, many times especially early on in Daylon's life when I would be in the middle of a bandage change or moments after I hurt him by trying to help him and I would think, "How can this be?".
  
   Yes, it's painful. Yes, it's heart wrenching. Yes, there aren't enough words and it's worse than any parent could possibly describe. The awful truth is, there is no cure. With a cure comes hope.
 
  I believe that we are close to a cure. Really close. There are some clinical trials out there trying to find a cure. I truly believe in the possibility to cure this disease with the Bone Marrow Transplant program at the University of Minnesota. There are still kinks that Dr. Tolar and Dr. Wagner are trying to work out, but they're making progress all the time. Unfortunately, like any other new development, they are only allowed to make progress when there's funding for the research. Because it's not a disease that a large population of the world suffers from, it's hard to get government funding, so it's left to the people.  The more research they do, the safer transplant gets.
   On October 11th of this year, exactly one year after Bella's passing, Nicholas Moore's mom posted to his blog that he had passed away the better part of a year after transplant. Thanks to funding, the last several transplants have been safer and the children are doing better than before the changes were made.

    I am an EB mom with hope for her child's future...and I want other parents to feel the same way. I believe these doctors have the possibility of curing this horrific disease sometime in the near future.

   I can tell you all day long how I think we're close, but I realized pictures can speak louder than words....

The following image may be too graphic for some.



April of 2010- Before Transplant

6 Weeks Ago-

   Raising awareness means that when people are looking to donate for a cause, EB can come to their mind because they actually know what it is. It's a pretty important reason to raise awareness.
  
   If you're interested in donating towards the cure now, you can text "Bella" to 50555 in honor of Bella or visit the puck website HERE to donate on a secure website. If you or someone you know is looking for a charity to donate towards at the end of the year for a tax write off, P.U.C.K. (Pioneering Unique Cures for Kids- Dr. Tolar's and Dr. Wagner's research  ) accepts donation all year long! :)


   A HUGE thank you to all of you who have helped in spreading EB awareness this week!! The support that the EB community has received from all of you; our family, our friends and our support system has been amazing!! Together we'll touch the world. We are so grateful.

EB Awareness- EB kids have feelings too

  
EB awareness week continues! Reason why awareness is important to us #2- EB kids having feelings too.
    Being the parents of an EB child means that your weeks are either hard or harder. Taking care of the child is work and it can be very hard, but the parenting aspect of having an EB child is more of what I am referring to. When a child is bullied at school, it doesn't just effect the child, it effects the parents too. It's a hard thing to watch happen and know you can't completely control the situation and shield your child from the pain of others...the pain of life. The same is true watching your child suffer through Epidermolysis Bullosa.
    Lately, things have been rough. It is so hard to watch people stare at Daylon when we go out and make rude comments like he can't hear or understand their hatred for something they don‘t even begin to understand. He is a person. We have been kicked out of stores (Yes. You read right.) and been the reason for an obnoxiously boisterous parent to literally drag her children (by the collar I might add) and flee the store yelling to the point that everyone, including the cashiers stop and look, "LOOK AT THAT BABY!! LOOK AT THAT BABY!". Yeah. I'm not joking.

     That was just this week.

      Last May in a Target store, a middle aged woman had her attention directed to Daylon, after her adult daughter gasped. She quickly turned to look at him and replied, "Oh. That's just gross!" and then surprised by what she let slip, sheepishly left. I stood there, with the blood rushing to my face and the sting of her words pinching my entire body. I have begun to worry that if people keep behaving like this when he goes out that he'll develop low self esteem and think of himself as less that than the perfect little boy that he is. I don't want him to be afraid to go outside and enjoy life. After that woman's comment I went back to the hotel, cried of course, and then made little business cards to hand out to people.

Front

Back

      As Daylon’s mom, it breaks my heart into a thousand pieces to watch him go from playful and happy, to embarrassed and shy when he notices people staring or sees children pointing. At just two years old, he lowers his head and stares at the ground. I now have these business cards to hand out and they have been helpful in convincing store managers and some strangers that he’s not contagious, nor a threat to anyone, but I can’t spread the word to everyone. Especially to those loud, stubborn people…you know the type.

    Thank goodness for EB awareness week.

    A popular comment we hear after people realize that he’s not contagious is, “ Why don’t you keep him at home so people can’t make a big deal or stare at him?”. I mean this the nicest way possible, but that is not an option. Yeah... No. I’m not leaving him locked up at home as if I were embarrassed by him. EB sufferers are like anyone else and have every right to go out and socialize without feeling like they’re being judged. They already live a life of pain, how can they possibly be denied interaction with the world. That’s just ridiculous.
    Most times, children are the worst at staring, pointing and ugly remarks. I can only imagine what school aged EB children most go through not only at school, but in public, understanding exactly what is being said.
  
   Wouldn’t it be wonderful if we could raise enough awareness of EB that when a stranger sees an EB child, they could recognize EB and offer a warm smile instead of a glare. Wouldn’t it be wonderful if we could always remember the God loves all of his children and is especially with those who are sick and afflicted.

    “…Inasmuch as ye have done it unto the least of these, my brethren, ye have done it unto me.” Matthew 25:40

     If we could raise enough awareness, we could take away one form of pain from the lives of those who live with the disease called Epidermolysis Bullosa.

     Check back soon for Reason #3- To cure the incurable

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

Admitted...

Content after his day of procedures was over

  

   I'm not sure if I've mentioned this or not, but Daylon gets sick every morning. Seriously, e-v-e-r-y morning. He throws up for the first 45 mins or so that he's awake. Sometimes more, but never less. Three or four nights a week, the vomiting starts in the middle of the night and goes into the morning with an hour or so of sleep in between.  As he's gotten older and his stomach content is no longer that of a baby's, this has become especially rough. In the morning, while he is sick, he is no mood to play and often cries with sudden screaming. During the day, if he's given anything too sweet (juice, yogurt), meat, veggies or red dye, then he becomes sick again. Am I sick of being sleep deprived and smelling like puke or worse yet, watching my son in misery? Oh yes. But this is his life and to be honest it's a huge step up from where he once was.
   Last week, as the temperatures climbed up to 100F, Daylon's benefits of transplant began to fall. His skin started to tear and slough, his vomiting became projectile, there was blood in his stool and one of my biggest fears, his cry and voice became hoarse. The hoarse cry is a classic JEB thing. It's a sign of the larynx blistering. When Daylon was a baby, his cry was a whisper and we would set our alarm clocks for every 15 minutes so we could check and make sure he wasn't crying. Because the damage from the past, Daylon can't afford to blister in his throat and airway now. In the middle of the night on Friday, he rubbed his eye and caused a corneal abrasion. He kept his eyes closed most of the day Saturday and managed his pain quite well. He did however, continue to throw up through out the day.  On Saturday night, while getting him ready for bed, I accidentally poked him in the eye and caused the eye to get worse and consequently, his pain. I hate that I hurt him. On Sunday he was in pain and cuddled most of the day. Just before dinner he was content enough to sit on the ground so I could drain my pasta (Brian was busy with the kids playing a board game.). Anyway, he picked up a foam sword and because he can't see, poked himself in the other eye causing abrasion #2. He was so miserable the rest of the night. Because he's been pretty healthy lately, I don't even have any pain meds in the house, so he just had to tough it out with Tylenol. It was awful. Over the course of the weekend he also started scratching like MAD! I have no clue why he so much more itchy, but that didn't help the skin situation. He was just plain miserable.
  Monday morning while I was getting the girls up for school, I could hear Daylon start to dry heave. I went in to check on him and he was covered in blood and vomit. Brian came in and we cleaned him and his bedding up. He was breathing really quick shallow breaths and his whole body shook. He didn't have a fever, so I couldn't figure out why he was shaking.
   I will be honest, it does throw me into a bit of an emotional and mental funk when Daylon's JEB symptoms come back. His skin does still fall apart and he still has many signs of EB, but it's the JEB symptoms that freak me out. Once we got over the big scary part of the transplant and it really started to improve his condition, then I've just worried about things like kidney function and malnutrition.  I guess I've just gotten to where I look at him and think that the transplant will keep him safe from JEB and what it brings. I do know that there's a chance it could reverse, but I don't let myself really process that most days and that's how I keep from going insane. The last few days, I can't help but think that. Daylon is 27 months old with a life expectancy of less than 12 months. I am grateful for everyday, but I do get scared some days too.
  Anyway, I called the doctor to see if I could get him in that day and he wanted us to take him to the ER. Given Daylon's history, several different things could be going on, which is their big concern. He was admitted to the hospital, had a chest X-ray, a bowel X-ray, a head CT, blood cultures drawn, a virus culture, urine sample and MRSA check. In other words, he was poked and prodded all the live long day. For the most part, everything came back fine. His white count is high, probably from infection and stress and his bowel X ray shows build up and blockage. More than likely, his GI tract is blistered causing bleeding and slowing in his GI tract. All and all, it's just EB stuff and he probably would have done better at home, but EB is a lesser known disease at CHOC, so they like to error on the side of caution. After he pulled out his second IV in 12 hours, they decided to can the IV antibiotics and go with oral so we can just stick it in his gtube. Since the blood cultures were negative at 36 hours, I was able to convince them to let him go home.

This was written all over the couch in Daylon's room in the Oncology ICU. Needless to say, Daylon does not feel the same way the couch does about the hospital. :)

  
   We've kept him comfortable with bed meds and an increased doses of hydroxizine (his itch med). He's still puking a super ton and acting out of sorts, but there is about an hour after the pain meds kick in that he's happy and being his silly self. He even crawled for a few minutes last night. At one point, he opened his eyes a tiny slit looking for a toy, so I think there's improvements there.
      A huge thank you to everyone who has offered prayers and service on our behalf! Especially to my mom for driving out to Lake Elsinore last night and staying with my kids today (keeping up with 3 different school schedules and extra curriculum can be a total pain!) so Brian could work! You're a lifesaver Mom! Thanks!!
  Oh! I've decided to post a family video of Daylon when he was first born for EB awareness week. In my opinion, it's a pretty powerful video. I hope that all of the blog readers will take the time next week and share what they know of EB with one person (if you feel like more, go for it!). Let's get the world talking about EB next week! Check back next week for the video and EB awareness post! =D