Day +36

Today has been the hardest day we've experienced yet on Daylon's BMT journey. The MRI went on as scheduled, and thankfully, without a hitch. In about an hour's time we were there and back up to his room, ready for dialysis. As they switched Daylon over from the portable monitors to the room monitors, we noticed his heart rate was running a bit lower than it has been. Once blood pressures started up again, the readings showed he had a higher pressure. We been watching for this since this weekend because it's a sign of increased pressure in his brain. The pressure on his brain, cause the brain stem to tell the heart to slow, thus causing increased blood pressure. Long story short, we were pulled into a conference room and told that Daylon's MRI showed not one, but two hemorrhages and brain damage. The damage is located near the motor skills and cognitive thinking section of the brain. They can not tell us how severe the damage will be. It could be movement issue which require physical therapy or mental retardation. It's any one's guess. They did bring to our attention that brain damage down to someone younger than three has been corrected by the brain finding alternate routes to send signals. There would be some permanent damage in that scenario, but it would be minor.
Due to the temperature being all over and his new heart rate and blood pressure issue, the think the brain stem may be involved as well. His brain stem can tell his heart to stop at anytime. After one months time, they believe that risk will end. Dr. Wagner is stumped. Dr. Cooy (PICU attending) is stumped. Neither one of them have ever seen something like this and have no clue what to make of it. Dr. Wagner thinks it may be a Junctional EB thing. I'm not sure if he thinks it's Herlitz or not, he never specified. The problem is, according to Dr. Wagner, Junctional EB is a mystery because no one has been patient enough to give these kids a chance. Dr. Wagner wanted to have Brian and I agree to a partial DNR (Do Not Resuscitate order). If his heart were to fail, chest compressions would rip his skin off his chest. Not to mention, if the brain was that far gone, there's nothing they could do anyway. We agreed, of course. We want what's best for our baby. The neurologist will be in tomorrow to answer more brain related questions.
I'd be lying if I said I wasn't heart broken by the news. We came here to save our baby from death and to try to offer him a normal life. The idea of him dying is devastating, but so is the idea of our little boy living with EB (for the next several years) and now being mentally or physically disabled. The idea that I may never hear his dinosaur growl or watch him blow me a kiss, is heart wrenching. I can barely stand it. How do we take on this next big obstacle? As I wrote the question, the answer popped in my head: "Faith". I've been thinking this all afternoon. Brian and I lingered in the conference room and said a prayer together. It was a comforting prayer. I know we were meant to come here. We both felt that this was where Heavenly Father wanted us to be. Once we made the decision, all of our ducks lined up in a row. Daylon got stronger, his cells were fixing themselves. Life has been known to throw us all through the loop. It's so important right now, that we have faith in the impossible. There is a reason for all things, and sometimes you have to dig to find them, but they're there. I had a talk with my mom today who reminded Brian and I that they were told my sister Emily would be severely mentally retarded and as I write this, she's away at school getting her Master's degree. Tim (Bella's daddy) and I talked today and he encouraged me to remember that no doctor alive, can tell us what will happen with Daylon's brain. The brain is a pretty incredible mystery. My dad told me that "everything will work itself out" and to stay optimistic. I sure am trying. I can't wait until I can hold him again. My arms ache to rock my little baby, so bad that it literally hurts. I know that wonderful things are in store for Daylon. This is so much for such a young person, or any person to go through. I think very special, strong spirits are given such challenging obstacles to overcome and so many blessing to be received by them and everyone around them. It's an honor to be a mother of such a spirit.
Thank you all for caring so much about Daylon. We ask you all to please pray for a full recovery for Daylon. Prayers are all we got. We love you.

Day +35

warming up Daylon's body after he got some fresh bandages!

The guards stood at their post today, ready for action. When I say "guards", I mean Brian and I. Today, two new BMT doctors (the attending and the fellow) came on active duty and trying to make sure they're up to speed on Daylon, while still trying to make sure the new PICU doctors, who don't visit the room, are on the on the same page as everyone else, leaves you feeling a bit guarded. It's not all that bad when we're being annoying by asking a million clarifying questions, but I hate when we're absent for a conversation. Darn lunch. I'm probably just paranoid. Here's the run down for today...

This morning, we over slept (those metal dark out blinds do it every time) and missed rounds. We learned when we called in (and spoke to a nurse who's never had Daylon) that tomorrow morning (Fri.) he's scheduled to go down to radiology for a MRI. This is beyond confusing to me because just the day before the BMT docs said he wasn't stable to be moved. He's not even being rolled in his bed, more or less wheeled down several floors, moved onto a metal bed and bagged for an hour long procedure. The other weird thing I noticed is when she was ready off his blood results from today's lab, his white cell count jumped from 6.6 to 13.0. That's a hardy jump. Kind of a scary jump. When the count moves up REALLY high or really quickly it signals infection. So, half a day worth of time and 10 conversations later, Daylon is going in for an MRI tomorrow morning because the doctors are worried about meningitis. Lovely. For those of you only vaguely familiar with the disease, like me before this afternoon, don't look it up. Lets wait for the test results to come in. His temps are over 102 at one check and then they're 93 the next. They're hoping that the MRI will show what is going on with his little body. We do too. As far as him being too sick to travel, he might still be, but they need to know what they're dealing with. We've been told that a parade of people will accompany us on the outing which is a comfort.

His bilirubin is down to 1.1, which is wonderful and his liver ultrasound showed that all three major veins are flowing in the correct direction! Oh! Plus, his liver is down in size from last week! The VOD is going away! We love good news!

His Creatinine and BUN (kidney function) have gone up,which is expected since dialysis was once again cut short yesterday. Yesterday his dopamine was turned up to 20 (that's maxing the dose out, which he's never done) and left on until 5 am. Today, several new steps were taken to help with his blood pressure during dialysis (remember he can't do the all day dialysis) including a dose of steroids to help with his adrenal glad function. He went up to 18 on the dopamine within a half hour of dialysis, and then 20 by 90 minutes in. We were starting to stress about his pressure when he plateaued for an hour and then gradually started to come down while still on dialysis! He was on 15 when dialysis ended and completely off dopamine two hours afterwards. To say I am shocked is an understatement. This afternoon they were able to pull off 90g. of fluid! Yay! The ups and downs of the roller coaster we call, BMT are amazing! I can tell you one thing, at the end of all this my hair will be snow white!

Daylon has handled sedation pretty well today, which is great since the box of sedation drugs that they can pull from is empty. Tomorrow after the MRI, he will be rushed back to his room where dialysis will be waiting for him (they have to get the dye they'll give him out of his body since his kidneys can't do it themselves) and then about an hour after, they'll start to wean him off some of the less powerful sedatives.

I really feel like he's moving forward. I'm not too concerned about the meningitis scare. I don't know why. It could be that my brain can't process one more thing, or it could be that he doesn't have it and our Creator is sparing me the stress. Who knows. Tonight when we were changing out his headgear dressing, I saw a side of my little guy I haven't seen in a month. He started to wake up when I was doing his mouth cares and suck on the Red Robinson (suction tube). He peeked his eyes open a bit and when I moved it away, he smacked his little lips the way all babies do and settled back to sleep. I loved seeing his whole face. When I watched him, just for a second, it was like I was back home, putting him to sleep for the night. Those nights feel a million miles away now. I'm so glad I was able to watch him be a baby again.

We need to head back home to grab some shut eye before our busy day tomorrow. I'll leave you with the some pictures from our day. Thank you for all your support and checking up on Daylon. Sweet dreams.

Keira and Caleb participated in the RMH lemon "Aide" stand. The kids all worked so hard in the hot, hot sun! They came away with new friends and nice tans!

In the words of my mother, "We made out like BANDITS!" tonight at BINGO! We won a 3ft talking doll house, legos, a giant floor puzzle, tinkerbell stationary, Strawberry Shortcake activity set, and baby rubber tonka trucks just to name a few! What a blast!

The kids love their new floor puzzle!

Day +33 and Day +34

Say, "Cheese!" Daylon, Daddy and nurse, Rachel

Clown Baby

Things around here have been as crazy...Good crazy, mostly! If you can believe it!

Skin and BMT:

Daylon had his blood drawn on Day+28 and the results are in! Daylon's CD 15 cells (short lived) are 100% engrafted with Caleb's cells! Daylon's CD3 cells (long term)are 91% engrafted with Caleb's cells! 91% isn't 100% but it's still really good. They expect to see that in the upcoming checks that Caleb's cells will gang up on Daylon's and put them out. Anyway, the transplant was a success!!! Dr. Tolar came in yesterday for the day +28 skin biopsy (it was Day+33 but the lab was closed on Day +28). I had all my bandage and bath supplies ready, chucks on backwards (help with contrast on the pictures) hand washed and ready to go! Dr. Tolar and I each took a leg and started cutting off layer after layer of bandages. Once both were removed, I looked up at Dr. Tolar and he just said, "Wow. This is very rapid healing." The photographer snapped leg and toe shots, and we moved on to undressing the arms. Dr. Tolar just kept repeated, "This is very rapid healing" and his tone almost became serious. More pictures and then his chest was uncovered. By this time, Dr. Tolar is just kind of staring, so I offered to show him the before and after post from the blog. He and the scientist just nodded as I showed them picture after picture. I was kind of giddy initially, but then watching face caused me to be a bit concerned that somehow the "rapid healing" was bad. Finally, he pops up and says " I agree that he his healing really quickly and I can't explain why. This would be unusual for normal skin to heal so fast...but we'll take it!". WOO-HOO!! That's Caleb's X-men powers at work!! Ha, ha! He said only one other patient had so few new breakdowns, but no one has healed like this. He had to ask we where he did the first biopsy and when I showed him he said, "Where? OH! I see! Wow, this is amazing!". They took 8 new samples of skin by a blood blister on his upper left thigh.Oh! Cool side note: the lady who preformed the procedure was trained by Dr. Metz (his pediatric dermatologist back in CA)! Small world!

Next was a skin fragility test where they hold a 3 hole light suction cup up to his skin to see how quickly he blisters. Last time it was 5 min. and 18 sec. and this time it's 8 min. and 49 sec.! 3 1/2 minutes quicker in one month! Aaaaamaaaazing! Normal skin is 60 minutes, so he has a way to go,but wow! We're all so stoked at the possibilities that lie in front of him!

Current Condition

Subdural Hematoma: Still no more seizures, which is wonderful, of course. Neurologically speaking, he his moving forward. Everyday further away from this weekend we go, the safer he is. They would like to do an MRI on him, but the PICU won't allow it because they feel it's too dangerous for him to travel. If the machine can't fit in his room, it can't be done.
Kidneys: He weight has been somewhere in the 14kgs before dialysis and after it's somewhere in the 13 kgs. Since this weekend the goal has dropped to a net of 600 grams. The last two days they tried to up the game again to keep up with all the platelets he's being transfused with (if it drops below 100 he gets transfused and BMT kids naturally sit in the teens, so the transfusion are constant). His body can NOT keep up with these crazy new demands, and his blood pressure is dropping quickly. He's on dopamine for low blood pressure almost constantly now and before he was off within a few hours of dialysis. At this point they would put him on prisma, a 24 hour dialysis (what Bella's on) but there is a list of problems that it will cause:

-he'll need a new line, and they can't move him to the OR
-an additional risk for infection, that his body couldn't handle right now
- it would pull his meds off him 24 hours a day and right now they are having a SERIOUSLY hard time keeping him medicated during dialysis, which is crucial for neurological reasons.
- (This one is more my problem) no BMT nurses. You have to uses nurses trained for the machine. Boooo!
Please pray that Daylon's blood pressure will behave and they can take off him what they need just using the intermittent dialysis.
Liver: Bilirubin level dropped all he way down to 1.6 (1.3 or lower is great)! We are shocked and so excited that the VOD is turning around! It typically lasts 1-2 months after transplant so the name of the game is to stay alive. If you can do that, you'll out last the VOD. He's over a month in, and it's time for things to turn in his favor. :) He gets a liver ultrasound tonight and we really hope it shows some improvement!

Stomach: The bleeding has officially stopped. Yay! His G-tube is back to being a gaping hole, but hey! We know it will heal!

Heart Rate: His heart rate is still really low when he's off dialysis, but the good news is that the brady episodes have ended!

Tomorrow both our BMT attending and fellow will be replaced and since the NICU attending and resident are new this week, I'm a bit nervous of the care he'll get over the next week or so. I'll have to be on my guard again...just when I started to relax! Oh, well. Such is life, right?

Some news in our lives outside of the hospital is pretty great! My mom had a horrible 19 hour plane ride here, but she's here! The kids are excited to play with grandma and life has become so much easier for us. I love my mom! Brian and I have been together for every mishap today. I love having my husband beside me. We can tag team the docs with what questions and he's great at making me laugh when I'm stressing out. Plus, no one hugs like my hubby! :0)

Yesterday we got a call from Best Buy and our computers back!!! Picture time!

Keira got to participate in the RMH Queen for day! They painted nails, decorated cupcakes, played games with beauty pageant princesses, and she even got to wear a real crown! Mommy threw up her hair in a "Queen Do" and she even got to wear some shinny lip gloss!

Brian at the EB dinner held by PUCK (Pioneering Unique Cures for KIDS) looking out at the view.

Violet got into my mascara when I hopped in the shower. "Look! I'm a pretty grown up!"

We went out to dinner when my mom flew in to celebrate and Caleb refused to eat off the "angry plate"! It had us crackin' up!

This beauty was in Sadie's nose and required a trip to the urgent care (we got the call about Daylon'a first seizure on the way there). It was waaaaay up there! Once again our family trip to Sam's Club was cut short. Once for Violet's head being cracked open and now for this lego adventure!...mind you, there's only been two family trips! Needless to say, the tiny legos are on the top shelf now!


The kiddos and our landlord, Ron

Photo therapy!!

Caleb's, Violet's, and Sadie's favorite dolls

MVI 0809

I LOVE watching this! I wanted you all to see Daylon in action! This is the day before he was admitted.

Day +32: Moving forward

Erik Hendrickson (I used to babysit him!) is serving his mission out here and stopped by for a visit! It was so sweet! He's so grown up now!
Today was better!! Daylon hasn't seized since late Sat. night, his "soft spot" is flat and his eyes are focused. All good signs pointing in no increase in size of his brain hemorrhage. On to level two of the danger zone! Yay! This is such a HUGE sigh of relief! I can't even tell you how excited I am. I had faith he would make it through (although I had to keep reminding myself of every spiritual confirmation I've had that we should be here), but now I have hope. These are two very different things and I'm learning that more and more. I'm smiling today. I like being happy. I know he's still "very critical and can turn at any moment", as the doctors put it, but I've been able to be self indulgent with my time today and do things like visit the ladies room, and come back without finding out something horrific has happened. His bilirubin and creatinine levels are down today, which is really surprising, but we'll take it! His weight is down to 13.8 (it was the same yesterday), so that's a good sign that his VOD, and therefore his kidneys are getting better. Dr. Wagner informed me that the body won't let the weight off if they weren't improving. Woo-hoo! The bleeding from his g-tube site has stopped and there isn't much blood in his stomach now either. They may even start up his feeds again...although he will be back to TPN (IV nutrition). His temp. is still all over the place. He would be 93F for an hour and we'd have to cover him up and then it would jump to 101F and hurry and uncover him. This is so weird. Both Dr. Tolar and Dr. Wagner have been coming into visit him and the only thing they think it could possibly be is the blood sitting too close to a spot on the brain that controls your body's temp. The attending Dr.s switched today, which meant a morning of trying to convince the new doc. that all the sedatives Daylon was on were needed. It was a rough battle, but we won. He's on enough sedatives to knock a large adult into a coma, but he's up and jerking within 30 mins. of dialysis. The problem is of course, wounds and infection, but pulling the meds off so quickly can trigger a seizure. Plus, once they loose the sedative on Daylon, it's gone for hours. It takes them soooo long to get him under control! Once he woke up this morning, and saw him in action, they joined our side with little persuasion. :)
When Dr. Wagner came in he told me that he would have never dreamed it would be this bad. Daylon is his first patient (not just EB) to ever have a brain hemorrhage. He said he designed the EB BMT chemo to fit an infant and the Busulfan (chemo drug) has caused both Daylon and Bella to get VOD (which inturn causes almost every other problem he has), so he thinks he may have to reevaluate the dosing. It's nice to know that in a way, they're helping other infants coming through. He's never had an EB patient on defibrotide (b/c no others had VOD) so he's not sure if the hemorrhage is more likely with EB or not. I think it's a fluke.
So, the long and the short of it is, Daylon is about the same as yesterday. He's not any worse and I like that. I'm trying to think of this stuff as a bump in the road. The road is a bit bumpy, but the road is still there and he's walkin it. Thank you for all of your prayers on Daylon's and our family's behalf. Not only have the complications stopped piling up, but Brian and I feel comfort and a sense of peace that we've desperately needed. All of your comments were so touching to us...they made me cry!...good tears, though! Thank you, again! In the words of Dr. Sumani, the PICU attending, "God is good and we'll take this one day at a time".

Day +31

I want to apologize in advanced for a another picture less quickie. Hopefully, I can make this short but sweet!
After I posted yesterday things took yet another change. Daylon had several more seizures with the longest (and thankfully the last one) lasting over an hour. They think that the extra blood has made is body's tolerance very low. It all started during dialysis and dialysis has been diluting his sedatives really quickly. So much so, that he's awake by the end. The sudden drop has become too much for him now. He had another CT and it showed although the bleeding had increased, it was very minimal. The Neurologist told me that we are going to continue down the same path but he doesn't think Daylon is strong enough to undergo surgery (that was hard to hear) and all the additional risk, just don't make it a possible option. He is topping out doses on the only 4 sedatives he can have (having a failing liver and kidneys seriously minimizes the meds they can give you we are learning). After the last seizure ended (around midnight last night), Daylon started jerking. It was really weird. None of the doctors have a clue what is going on but it's kind of like his stomach and chest are bucking. An EB kid bucking for hours is a recipe for disaster. Nothing thing they gave him would calm him down. His G-tube has started to bleed from all the movement and the blood is all over his skin and falling into his stomach. Or at least we hope it's falling into his stomach because his stomach is now bleeding. I feel pretty confident that it's just the movement. His temp. was up to 105F at one point last night and then it just dropped to 97F. Weird. Again, they can't pinpoint why. The BMT attending thinks it could be the blood sitting on his brain...I like that theory. He can't afford to get a blood infection now. So far all the blood cultures are coming back negative for infection so that's a relief! Yay! His Bilirubin (liver) and Creatinine (kidneys) levels are both up, but it's kind of expected since they took him off the difributide and lowered dialysis.
We are in this so deep right now that I can hardly see the light. If I'm being completely honest with you, I am more scared now than I've ever been for his life. This is completely overwhelming. This is WAY MORE difficult than I could have imagined! I've cried now in front of 2 doctors in the last 12 hours. My chest is heavy, I feel sick and I've been mid-prayer for 48 hours now... and it's not even a good prayer, it's more like a beg.
Thankfully, I have three bits of news that have pulled me through the day!
One: After I came home last night to grab a quick shower and a meltdown, Brian and I decided to ask my mom if she'll come back up to help us. I called this morning and she's coming in tomorrow night at 8pm! Woo-hoo! Help is on the way! We're just being spread way too thin and something's gotta give before we break. I'm sooo happy to have some stress relief! It is kind of embarrassing though that my aunts just left on Weds. and we already are calling!Oh, well! Don't judge us! :)
Two: We made the decision that Brian and the kids are not going to come home Aug. 7th. Keira will go to school at the RMH and Brian's job will still be held at Interact Power. I love that we don't have to be separated for such a very long time! Plus, I'll be able to see Keira off to her first day of Kindergarten (my dream!)!
Three: The BMT doctor made it clear that he can turn around from here and if he didn't think it were possible, "we would be having a whole other talk". I really like Dr. Verneris. He knows his stuff , stays on top of Daylon's condition and it's so nice to have a doctor on the team that cares about him and sees him as "Daylon" and not "BMT EB patient 11, 12 month old with Junctional EB".

Day+30: Update

Hey everyone!
I don't want to be out of the room too long so I need to make this one quick and picture less. Daylon did well through the night, with no additional problems. The experimental drug for his VOD (defributide) and dialysis are to blame for the bleeding since they both involve blood thinners. Brian and I decided to take Daylon off the medicine and the doctors agreed. His 21 days would have been up on the 27th so he got a hardy dose and will still see the benefits of the drug. They upped his platelet count to 100 (he was being transfused at 30 before now), so he's been receiving a constant platelet transfusion since midnight last night. The heads of BMT (attending and the EB docs), PICU and Neurosurgery got together and decided to wait on the surgery and watch Daylon closely. The head is a giant surface of bacteria and since they've been pumping him full of immune suppressant drugs and he's an EB kid, his chance for infection is extremely high. Too high to risk it at this point. Daylon was unable to move his right hand last night (from the pressure on the left side of his brain) and starting 3am or so, we saw some finger movement. It's still not any big movement, but at least it's something. The doctors believe his condition is improving. There are three levels he has to go through until he's out of the woods: the first 24 hours, the first 72 hours, with the last level being 3 weeks after the bleeding. Brian and I are so thrilled with the news of being able to avoid surgery. It would be such a risky procedure for him. Looking back, I can really see how the events of last night, although scary, were a real blessing. Had Daylon not had a seizure, we would not have known about the hemorrhaging until it was too late. In the very least he could have suffered permanent brain damage. But since the doctors know, they are able to thicken his blood and try to stop the bleeding. This has been such a great reminder that although we are given trials, we're never alone. There's always something to learn, a greater purpose for our pain. Thank you for your encouraging remarks and all of your wonderful prayers. They've helped Brian and I get through our toughest times.

PS- We owe a HUGE special thanks to:
* Tim (Bella's dad) for camping out at our house last night doing 3am potty runs with the twins and searching for binkies in the dark! We'd have been lost without him! We can't imagine doing this without you guys!!
* Lonni and Jay Moreland for taking all four of our (coherent) crazy kids for the afternoon! Our kids are so psyched to hanging out with their family! I still can't believe they braved the park with them!
It has been so nice for Brian and I to be together with Daylon at the hospital. We're so grateful that we've been able to focus on Daylon and not have to worry about the other kids! Thanks again guys!

Day +30- The adventure continues

Today ended up not being so quiet. I'll give more details later. This post is more like a world wide plea for anyone who cares about Daylon to pray for him. Tonight Daylon had a seizure, which lead to a CT that showed the left side of his brain is bleeding. The good news is that it is on top of his brain and not in his brain. The bad is that there is enough blood to put enough pressure to trigger seizures. He's going in for brain surgery as soon as his platelet count goes up (probably a few hours) so they can drain it. When I asked how much experience the neurosurgeon who would be preforming the surgery had with EB, the doctor asked, "what's EB?"when I told him he said,"Who cares about his skin at a time like this!?". Long story short, me and this guy got into it and he won. I just stood there crying so angry I could slug him, while he listed off the risk of different type of skin infections that could occur at the site. He really has no clue.
Please pray for Daylon. Pray that the surgery can be a success. Pray that his two "smaller" incision don't become two huge ones. Please pray for the doctors and all of the medical personnel attending them. I wish I could fly you all here to sit with Brian and I in the lobby. We'll feel you in spirit I'm sure. I'm so grateful that there are so many people who love our son. We love you.

Day +28: Before and After

So, here are the pictures of Daylon! Please keep in mind that his skin got worse after transplant (thanks to swelling and chemo) so the good that you see has happened really quickly. Normally, his skin takes one to several months to heal. It will take years for the EB to actually go away, but it's a start! Here we go...

Left Hand:

Day+0 Day +10

Day+28

Left Palm:

Day +18

Day +28

Left Arm:

Day+0Day +10

Day+28



Right Hand:

Day+10

Day+28

Right Arm:

Day +10

Day+28

Chest:

Day +0

Day +28

Right Leg:

Day +0Day+28

Right Foot:

Day +0

Day+28 (infection)

Left Leg:

Day+0 Day+28

Left Knee:

Day +10

Day+28

Left Foot:

Day+0

Day +28 (infection)