Crazy skin

Yesterday we had Mommy-Sons time since Caleb had a check up and Daylon dialysis.

First off, I need to say thank you to all of you who prayed for Daylon's appetite to improve and for the kids and I as we all headed off to dialysis together on Monday. The kids were absolute angels! I couldn't be prouder of them. They sat from 12pm until 5 pm, in their chairs and were so quiet and respectful of all the sick kids in the room. Like I said, I'm so proud! The nurses were so impressed that they gave each of the kids their own can of playdough. Nice, huh!

  Over the past three days, Daylon's desire to eat has picked up. Yesterday, he drank 20 oz! I know it's still not very much, but at least we're seeing some improvement. :) I'm praying that he's intake will continue to climb everyday and he'll soon be eating enough to sustain himself. The doctors have decided to give him two weeks to improve to a healthy status, but if we don't see enough improvement then they'll start giving him TPN during dialysis to force some calories into him. Please keep the prayers coming!

 
  Daylon has had insane Kramer hair the last several week, as you may have noticed. Everywhere we go, people comment on his quote, "Rock star hair". LOL! Yeah. That's not intentional! :) His scalp has been cracked and dry. It looks like the dry desert floor. Anyway, it obviously itches really badly, so he scratches his head and rips up the skin. He has a bunch of random bald spots and scabs as the end result. Because his scalp is either scaled or scabbed all over his head, his hair stands up on end. With Daylon's skin, the area around the breakdown is always extremely fragile and so it's really easy for the breakdown parameter to spread and spread. Just since my last post, the parameter has spread from his forehead, down his face. His upper chest has started to blister and tear too. His chest hasn't looked like this since before transplant, so that's a bummer. It's become a lot more difficult for me to get Daylon not to rub or scratch his sore skin. When this would happen pre-transplant, I would bundle him up in a blanket and hold him almost constantly for a few days to get his face to try to scab up but at 19 months, he's a lot harder to contain. It's all about being creative when it comes to parenting an EB kid since everything is off the books.

 
  Outside of that, things have been going well. Daylon has been happier and smilier than he has been since his surgery, so that's always a treat for us.
 Oh! Kind of funny story:
 I put Daylon in his bouncer so I could sweep up the kitchen floor after breakfast. Five minutes later, Caleb came running in saying, "Mom! Something is wrong with Daylon! I think he's dead. Come see!" My heart fell to my feet and when I ran in I found this:

No worries. He was just that tired. It's rough playing . :) Sleeping with his forehead on little piano keys didn't help the blistering!
  Hope you're all doing well. We're so grateful to have so many wonderful people rooting Daylon on in his journey.

Yesterday we got to see Dr. Blumberg his Pediatrician and Brooke his nurse. They have been such a HUGE part of our support group for Daylon and have helped us fight to get some many things covered by our insurance. We love them!

Day +200 and something...

  You know how they say that you can't miss something you've never known?

  They're wrong.

  Tell that to Daylon. Let me explain:

  These days, I'm no longer worried about Daylon dying any time soon of EB. Instead, I'm worried about him dying of malnutrition. We are seriously at a cross in the road when it comes to his eating, only to find that there is no more road.

  Really quickly, we're back from Minnesota. The recovery has gone great and his mouth never did get any blister in the hours following the surgery.

  Okay, so back to the eating situation...Daylon is gradually dropping a tenth of a kg every four days which probably doesn't seem like a lot but it is. His dry weight (what he should weigh) is 31.24 pounds. He now weighs 23lbs. Did I mention he's over 3 feet long? Just looking at him, he looks okay because he has a bigger head but once you lift his shirt! Wow-wee. He is literally transparent skin and bones. Dr. Tolar was all smiles and playtime with Daylon until he lifted the blanket and then he just looked at me and said, "Oh, no. We've got to fix this."
  I spoke with the nutritionist and got a recipe for pretty potent formula but the only problem is, Daylon is refusing to eat. Like clamped down, shaking his head from side to side, kind of refusing to eat. The last two days I've actually had to resort to pinning him down and syringing formula into his mouth when he screams. It's awful. Not to mention his poor skin isn't handling it either. He's been this way for the last 2 weeks or so and it's getting worse everyday. It's like he's just lost the desire to eat. I'm having flashbacks to the first several months of his life. Ugh.
  The funny part about it is he does seem to show some interest in "real" food and drink though. I don't get it. He's NEVER had real food before. Pre-transplant over ripe bananas and egg noodles gave him a blistered and bloody mouth, so most foods were automatically out based on texture. Because his GI and urinary tract blistered so easily he couldn't have anything acidic (juices, tomato sauce, etc) or else he had blood in his diapers and a VERY upset tummy. Seriously, he has no clue what food taste like. Ha... and he wants it anyway. I would be willing to try it out, but ever since he woke up from the coma he has not been able to chew and has difficulty just swallowing and breathing at times. I tried giving him refried beans last night and he was willing to try it, but choked and gagged and then puked. He has the world's easiest gag reflex too, which isn't helping anything. I will say though that once the doctor added the third anti-nausea med, his everyday puking has gone way down.
  Naturally, putting in a G-tube again would be the next step but he's no longer a candidate for a g-tube. They feel that his skin can't support the constant friction and the catheter and trauma that's caused by the tube being there will make his nausea worse. I gotta say, I think so too. The next step is to put in another PICC line and start him on TPN again. The reason the doctors have been trying to avoid that route is that TPN has significant side effects when on for an extended period. It's supposed to be a resolution that can last a couple weeks until nutrition picks up. Daylon's been on it for months. We'll see what the doctors say tomorrow, but I have a feeling that he'll have to go back to a PICC. I really don't know what else to do. I'm traumatizing my poor baby and he doesn't even want to be around me the last two days because I'm constantly trying to get him to eat. By the end of today; he was crying, I was crying and he had eaten 11 oz for the day. A far cry from the 34 oz minimum that the nutritionist set.
  Please pray that Daylon will have the desire to eat and I will be able to think of a way to get him all the nutrition he needs to be healthy and quite frankly, live.
  That's all for tonight. I'm mentally drained and I want to sleep. Tomorrow I have a 10 am appt in Irvine and then dialysis at 12 with all five kids in tote. I gotta say, I'm not looking forward to it. Oh well. At least we'll all be together. That's about 20 steps of improvement from last week and 1600 mile distance between me and my kiddos. Keira doesn't have school tomorrow either and that's a huge plus. Just looking for the lining. The blessing are always there, sometimes I just have to make myself look for them.

  PS- We had some time before church. Pretend like we weren't surrounded by cars and houses. :) Here's how we entertained ourselves:

After looking at these pictures, I am reminded that I have so much to be grateful for.

Good Night.

Day +235- out of surgery

The resident that was on the night Daylon coded all those months ago was a doctor named Brian. He helped us through the mess with chemo and his last night on was that horrible night. His rotation was up and he moved to a different unit. I've never forgotten the kindness he showed us during that awful time in our life. One day in particular has stuck in my mind. I was border lining a nervous breakdown and he came in for a "social call". I was a complete wreck when he came in the room and I felt so much better by the time he left. He treated Daylon like he was a person and not just a patient. Anyway, he was on call tonight and when he saw Daylon's name on the patient list, he came in to say "hi" and catch up. Camera phone pictures again...sorry guys.

This may be the worse picture I've ever taken of Daylon. His poor eyes give him such drama.

 I want to thank you all for your prayers and thoughts of Daylon today. I wasn't the only one nervous about the surgery today. Turns out, all three doctors were on edge too. They brought in the anesthesiologist that has intubated and extubated Daylon in the past and we all went over the plan for surgery...and then we went over it again. The plan was to use Propofal through his Hickman and not intubate. Instead they would just use a hose of blow-by (oxygen blowing on you). Dr. Rimmel was going to cauterize and seal the tongue and they would use several Red Robinsons to grab the blood as it came to prevent him from aspirating on it. If they couldn't keep on top of it, then they would emergently intubate him. Dr. Tolar would be in the room the whole time over seeing Daylon and collecting the tissue at the end. Sounded good to me.  I opted not to go into the OR this time. There was nothing for me to bandage, I made the pulse oximeter EB friendly during the pre-op evaluation, Daylon was wearing pants so blood pressure reads would be a breeze, they weren't going to use ECG leads and all of the doctors and nurses knew Daylon and had worked with us on several occasions.  It's really hard for me to do, but there really isn't a reason for me to go. I'm trying to learn to loosen my EB mommy apron strings as Daylon gets better and better.
  After about an hour in the lobby Dr. Tolar walked out to tell me that everything had gone PERFECTLY (of course, not without scarring me half to death. What doctor comes out of the OR shaking his head and looking at the ground? I introduced him to a new rule: make eye contact with the family and smile when you leave the OR and then you can shake your head all you want! LOL!)! Not to be braggy, but he said that Daylon amazes him and just jumps over one hurdle after another like it's nothing. He also said that he didn't cry or act scared while he just lied there waiting for them to get started. He said  that he just looked around at everyone and babbled. That made me so happy to hear. I wondered the whole way down the hall if he was screaming and scared in that cold, bright room.



after surgery--looks good, right?! :)

 Anyway, everything went so well that Dr. Rimmel said he could be released home (RMH) today. Dr. Tolar vetoed that idea, so we're still here. We're happily situated in a room on 5D. It's so nice to see everyone!
 Some pictures of him "playing" after surgery:

lets pretend that he's wearing an adorable smile holding his Valentine Pooh ;)



Pooh is getting some smooches



Okay, no denying it. Daylon was less than thrilled after surgery. I'm not sure if he's screaming at the doll or going to eat it... Either way, it's not a good day to be Pooh. :) 

  Daylon did have an allergic reaction to one of the meds given to him in the OR so they knocked him out to keep him from scratching his skin off. We are seriously becoming limited on the pain meds he can receive.
  Well, that's the news on this end. I let you know when we get the results back from the head CT he had this morning.

HAPPY  VALENTINE'S  DAY!

Day +234 -preparing to go under the knife

earlier this week with his eyes open

...or the blowtorch. We're not really sure yet. I asked Dr. Tolar when I saw him on Friday if he knew how Dr. Rimmel was planning on removing the granuloma and he said he didn't know. In fact, he said he was the best ENT he has ever worked with and he trusts him completely, so he's not going to worry about it. How's that for a compliment? Granted Dr. Tolar the kind of guy who has a compliment for everyone, but still. It made me feel a little better about the situation. I do have to admit though that it didn't stop me from stressing about the surgery all weekend. I've got the whole butterfly in your stomach, can't sit still feeling. It's awful. Part of my paranoia (or at least that's what I hope it is), is that between having regular blistering and scarring from his airway, he has suffered some permanent damage from having such a horrible case of mucositis and being intubated for a prolong period. When they went in in December to intubate him the doctors hmmm-ed and haaaa-ed in the OR before telling me that the scaring was pretty bad and his vocal cords were fusing together. After the burr hole surgery he bled for about a week down his throat and vomited bright red blood. This time around, the want to do the surgery without intubating him. Normally, that would have me "woo-hoo"ing out loud, but this is a scary surgery to not intubate. The mouth is such a bloody place when aggravated and I know from daily experience with Daylon that it doesn't take much. There's a fairly good chance that all the blood will pool in the back of his throat and he could aspirate on it causing pneumonia. They granuloma covers most of his tongue, so there will be such a mess to deal with guaranteed. To add to everything else, I've had now three doctors tell me that it's more likely than not that the trauma from surgery will leave his mouth in bad condition resulting in further surgeries. I feel so horrible for our little man. No one can understand why Daylon's mouth is worse post transplant...and it was bad before! From what I'm told, the other patients mouths were one of the first places to spot improvement. Oh well. His skin is doing great, so we're very thankful for that. We'll just have to pray and wait that his mucous tissues and organs will eventually catch up. Dr. Tolar is going to run tests on the tissue removed and check it for Caleb's cells to try to get an understanding of what's going on with his mouth. I'm so glad we're up with such wonderful technology!

Daylon getting ready to turn in the pop-tops that some of our friends and family have saved from back home! Thanks guys!

  I'm sorry to be such a downer. I've been stuck in our room at the RMH with the exception of Tues and Friday ALL DAY LONG. Daylon has been so fussy. He has every right to be. He's bored out of his mind (Mommy isn't as entertaining as 4 siblings) and his constant corneal abrasions have lead to a nasty infections in both his eyes again, which have lead to rubbing, which have lead to more abrasions. Vicious cycle. Oh!...and being the genius that I am, I left his glasses at home. They're kind of important when your kid is going blind. :) Did you know that a mother who takes care of a child with special needs loses 6 years off her life for every year she takes care of said kid. It's even higher if they have other children. Learned that tonight watching a documentary on PBS while I was waiting for Master Piece theater to start. That's bored I am...Well, truth be told, I actually do like the Pre-19 century, British aristocrat movies they have on from time to time... back to the rant :)  I'm going nuts not having my kids drive me nuts. Funny how that works. I miss the madness. Looking at the Courtyard is when I miss them most. It's like a movie. I keep finding myself staring out the window and I can literally see them. I see them running through the sprinklers, I see them sliding down the giant caterpillar with Chloe (Elle's sister), I see Keira and Caleb pretend to go fishing off the bridge, I see the twins running around the porch of the playhouse, I see Brian rocking on the swing laughing. It's seriously like something out of a Lifetime drama.  I think I'm going crazy. Either that or it's an aneurysm. :p
  Okay, happy thoughts...
  So, Dr. Tolar did give us some wonderful news on Friday. Check out this picture!
  Control= Dr. Tolar's skin   
  DE= Daylon Edling and the number beside it is the number of days post transplant when the biopsy was taken.



The red line is what you need. In the middle picture Dr. Tolar explained that the dye picked up some of the collagen instead of laminin b/cwhen he ran the tests, there was nothing there. In person you can see the line is polka-dotted and kind of odd looking. In the picture on the right, the red comes in like a line. Look at it compared to the controlled sample! It looks great!



In this picture the tinsy bit of red you see in the middle picture is real. On Day 0 Daylon did have some of this form of Laminin. In 30% of JEB kids, their bodies can begin to fix their mutation here and there. For Daylon this didn't help a ton because he has a mutation in a couple places, but it may have helped him to live as long as he did without the transplant. The picture on the right shows an AMAZING amount of correction in his skin's DNA!! Dr. Tolar is really excited about the possible affect of the transplant on JEB kids! I am too! He said that he believes that where there is a little gap in the red (off to the left of the picture) is only there because Caleb's cells haven't had enough time to multiply and fill in the area. With time, that very well may happen!! :)

 Isn't this great news!?!
 Daylon and I will head over to the hospital around 10am tomorrow for a head CT and then over to pre-op from there. The surgery is scheduled for 1:50pm. Please keep Daylon and the surgeons in your thoughts and prayers. You all are the best!

PS- anyone who's interested, the Press Enterprise Newspaper has done a follow-up story on Daylon and the Children's Cancer Research fund. You can read about it here or by going to http://www.pe.com/ and searching "Daylon Edling".

Minnesota...we're back!

 Well, we're here in Minnesota! Daylon and I flew in on Monday. I really, REALLY don't like flying alone with him (security is a big pain, passengers fear that he's contagious, carrying all his stuff, not to mention the all the fun anyone has when traveling with a one year old), but this trip was too horrible. He actually slept for a third of it too! Yay!
  Anyway, he had dialysis on Tues. and they decided not to give or remove fluid. At the end of dialysis he was 11.6kg. Yikes. That was his dry weight at 10 months old. His vomiting has gotten better, but he has very little desire to eat and literally clamps his mouth closed if he sees a spoon come at him. All he'll open for is his squeeze bottle. Not even a syringe of smoothie is tempting anymore. Dr. Tolar and I have been emailing back and forth and I think we're going to plan on added a PICC line when he heads to the OR.
  ENT needed a few days to get everything in order for the surgery. It looks like Monday at 1:30pm is the big day. Looks like we're looking at a looooooong weekend ahead of us. It's really okay though, because I'm enjoying the bonding time with Daylon....although I do have to say I miss Brian and the kids like mad! Thank goodness for technology! :)
  I want to throw a question out there to all of you:

Are you thinking about having a baby?
You don't have to actually answer that, I just wanted to get your attention! :) A few months back I started emailing a woman named Natasha who has come across a little Russian baby named Anton. Anton's parents hired a surrogate to birth a child for them. She ended up having twins: one healthy baby and one baby with EB, Anton. The parents decided to only take home the healthy baby and left Anton behind at the hospital forever. He's been living in the hospital his whole life. This story broke my heart. I instantly wanted to adopt him, but Brian was level headed and reminded me how much we have on our plate right now. I started telling all of the BMT nurses and doctors about him because I figured they'd be perfect having EB experience and all. We moved back home and I kind of dropped the ball. Anyway, I got an email from Natasha last week that the hospital is moving Anton into an orphanage where there's one adult to every twenty kids. I hate to say it, but it would be really hard for an EB child to survive under those circumstances. They require hours of care and a 1:20 ratio won't allow for the time he needs.
  Anton just had his first birthday in January. Psychologists say that he is calm and loving little boy with a surprisingly sweet disposition. Financial help is available.

 I've added a link to the sidebar and another at the bottom of the blog about Anton. Please visit his website and pass the word along.
Let's find a home for Anton!!

Day +226 heading out

Hey Everyone!

A couple things...

One is that I just wanted to let you all know that tomorrow Daylon and I are heading back to Minnesota. We'll be there roughly 2 weeks. He has an appointment scheduled for Tues and with some luck, surgery will be Wed. He will be intubated and hopefully only spend a few days in the PICU. After that, he'll spend the rest of his time over on 5D with all our BMT folk. I'm really excited to see everyone! They're like family to us! :) This is some risk with the surgery and I'm really nervous. Daylon's mouth is one of the most sensitive places on his body and his last oral surgery resulted in the latest oral events. They have said that trauma will more than likely occur with this surgery and the mass could grow back. Anyone who is inclined to pray for Daylon, please pray that the surgery goes well and that no damage is done to his mouth. Please pray that the intubation goes well also. His poor little airway, is an absolute disaster, which I know we need to fix in time, but I don't want that to be now.

Tripp last year at his birthday

I have a favor to ask of all of you. Will you please stop by Tripp's blog and post a note for Tripp and his mommy Courtney. If you don't know Tripp, he will steal your heart, like he's stolen mine. Tripp has the exact same form of EB that Daylon has and he holds a special place in my heart. I've been reading his blog for quite some time now and so much of what Tripp was going through, Daylon was going through. Their lives almost mirrored each other at times.  I always really felt like Courtney just spelled out all of the fears and emotions I was having. It's not like I could just go to my local EB mommy support group at the local civic center. EB is such a lonely disease and when you find out that you have less than a year to help your kid (every parent wants to find a cure)...I'm not ashamed to admit I was panic stricken. You know the nightmare where you run and run, but you never get anywhere and no one can and or will help you? That's a lot what raising these babies is like. Anyway, ever since I came across their blog, life has been so much easier for me knowing that there's another family fighting the battle with us, even if the are across the country. Courtney is a wonderful friend and an amazing mommy to Tripp. Sweet little Tripp is such a strong fighter. He was truly blessed to have such a great mommy who has filled his life with love. Tripp is having a really rough time lately and he can use all of our support, love and prayers. Please visit them.

I'll try to write more tomorrow once we're settled. Sweet dreams.

UPDATE



Daylon and Papa

 Hey Everyone!
Just a quick note to let you all know that Daylon is out of the hospital. The bacteria in his PICC line cleared up and we were free to go! Thank goodness that's over with! Round one million in the hospital and Daylon wins again!! Woo-hoo! All seriousness though, the Oncology ICU (aka in MN: BMT unit) staff was wonderful and super considerate of Daylon and all of the difficulties that are presented with tending to a patient you can hardly touch. His right arm pit is the only injury from the hospital stay (one nurse forgot to use aquaphor when taking a temp. and then pulled it out instead of lifting the arm first. She forgot...it happens), but all and all, it went well. :) The sent him home on a strict Vanco lock regimen and IV antibiotics twice a day. Kind of a pain, but hey! It's worth it, if we all get to be at home as a family and Daylon has a healthy PICC line, right?

This is Daylon.





Daylon hates to be bored...apparently...so he thought he would spice things up.

What? Was he not there for the last 18 months?!? They're spicy enough, Little Man.

While I packed our diaper bag for dialysis, Caleb followed me around the house saying, "Mommy, Daylon broke his milk". Yes, I stopped and looked up at him, but everything looked fine, so I kept working...besides! How can he brake his milk? Once he started to raise his voice, I decided to go back.

 This is what he meant:

He pulled his entire PICC line out. Stitches and all. Nice.

I got the bleeding to stop and spent the next hour in a frantic, wondering to do with a gaping whole in my son's arm. Good news is: no more TPN!!!
 The bad news? Daylon has a high caloric needs for his age courtesy of the BMT and EB, a low mineral requirement (renal failure), however his phosphorus and potassium are dangerously low, low protein requirement (renal failure again), is unable to swallow anything thicker than...well, let me just say he chokes and gags on pudding (mass on the tongue), can't chew (head bleed), and throws up ALL. THE. TIME.  Needless to say, this has been a trick. Every morning I blend things like avocados and whipping cream and Suplena (high cal, renal failure milk) and strawberries together. It's been...interesting. Some potions he likes, others have resulted in instant vomiting. Live and learn, right? :)

mac'n'cheese shake (made with whipping cream instead of milk)

 There's one other catch in the "weight issue". Since we've been back in CA Daylon weight has slowly and steadily dropped from 14.2 kg (his dry weight) to 12.2kg. That's 4.5 lbs. Come to find out the prescription for his TPN in CA has had 660 cal, which is less than half of the calories he needs. We just found out this week about that, but it's nice to at least know why his weight was dropping. On Monday at dialysis he weight 11.9kg, but they filled him full of fluid and we left with his weight at 12.8kg. We're hanging out right now without the PICC because despite his weight loss, his labs look better than they have since transplant. Woo-hoo!
 We went to the ENT doctor today and he said that the growth does not like it's cancerous, but they'll have to wait until the results come in to know for sure. He needs to have the entire growth removed and it will more than likely grow back, but it's growing too fast not to come out. He will need to be intubated and will probably spend a few days in the PICU afterwards. He said that Minnesota might be best for the procedure, but he could do it if we choose to say here. Brian and I are really leaning towards Minnesota, but it will all depend on what Dr. Tolar says. I'll keep you posted.
 Now I'm not one to post naked bath baby pictures, but I had to show off his amazing skin!! Oh! Did I mention he actually took a bath with out shedding a single tear? Yep. See, it's moments like that that make this all worthwhile.

 One last bit of good news. We were FINALLY able to tapper off Daylon's CSA (it lowers his immune system to decrease likeliness of CSA, but thrashes the kidneys, makes you hairy, nauseated, and your gums swell) on Sunday night!!!  We are praying so hard that in a couple of weeks Daylon's kidney function will completely return! We have been so excited for him to be off the drug since July and it's here! After his last dose Brian and I "high-fived" and tossed that bad boy in the trash!

  I need to head off to bed. Good night everyone. Thank you for your patience. I'll try to post more frequently. :) We love you!

Keira's first day of Kindergarten in CA (at my old Elementary School!)

On Friday my brother flew down and we got the okay to take Daylon to a non-crowded beach. He can't touch sand or the Ocean, or be in the sun, so needless to say this trip was a quickie at night. Come to find out, the privacy was pretty easy. There aren't a slew of people who go out for a wienie roast in January when it's in the low 50s. Shocking. We sure had fun though! It was Daylon first trip to the beach ever!

note the lighter fluid in Jonathan's hand

fluid in flames

...and this one because it's fun and crazy like Daylon

  On Saturday the Corona Fire Fighters invited our family to come down to the station for a tour and visit. Our family just loves the fire fighters in our area. They have been so wonderful and sweet to Daylon and our family this last year. Before we left for MN in May, 30 or firemen came to meet Daylon so that if we ever had to call for emergency help, they would know him and his situation. Pretty nice, huh!

 of course the kids always love testing out the fire fighter stuff too!

The only time our girls have ever been twiners