Day +211: Here we sit...

Well, the title pretty much says it all.
 I'm here at CHOC with Daylon, Keira and two different forms of bacteria growing in Daylon's blood. Remember how in my last post I said he might be admitted? Well, turns out he had a staph infection from his Hickman line, but since the fever only last two days Daylon was free to come and go as he pleases and IV antibiotics could just be done from home. I am SO THANKFUL Fairview (the hospital in Minnesota) made us undergo a series of classes so we could take care of things ourselves and spend less time in the hospital! Dr. Buchbinder had Daylon go to outpatient infusion to get IV IG, which is a blood product full of antibodies given over six hours. Daylon is such a trooper! He did cry for about 30 minutes of the six hours, but have you ever know a kid to sit still for 6 hours? He's such a great kid!
 Cultures from his PIC line have continued to come back positive for infection and on Thurs it came back positive for two infections. That was the last straw for the docs and they had us come in. If everything goes as planned, we should be discharged late Sunday night which is perfect because Keira starts her new school on Monday and I have to be there for that!

  Minesotta called with the first step of his genetic testing results. Basically, they said that it looks to more than likely be genetic (the blister test that Brian and I took, proved that) and he's without a doubt Junctional Herlitz. There's a bunch of other things we learned (which to be honest, I'm not sure I entirely understand), but he's pointless to explain because it doesn't mean a whole lot just yet. Brian and I will be tested next, this June when we return for Daylon's "One Year" check up.
 More highlights of the week:
 Daylon rolled over on Monday night! He hasn't done that since he was 3 months old, so that's a biggie. Our personal goal for him to have him stand or crawl by Easter. We'll see. Knee skin tearing and hospital stays will definately slow the goal.
  Six days ago, as a last attempt to save our son from continuing malnutrition and vomiting, we started giving him 3 ml every 10 minutes all day while he's awake to keep his stomach constantly going a bit. I'm happy to report that it's actually working!! He's up to 10 ml every 5 minutes and vomiting less than 10 times a day!! He even had two days this week where he didn't throw up at all! Amazing!
  His skin has also made big stides in improving this week. Almost everything has healed up and he's only been getting a blister, at most, a day! Woo-hoo! We have need to go back to the open cloth diaper over the disposable diaper, like pre-transplant, but that's okay.

  Starting next week, dialysis is down to two days a week!!!! Can you believe it!?! His BUN today was 15 (under 19 is good) and his creatinine is 1.34 (under .58 is good). Improvements everywhere!
  The lowest point of the week, well, sadly it's not the admission to the hospital. Remember last month when Daylon had oral surgery to remove granulation tissue? Well, during the procedure they used tools to keep his mouth open and remove the tissue that were too rough for his mouth. His mouth developed very unusual blisters, which eventually became infected, then healed up. As they healed, the blister started to clump together into a mass. The mass has grown and grown. It's alarming, actually. The best way I can describe it, is it's like he has a double decker tongue. See for yourself:

Mid-December (the circles are the odd blisters)

This week

I sent the pictures to Dr. Tolar and he is having the ENT doctor from out here take a small biopsy from his tongue to check for cancer. It's just a procaution, but it's growing so quickly that they need to check for the obvious. The thought of Daylon having cancer on top of everything else makes me sick. Brian and I have decided to just put it out of mind the best we can until we get the results. Please pray that his tongue is nothing more than unusual scar tissue.

 Other Edling news:

 - So excited to speak to Aunt Emily on Skype, Keira ran onto the tile and fell, busting open her chin...AGAIN! Second time in 6 months! I've never, EVER heard of anyone doing that and she's done it twice! Poor kid! I think she must not put her arms out when she falls! :) What a goofball! Six hours in the ER and five stitches later, she was as good as new!

  I hope you all are doing well! We're so thankful to have so many wonderful people in our lives! Good night!

The twins are enjoying the Summer like weather in California!

...While, Caleb hasn't adjusted to life without some sort of jacket! :)

Day + 202 We're still here...

  I can't believe how long it's been...everyday around here has been packed full of crazy! Between dialysis and Daylon's doctor appointments all being 20 to 30 miles away  (in CA that means up to 90 minutes of travel time one way on a Friday), almost everyday, I'm constantly running late for something. :)

Practicing crawling on New Years Eve!

  Starting this week, Daylon is down to three days a week for dialysis. Yay! Monthly dialysis labs were done yesterday and Daylon's Creatinine was 2.4 (less than .58 is good) and his BUN was 54 (less than 19 is good). Obviously, we're still a bit off from being off of dialysis, but these labs were taken after not having dialysis for two days, so really, it's not too bad. Like I said, "Yay!" :) LOL! Keeping his weight under control has NOT been a problem! His dry weight is 14.2 kg and he's keeps weighing in lately at 13kg. His TPN was next to nothing fluid wise, but since he has started peeing, he lost a lot of weight and is constantly dried out. They actually give him fluid during dialysis. That's a switch! Dr. Buchbinder almost doubled his TPN and he's peeing that out too. On Thursday his newest order of TPN will come and that will have even more fluid and they're upping his protein and lipids (fat). Right now his poor little mouth is so cracked and bloody from being dry. I hope this works!

  A CT scan of Daylon's head was done and we had an appointment with the Neurosurgeon at CHOC a few days later. He said that it looks like about 70% of the fluid is gone from the newest head bleed. There's a line that goes down the center of your brain and when there's too much fluid in your head, the line is bent. Daylon's line, pre-surgery, was moon shaped. It was awful. There's still a shift to his brain but it is straight in parts, so I guess we'll take it. This Neurosurgeon told me that he felt that Daylon's brain damage was really minimal and that he hardly suffered any brain injury at all. Of course, I was thrilled to hear this but I couldn't understand how his diagnosis could be the complete opposite of 15 or so other doctors that looked at his scans. I left a message for Dr. Tolar and he called me back the next morning. He felt that this doctor probably came to his conclusion after watching a healthy Daylon clap and play on his examining table. It would be hard to say that he should be severely handicapped when the only version of him that you've seen is one where he's acting like a normal toddler. He also said that he thinks the others were more dismal for the same reason. They saw him in a coma in the PICU. Life for anyone can easily look grim at that point, I guess. So plan is, he gets another CT scan in a month and if the fluid isn't gone then he gets another Burr hole surgery if we and Dr. Tolar agree it's necessary. Daylon and I would return to Minnesota at that point. As much as I love Minnesota and the wonderful people there, I was kind of hoping to stay in CA until June. Plus, more than any of that, I REALLY want this kid to get a break. Anyone who knows his story the whole way through, knows that he has been through more in his 18 months of life that most adults have. I know he's been through 10 times as much as Brian and I combined.

  There was a confusion on the level to transfuse Daylon last week. CHOC dialysis transfuse at a Hemoglobin of 6, CHOC BMT 7 and Minnesota transfuses at 8. 10 days ago they found out the Daylon's hemoglobin was 7. something and he would need to be transfused. The dialysis people said that it would be too hard to get the blood on the weekend, then on Tues they forgot to order it before we got there and they said they would have to work after closing to transfuse him, so they wanted to do it Thurs. By Thurs when he was finally transfused he was on the edge of a 5. It made him so sick. He was fussy and vomiting pretty violently. They're not used to having a parent involved like I want to be involved and I think it annoys the dialysis folks. I kept asking for the transfusion and they would say he was "fine". Once Dr. Tolar got involved, the job got done. It's kind of frustrating that when I ask, what's his blood pressure at the say "it's OK" and I have to repeat myself until I get the numbers. Same thing with labs. I get "they're fine" instead of the numbers. Needless to say, I've learn to ask for a print out. :)

  Skin wise, Daylon's not having the best time. He has started blistering again. On the days he gets blisters, he tends to have 5 or so, which is still an improvement from where he once was. His back is a COMPLETE disaster. There are only trace parts of his skin that are still intact. His left knee and now his leg have broken down and we had to wrap it up again. His left leg upper thigh, groin and penis area are also raw and inflamed. We switched from the disposable diaper, to Fuzzibunz (a popular cloth diaper among EB babies) for the first time. In the past, I didn't think Daylon's skin could handle the Fuzzibunz, but I thought he might now. Anyway, turns out, he still can't he ended up getting pea size holes in his back's skin, where the diaper's elastic (wrapped in fleece) touches the back. Oh, well. Back to our old technique. We're back to our 1/2 jar a day habit of Aquaphor and wrapping Daylon in fluffy blankets and sheep skin to protect him from life and his self too. I have to admit that this time, watching his skin break down has been tough on me as well. It's easier to deal with kidney and brain issues when he's able to enjoy his skin. Not to mention, I have my parting conversation with Dr. Tolar floating in my head. One of the last things I asked? "So, be honest with me. After all of this...is Daylon still dying?" I could tell by his face that he wasn't expecting that question. He told me that no matter what he did, Daylon would always have Junctional Herlitz EB. He could never have non-Herlitz EB because those kids have a mutation that is entirely different than Daylon's. They're missing Collagen 13 and although his level is low, he has collagen 13. He is missing all three Lamin genes and while the transplant is helping to produce the lamin genes, he will more than likely never have as much as your average person. With 30% of the needed lamin though, he could live a "normal" life. He said that as of right now, he didn't think Daylon was near his demise, however, he said that may change in a year from now or 5 years from now or 10. It's all a wait and see thing. Watching Daylon's skin fall apart this last week, I couldn't help but wonder how much longer we had with Daylon here on Earth. I pray this transplant bought us more than a couple of months with him.

  Taking a couple of minutes to realistically think about this, I believe in my son and he's a fighter. Skin break down is the normal part of post transplant life. I am sleep deprived, sick and nervous being 1600 miles away from the hospital and team that knows Daylon and knows the transplant. The race between the benefits of transplant and the prognosis of death in infancy that he was given at 3 weeks old have been a constant fear for the last year since we found out about the BMT program. Having self mastery over my thoughts is obviously not a strong point of mine right now. :) I'm sure this is just a bump in the road.

   I'm looking forward to celebrating Daylon's second birthday this July :)

   Everyone around here is doing pretty good. Brian caught a cold and it has made it's way through everyone except Sadie. Daylon ran a fever and should have been hospitalized, but the dialysis nurses didn't pass on the info to the BMT doctor, so they didn't know about it until he was better. Thankfully, (and I don't know how) but it didn't have the lingering effect on him that it has had on everyone else.
  At this moment it's noon and Daylon is still asleep. He's been flirting with a fever and vomiting all night. We may be heading to the hospital later for admission. Just a heads up.
  Thank you for all of your love and support. Happy New Year!

Dr. Daylon