I have been struggling with this story for quite some time. Please read it knowing that I just wrote down things that I am feeling or that I felt. I was not too particular on grammar. Also know that I did not intend for it to be 4,772 words when I started it. That is just what happened. Hopefully one day someone will read my story and it will help them through their own tough time.
Every parent always thinks their child is perfect, no matter what. Jason and I do not only think Jackson is perfect, we know he is. So what if he has a few extra chapters in his book? With any good book you are always left wanting more, right? Well with Jackson you get more.
On May 28th 2010, just 10 short days after Jackson completed our family, our lives were changed forever. Jackson was diagnosed with epidermolysis bullosa (ep-ih-dur-MOL-ih-sis buhl-LO-sah), or EB for short. EB is a condition in which the skin blisters in response to minor injury, heat, or friction from rubbing or scratching. There are three main types of epidermolysis bullosa, with numerous subtypes. At the time we did not know which subtype Jackson had. Each type is different from person to person and some are fatal. The word "fatal" and "your son" are not words you like to hear in the same day, much less the same sentence, yet we did. It took several days for the doctors and nurses to determine that EB is what Jackson has and it would be several more weeks before we found out the type that he has.
When Jackson was born he had a few abrasions on his back and elbow. Jackson entered this world so quickly that they thought that he got scraped up coming down the birth canal. This tends to happen A LOT. Jason and I thought it was pretty funny and a great story to tell, we laughed about it. Little did we know, that was the start of our very long journey with EB. After being released from the hospital we took Jackson for his follow-up at the regular pediatrician, Dr. W.
(Dr. W has been with my family for 6.5 years. He started seeing Nylin when she was just a few days old. We have a wonderful connection with him and we are so glad to have him as our children’s doctor!)
Dr. W took a look at Jackson's abrasions and the little rash that he had developed on his chin. He asked us several questions about our family history and mentioned herpes simplex virus, or HSV. HSV infections are very common worldwide. HSV-1 is the main cause of herpes infections that occur on the mouth and lips, including cold sores and fever blisters. It is transmitted through kissing or sharing drinking utensils. HSV in newborns can be very severe and even deadly. HSV usually causes seizures and brain damage. The symptoms of HSV in newborns are a low fever, one or more skin blisters and poor feeding. Of those things Jackson had some blistering on his chin area. The doctor sent us home and told us to keep an eye on the blistering and he would see us the following week for a weight check.
The next day Dr. W called us at home. In the 6.5 years that we had been going to Dr. W’s office he had NEVER called us at home. He called to check on Jackson and get a report on his chin issue. We told him that Jackson was doing well and his chin was much better. We had Nylin’s dance recital that evening and we were so excited to get out the door and on our way so I really did not examine him and give a full report. Dr. W told us to have fun at the recital and he would see us next week. Little did we know we would see him sooner.
Just as we were walking out the door to go to the recital Jason noticed a blister on one of Jackson’s fingers. I thought that this was from him sucking on his fingers. I told Jason to put a baby glove on him and we left. As the recital progressed Jason noticed a few other fingers blistered up. These blisters were occurring just at the bottom of his nail beds. Again I thought nothing of it. I really thought it was from him sucking on his fingers.
The next day we were expecting some visitors. During the first visit we realized that Jackson had 6 blisters on his fingers and one had popped. When it popped yellow fluid came out.
I freaked.
Everything after this point is somewhat blurry. It was all happening so fast. I barely had a moment to breathe. I had just given birth to this amazing little guy and brought him home to join our little family and the next thing I know I am taking him back to the hospital. I could not wrap my head around this at all. I did not show any signs of fear. I couldn’t. I am the mother and I have to be strong for everyone, or else they would break. All the while there are one million fears running through my head. I did not know if I would be coming back home with my little guy and I was scared, really scared.
Jason and I took the kids over to his dad’s house and headed to the hospital with Jackson. The hospital we chose has a pediatrician on staff at all times but is not a children’s hospital. The doctor’s office suggested this hospital since it is close to home, has a pediatrician on staff and has short wait times. We were in a room within ten minutes of arriving. The pediatrician came down within thirty minutes. We were assigned a nurse (which asked me if I burnt Jackson, I was SHOCKED!). The pediatrician, Dr. S, came down and saw Jackson and talked with us for a minute and then he realized that Dr. W was our doctor. He is actually friends with Dr. W, good stuff. Dr. S was able to get Dr. W on the phone with me and we discussed the next steps. They thought that we were dealing HSV. They decided that they were going to treat this as HSV. They said HSV was the worst possible scenario and we needed to be aggressive if we wanted good results. In order to test Jackson for HSV they would have to do a spinal tap, on my newborn son. In order to give him the medicines he needed they would have to start an IV on him. They would be giving him 3 different medications at different times throughout the day, all through the IV. They would not put him on IV fluids if he continued to eat and have the right amount of wet diapers. At some point they would be transferring us, via ambulance, to Cardinal Glennon Children’s Medical Center.
At this point I literally felt like my head was spinning. I could not breathe without telling myself to. I was scared, really scared.
Jason left to go home and have dinner with the kids and get them to bed. Jason’s mom came up to the hospital to be with Jackson and me. I am so thankful she was able to come up and be with us. She was there for the spinal tap. I could not be in the room with them because I was afraid I would collapse. She stayed in there with him and held him while the doctor took fluid from my son’s spine. The fluid came out yellow. They decided to do a bilirubin test, which tells you if the baby is jaundice. The bilirubin test is a heel stick and after they do this they put a band-aid on it. This helped the doctors later determine that HSV was not what we were dealing with. Every time they would put a band-aid on my little guy he would get a blister there.
Sometime they did several heel sticks and applied several band-aids, every time he got a blister.
That evening Jason’s mom left and Jackson and I were transported to Cardinal Glennon. I was happy to be there. They knew kids better than anyone. After the slightly traumatic experience with Jackson’s IV at the other hospital I was glad to move on. As soon as we were brought into our room Jason met us there. It was nice to have my husband back with me. I needed to lean on him. He was and still is the rock that gets me through my days. Nothing much happened that first night at Glennon. It was a Sunday and none of the specialists were there.
We settled Jackson down in a bouncy seat next to the pull out bed and tried to lie down for some sleep. I thought I would just crash; after all I was still recovering from giving birth. Instead I curled up next to my husband and cried. We cried together. Saying I was scared would be an understatement. I was terrified. I thought we were going to lose our son. The very son that I felt had completed my life and completed my family. The very son I just welcomed into the world and now there was a chance he was going to leave us. I thought I was going to have to say goodbye to my baby, my little tiny helpless baby. I cried myself to sleep thinking of the pain that he must have been in and the pain that I was no doubt going to feel when he was taken from me.
Not even 30 minutes later I was up again, I had a newborn son and he was hungry! I had never been so happy to be waked in the middle of the night. I wanted that time with Jackson. I wanted every single moment that he would give me. I needed that time. I thought that our time was going to be limited and I wanted to stare at him. I wanted to study every feature of him, his face, his cute little nose, his tiny little toes and his amazing head of hair. I wanted to remember it all, just in case he was taken from me before I got the chance to look at him again. He opened his little baby eyes and stared right back at me. He looked at me with such love. I could feel the unconditional love of my son through his eyes. It was almost like the light of God shining right through him. It was an amazing experience that I will never forget.
Jackson had a very rough day that day and he was not up for nursing. I let the nurse talk me into pumping and giving him a bottle. I really did not want to introduce the bottle to him yet but she threatened to put him on IV fluids if I did not get him to eat. I did not want any more wires attached to my son. We warmed up some pumped milk and he ate like a champ! For the rest of the time being in the hospital he would eat from the bottle mostly. Sometimes he would nurse but I really did not care, as long as he was eating.
After being up half the night with Jackson Dr W came in around 5 AM. The look on his face was enough to make me break down into tears. He had a look on his face that just said “I’m sorry”. He was sorry for not having the answers we wanted, he was sorry for having no answer at all. Most of all he was just sorry that we were in this situation. I could tell that he just wanted to tell us it was nothing and send us home. The reality is though, something was wrong and we just did not know what it was. Dr. W thought if this was in fact HSV we caught it early enough that there would be minimal damage (brain damage that is). The fact that Jackson had not had a seizure was a good sign. The fact that he was blistering from band-aids was not a good sign. We were not sure what kind of sign it was really, but it was not a good one. Dr. W decided to have dermatology and infectious disease come take a look. He also decided to put him under a bili light to get the jaundice under control. And then he was gone. He was off to his office to take care of other sick babies, sick babies with colds, ear infections or diarrhea. Oh how I wished for a cold, ear infection or diarrhea!
The nurses brought us a really scary looking incubator bed (the kind they use in the NICU) and a bili light. They broke down the top of the scary bed to make it a little less scary. Then they put a little mask/headband over Jackson’s eyes and they put my tiny baby in what looked like a tanning bed. His blisters looked worse under that light. They were glowing. Glowing blisters is not something you want to see on your newborn son, or any age son for that matter.
We waited almost all day for dermatology to come in. I think they made it in there around 4:30 or 5PM. They basically just looked at him and said a few super big words and told us that they were going to talk to the supervisor about it but she did not think this was HSV at all. Dr. S (the supervisor) is a highly sought after pediatric dermatologist and she was super busy. They said she would be in the next day to take a look at Jackson and we would go from there. The rest of the night would be spent talking on the phone to everyone calling and worried about Jackson.
Of course I still had a newborn son to care for so I just did. I took care of him like I would have if we were at home. I fed him, sponged bathed him, changed his diapers, rocked him in my arms and cuddled him tight. I did not know what else to do, so I just did what I knew best.
Sometime in the night I woke up and looked up at my little baby in the big scary bed with the bili light over him and my heart sank. I am not sure I can accurately describe what I thought I saw, and I am not sure I want to even go there in my own thoughts. What I can tell you is that I thought my son was dead. I thought that something had happened to Jackson and I had slept through it. I flew up out of bed and looked down at him and saw something totally different. I am not sure if it was the lack of sleep or a nightmare or what but it was scary and strange. I fell to the floor and began sobbing. For the split second that I thought I had lost my son my whole world came crashing down on me. My entire adult life flashed before my eyes and I could not think of how I was going to go on living. I myself wanted to die. I wanted God to take me and make my son the one that could continue living. I wanted to take his place in that bed. I wanted Jason to pick him up and carry him out of the hospital, living and breathing a totally normal life. I lied on the floor in the room for several minutes sobbing and thanking God for not really taking my son from me at that moment. I looked over at my husband who was sleeping soundly and I thanked God again for giving me a man who I knew was going to carry me through whatever it was that was about to happen to us and our little family. The nurse came in and saw me on the floor and helped me to the bathroom to clean myself up. She never asked what happened or gave me any BS lines about how everything was going to be ok. She just grabbed my arm and led me to the bathroom and got me a cold cloth. When I washed my face and looked back at her just in time to see her wipe a tear from her own eye. She still never said anything. She took Jackson’s vitals, handed him to me to rock back to sleep and she left the room. I never mentioned it again, to anyone, not even Jason. I saw that nurse several more times during our stay and she never mentioned anything about it. She just looked at me and smiled.
The next day was spent waiting more and more. We had a few family visitors and visitors that are like family. It was nice to have the support of our family and friends when we needed it the most. I will never forget the people who called us and came up to see us. I will also never forget the hundreds (yes hundreds)of people that lifted my family up in prayer.
Sometime during that day Jackson had blown his IV. This is very common in little kids since they move around so much. We knew it would happen at some point since the first hospital did not have baby boards. They taped it as much as they could and sent us on our way. Glennon has plenty of baby boards and it needed to be re-done. Thankfully they take him into another room do that because it would have killed me to see them wrestling with him.
While the nurses had Jackson in the procedure room doing his IV, Dr. S from dermatology came down. She took a look at Jackson and I think she knew right away what we were dealing with. After they removed the old IV (and the tape that held it on him) there were patches of skin missing. Dr. S ordered no more adhesives, cloth diapers and immediate draining of old and new blisters. A few minutes later they brought my little guy back into his room with a new IV. Dr. S threw out a few possibilities and said we were trying to rule out the worst of the skin conditions that she thought it was (EB). In order to rule it out she needed to do a biopsy. She also told us she did not think this was HSV but wanted to wait for the test results to come back (from the spinal tap) before taking him off the medicine. As fast as she came in, she was gone. Her crew stayed behind to tell us that they would be off site the following day and would come in as soon as they knew something.
We were so relieved to finally have SOMETHING to tell people. While they were in the room I wrote down the name of the thing they were ruling out but they told us specifically not to go home and search it on the internet. Jason did it anyway. As soon as he pulled up some info on EB and saw what it was he rushed back down to the hospital to be with Jackson and me. He was terified. I told him not to tell me anything that he read or saw. I did not want to dwell on that stuff. I wanted to take care of my son the best way I knew how and I wanted to just listen to what the doctors were telling me. I could not focus on the what-if’s. I couldn’t.
Sleeping came a little bit easier for me that night. Not for Jason. Every time he would close his eyes he would see those horrific pictures that he saw online.
Early the next day someone from Dr. W’s office came in to tell us we could take Jackson off the bili light and take him off the meds. I was so relieved but at the same time I was confused as to what the results all said or meant. We spent the day waiting. Sometime during the day Dr. S came in, took Jackson and did a biopsy on his foot. She said a few things about EB but was very vague. She did not want us focusing on the fact that it was probably EB. She kept repeating that she was ruling it out. I know that she knew the entire time. She was just trying to give me that last day with my baby.
The very next morning Dr. S and one of her crew members came in (Dr. M, LOVE HER!). They were not even in there for one minute when they told us that Jackson did indeed have Epidermolysis Bullosa. I got that crashing feeling again. I felt like my head was spinning. My heart started beating through my chest and it was hard to breath. I did not know what to do. What just happened? I swear I had ringing in my ears for 2 weeks after this meeting. Dr. S and Dr. M gave us a ton of really good information about EB. They directed us to some websites and to a specialty clinic for any questions. Dr. S does not specialize in EB but there is a clinic in Ohio that does. She wanted us to go there to get checked out. She kept giving us more information and at some point I think I went numb. I just started writing down everything she said. I was so glad that Jason and another friend were in the room for this conversation. I do not think I would have retained any of it had it not been for them reminding me later. They told us that the biopsy that they took showed that he had EB but they would have to send the sample to another lab to determine the type. The lab they chose was in California at Stanford University. It would take 4 weeks to get the results back. They gave us a little more information, some supplies, showed us how to pop the blisters and told us we could go home.
Home? What the hell was I supposed to do at home? I have 3 other kids I have to go face? I have in home nurses coming to help me take care of my son? I have to pop his blisters? I have to keep him healthy and free of infection all by myself?
Oh. My. Gosh.
I do not think that Jason and I could stand alone at that point. I thank God everyday that we were there to hold each other up that day. Several times at the hospital and even when we got home I felt like I was going to faint. I kept getting that crashing feeling. I felt like the weight of the world was on my shoulders and I had no idea how I was going to care for this tiny sweet baby.
They sent us home with a nice stash of supplies, a handbook on EB (that they printed offline) and a few instructions from the nurse in Ohio. Our nurse that morning, Jess, was amazing. She spent 45 minutes on the phone with the nurse from Ohio so that she could give us the best information possible. She came back with a list of good products to use for Jackson. We had no idea what kind of cloth diapers, clothes or anything to get for him. This was a whole new world for us!!!! Thankfully Jess, Dr. S and Dr. M gave us the info we needed to carry us over.
Jason and I went home and tried to resume our normal lives. For the next week I would spend half of each day crying. I would cry in the morning when I woke up, I would cry at the table eating, I would cry in the shower, I would cry in the car, I would sometimes pretend I had to use the restroom so I could go cry, I cried myself to sleep. Taking a simple look at my sweet innocent child made me cry. I cried for the pain he was in and the pain he would undoubtedly face in the future. I thought about all the things he would miss out on in his life. The older kids would be running around the house or wrestling and I would just cry, Jackson won’t be able to wrestle around.
I just thought and I just cried. Jason cried with me. It was all we could do. Just take care of our kids and cry.
After the first week of crying, doctors appointments and nurse visits we tried to resume an actual normal life. I threw myself into taking care of Jackson the best way I knew how, just like my other kids. Jason threw himself into research and support groups and taking care of the house and the other kids. Yes he did it all. I do not think I lifted a finger for weeks unless it had to do with Jackson. My husband really stood up and REFUSED to be knocked down. Don’t get me wrong he still cried but he did it in a way that showed me I could lean on him in order to get through this. I can’t really explain it but he was my protector, my rock.
Although our appointment with Dr. S was only 4 weeks from the day we were released, it seemed like an eternity. We took care of Jackson and popped his blisters and tried to prevent new ones. All I could think about was getting back to the doctor to find out exactly what we were dealing with. Did my son have the mildest form? Or did he have a more severe form that is sometimes mild as a newborn and could possibly progress? It was driving us insane. I just wanted to get back in to see Dr. S and have her tell me, AND NOW!!!
On June 25th, one month and 7 days after Jackson joined our family, we found out that he had EB Simplex. We were very relieved. This is the mildest of the three main subtypes. Although it varies from person to person, EB simplex generally only affects your skin and not your mucous membranes.
EB Simplex is caused by a mutation in the Keratin 5 or Keratin 14 gene. These are the genes that manage the formation of the proteins in the top layer of the skin, the epidermis. The result is incorrectly formed keratins which cannot perform their intended function: to hold the top layer of skin together. As the skin splits apart, a blister is formed. The way I explain it to people is that the glue that holds the top layer of his skin down is missing or extremely weak.
There are several different subtypes within the main three subtypes. Without doing the DNA testing on Jackson there would be no way for us to find out the exact subtype (within Simplex). Dr. S did not think it was necessary to do that testing unless in the future there are treatments for Jackson’s particular type. She gave us a few tips, told us to go to OH for more tips and once again sent us on our way.
Jason and I were (and still are) so relieved that simplex is what we are dealing with. From what we have read online and spoke to different people about, I think that Jackson has a very mild case of simplex. We try not to really focus on that at all. We just try to take care of him and prevent him from getting blisters the best we can. Just like with anything else, some days are better than others. Some days I just want to scream out how much I hate EB and everything that comes along with it. The amazing child that Jackson is makes it that much easier though. This child is just nothing short of amazing. He smiles and he carries on with his day like any other little baby, sadly he knows no different. I know I said it before but I explain it to people like he has the light of God shining straight through him. He looks up at me with those big eyes of his and it just melts my heart. He lifts me up every day. He makes me believe in all things good. This little boy is really something special, he is perfect.
