Day +6: Sleep baby boy

First off, I want to apologize for not writing sooner especially after such a scary post for our last entry. I have legitimate reasons, but who wants to read a bunch of excuses! So....

Overall, Daylon's life has been better since he's been on the ventilator. They can give him as much Morphine as he needs without any worries of him not breathing since a machine is doing it all for him. They can gauge it off of pulse and blood pressure. There was some concern yesterday that the mucositis had traveled into his lungs. Today, however, things are sounding better and since that kind of swelling can't go down in a day, his lungs probably aren't involved. Whew! We were pretty nervous about that! Daylon gained almost 2 lbs yesterday and his poor little body was blister central!! He's lost 1/2 lb. today thanks to a new diuretic that has been added to his towering IV stand. Hopefully over the next several days will see regular Daylon appear! He's not blistered today nearly as bad as yesterday so that's wonderful news. His heart rate is in the 120's at the moment from the high 160's yesterday. He had a Vac Holiday today (were they turn off the meds. to see how long it takes him to start to stir) and he started to move an hour in. That's just where they want him! More good news you ask? Why certainly! His fever is FINALLY being managed by the Tylenol!!! He's at a cool 99 degrees right now. I wish they could have given him Motrin, but apparently it lowers your platelet count so it's out for the next two years! Yikes. I love that stuff! It's the anti-fever's best friend. Today has been a great day for Daylon! What a blessing not to see him suffer and to feel a day were you can breathe a sigh of relief.

Brian and I are dealing with the situation fairly well. Of course, days like today make it easier. I know it might sound crazy but I do miss him. I'm sitting 18 inches away from him for hours on end and yet when I go to sleep I can't help but feel like I haven't seen him in days. This coma thing makes him physically here, but that's all. I can't wait to hold him. Today when I went home to the RMH for a quick sec. I ran into Cowboy Danny's parents (a little boy a few rooms down with cancer) and they asked after Daylon. When I returned the question they told me that their son had just passed away. There's so much hope here at such a wonderful hospital, but inevitably, there's also death. They told me to tell Daylon to "fight and never give up". The mother also advised me to take advantage and hug and kiss each of my kids goodnight. When I returned to the hospital I wanted so badly to pick up Daylon and rock him, but for now, that will have to wait. We are blessed to have enjoyed our day with our son.

I've really felt guilty the last week that I've spent so little time with our other little guys. I've felt like I couldn't leave Daylon for fear of something happening. Yesterday, Brian encouraged me to visit with the kids in the afternoon. I planned out a picnic at Lake Calhoun. The kids and I had such a blast! It's one of several lakes around here called the "Ring of Lakes". We had a picnic, they played at the playground (a cool boat shaped playground that the kids went nuts over!), and we dipped our feet and splashed a bit in the cool water. Afterwards, we went for a walk around part of the lakes. What a view!! Minnesota grows on me more and more everyday. It's such a beautiful state! There were tunnels and rolling grassy hills, flowers and sail boats everywhere you look. Very picturesque. We just wandered around enjoying nature and each other. Moments like that with my kids makes my life feel so full. Daylon and Brian being with us would have made it absolutely perfect. Things to look forward to, right? On the way home the kids and I were listening to the radio and a favorite song of theirs came on. I looked in my rear view mirror to watch their four little heads bobbing along and rocking back and forth as they belted out "Soul Sister". Those four little voices reignited a part of me that had pretty much burned out over the last month. So, I did what any parent would do: I turned it up and sang along. Life is good.

Day +4: so much to take in

Daylon had a late night last night, falling asleep at around 3 a.m. He was breathing well with the oxygen he was receiving with his saturation levels in the high 90's (remember 100 is perfect, anything below 90 and they up their game). At 6:30a.m. Erin (our primary nurse), woke me up to change his diaper. Erin stood by his bed holding the mask to his mouth as I changed him. Daylon was agitated and his oxygen saturation level was in the low 90's and upper 80's. The charge nurse joined us in trying to make him comfortable. They messed with how much oxygen he was receiving, we sat him up, he was suctioned (which is just painful to watch, more or less endure I'm sure), I cupped his back (a trick I learned when Sadie has RSV last year), we rolled him onto his back to try to let all the secretions drain forward. It worked for a few minutes but eventually his levels fell again and his g-tube site and chest started to bleed, so we rolled him back. Some more nurses came (the shift was going to change) and the docs. came in. They decided to get another ICU consult and left to speak with the other doctors. By this time his levels were in the 80's and nothing was working to bring them back up. Erin, Rachel (the daytime nurse that was coming on) and I fussed over him. As the levels dropped he was no longer responsive and became limp. I watched his face become blue and then gray. I kept calling his name but he wouldn't respond. He wouldn't breathe. The nurse pushed the code blue button and two dozen people rushed the room, pushing me aside. He was bagged and EB or not, suctioned deeper, his clothes were cut off, and the sea of people grew deeper. I just stood in the corner completely helpless. It was one of those times in your life where it's like your numb and stuck in one moment while life rushes by you. It's like your watching your life from a third parties perspective. It's was only for about two minutes but it's played for hours in my mind today. They got him back to a healthier level and rushed him to ICU. He was there for about 30 minutes and then taken to the O.R. to have the breathing tube placed. The risks were great for the procedure because they didn't know what to expect. No one at this hospital has experience with intubating JEB kids. The airway may be too tight, permanent damage could be done to his airway, he could bleed excessively.


The procedure was about an hour. They said the airway was really tight. The rubbery camera was bending against the swollen walls of his throat, so they had to use a sturdier camera. It took some time, but it was successful. A ventilator now does all his breathing and he's saturation level is 100.

He spent the first several hours fighting his tube and new condition (that wasn't supposed to happen). He was given large child size doses of sedatives but he really fought it. That's just like Daylon. Tough. Daylon is now being given 3 new medicines: one for memory, one to sedate him, and one to paralyze him. He'll stay this way until the mucositis heals, which is at least two more weeks. The good thing about it all is now he doesn't have to suffer through the horrible side effects of transplant. I'm so very grateful for that. He deserves some rest.

After he was sent down to the O.R. I text everyone on my phone's address book and asked that a prayer be said for Daylon. Brian and I went up to the seventh floor in the hospitals meditation room to be alone and pray for our son. After we sent the text the replies were so comforting to us. Everyone was so supportive. Thank you. Again, it's nice to know we're not alone in this. Have a happy day.

Daylon and his lime green shadow

Day +3 : The fighter

We're three days in and knee deep in transplant chaos. Shortly after I posted last (like 30 seconds) Daylon's oxygen saturation began to fall quickly. From that moment until 5:45 this morning there was a team of medical personnel hovering over Daylon attempting to make his breathing comfortable...and possible.

Daylon and Nurse Emily using the "troft" -2 a.m.

EB kids have a bit of a challenging time receiving respiratory assistance since they can not be suctioned and many masks can tear the skin. Around 3am there was talk of him being moved into the PICU (Pediatric Intensive Care Unit) and have the breathing tube put in. The BMT docs want him over there before it's a life or death emergency so that he can have EB experienced Dr.s intubate and look after Daylon. The PICU docs want him to only have it when it's an absolute emergency since he has JHEB. After hours of not really knowing what was going to happen, they decided to wait a bit longer. His pulse/ox is being checked continuously, which is a EB challenge and he now has a nurse all to himself. It sounds like a casino in here with all the chiming. Another way they've decided to help Daylon breathe is by doing a light suction in his mouth. I'm not thrilled with the idea. It's grabbing secretions but it's also blistering his mouth. Kind of a catch 22.

Daylon and his dinosaur oxygen mask (w/ mepilex transfer for protection) - 4a.m.

The oxygen mask bandages finally perfected for Daylon! -6:30a.m.

Early this morning he also started to run a 102 degree fever which is being controlled with Tylenol. Thankfully all of his labs have been coming back infection free. From what I'm told, fevers are kind of the norm around here in immune system free world.

Late this afternoon Daylon began to vomit blood and the diaper attached to the end of his g-tube was soiled with blood. The labs that they ran following that showed that his platelet levels were low so a bag of platelets were added to his IV stand and his hemoglobin level dropped 2 points in 12 hours showing signs of internal bleeding (most likely from the mucositis). So like I said, we're knee deep in it now and if my emotions the last two days were any indication of how I should be feeling right now, I'd be on my way to the psych ward. But it's just the opposite actually. I feel totally calm. I was really able to enjoy the 20 or so minutes today that I was able to sit and hold my son..without oxygen. After last night, that was like it's own kind of little miracle. Yes, he is back on oxygen right now, but I know what a fighter Daylon is. He has a desire to live like few people do. One of our nurses walked in today and said,"This is the first time I've ever seen him where he's not laughing and hamming it up!". She's completely right! He has such a great attitude, pain or no pain. He loves life. What a great example he is to me. I can't wait to see him sit up and give me his great, big "Grrrrooowwwwlll!!".

Day +2 : steep hill to climb

So. What do you want to hear first? The good news or the bad? Let's do the good news first because I hate being a downer.



Alrighty. Well, first off, Brian's mom (Dee) came into town for a few days to visit and help out with the kids. This has made things the past few days so much nicer. I love being able to have Brian here off and on through out the day during this rough patch. She flew in last Wed. (I wanted to post about it sooner, but I didn't have a picture to show.) and will leave Mon. afternoon. It's been nice to see another familiar face, ya know?

More good news you ask? OK! Daylon's labs came in this morning and he's they show that he's doing great. Miraculously his hemoglobin level went from an 8.5 (at 8.0 you need a blood transfusion) to an 11. No one can explain why, but I'll take it! I don't need a reason! He was having some high blood pressure,so they increased his Morphine drip and it lowered his blood pressure. Guess his pain is back to a comfortable level for him. :) I neglected to tell you all but Caleb and the twins have had a flu bug. Brian told me this morning that they're back to their old selves. That's good two-fold: one, they're not sick and feeling crummy and two, I can start seeing them again! I miss my kiddos and can't wait to give them each a giant hug!

OH! There's been CRAZY thunderstorms out here! Tornado warnings too! The storm has died down (for the moment) and there's been no sign of a twister, thank goodness!

Now, because I can't put it off any longer, the bad news.


Daylon's worse today than yesterday, if you can believe it. The mucositis is consuming his little digestive tract. I can see blood and blood clots draining from his g-tube as I type. I can't hardly see what is happening in his mouth anymore because it is so filled with blisters and drainage. We literally have to turn his head face down to try to force out the mucus so he can breathe. He sounds like a lawnmower starting up. The docs have been in and out through out the day checking on him because they're concerned at how quickly the mucositis is progressing. His oxygen saturation level is in the 80's so they put him oxygen (blow-by) moments ago. They told us that if he keeps going at this rate he'll need to be sent next door to the ICU and intubated (tube put in that will run from his lungs, out his mouth and coneccted to a ventilater that will breathe for him). That scares me to death. There are so many variables to consider. Lets not think about that now. We're not there as of yet. Another problem that Daylon has run into is his body retaining fluid. He's been putting on quite a bit of weight over the past 36 hours and has all but stopped peeing. They've tried Lasix every few hours to help him drain the extra fluid but it's not working. Tonight they're starting him on another type of diuretic. With any luck, he should be back to his normal weight soon. That being said, being swollen is not something well tolerated by EB kids. His skin is pulled tight resulting in an insane amount of blisters. Of course, it doesn't help that his skin is breaking down as a result from the chemo as well. Today I used over 50 needles. Each needle represents one to two blisters that we've lanced. I am resisting the urge to show you all the pictures Brian took at his bath and dressing change tonight. Although EB skin/wounds have become part of our daily life, it's a bit much to take in when your eyes are first introduced to the sight.

Well, it's off to bed with sweet dreams of a better day tomorrow.

"Walk towards the sunshine and the shadows will fall behind you." - author unknown

Day+1- the start

I can't believe it's over. The bone marrow is there, in his body and (fingers crossed) getting to work. It's such an unusual sense of relief. Presuming that all goes well, it will be years until the EB is gone, but by day +60 we should see some marked improvements in his skin which also means improvements in the quality of his life. I'm so very excited. I can't wait to see him play with others, and not be in pain. I can't wait to really hug him. It's been hard to watch him suffer and then suffer more when I comfort him. Brian really seems to think that he'll be healthy at 5; I don't know why. Five is a good number, though. I would definitely be happy if that were true.

So, it's the start of good things to come. Now for the unfortunate truth: it's also the start to some bad.

Yesterday afternoon Daylon started crying and was inconsolable. Once I got him to sleep, I noticed he was having a tough time breathing, then he woke up and started gagging. We thought he was just going to throw up (which is odd since he's not eaten anything at all for two days) but then he started to not be able to breathe in. The mucus membranes were rupturing in his throat and he was choking on the fluid. Within a few minutes and several hardy chokes, his eyelid started bleeding and blood was going into his eye. This dry heave thing went on for three hours. There was nothing they could do. You can't suction because his mouth would tear. He cried and cried. His cry is just this pitiful, sad break your heart sort of sound. The parameter of his tongue looked like a turtle shell with mounds of blisters. There were on the roof of his mouth, his gums. It was overwhelming how fast they sprung up. Some bloody. Some regular. They eventually gave him a few doses of morphine and he fell asleep around 3:30 a.m. He woke up this morning and it was the same thing again. Instant crying, choking on mucus (result in bubbles near the size of his head. No joke) and breaking blood vessels . His tongue is so swollen it rests on the his bottom teeth. It's so blotchy and painful looking. He can't sit up on his own anymore and even with help he struggles.

before chemo

today

His skin has become a story of it's own. It's like he's a newborn again. The blistering is becoming more than I can keep up with. The slightest touch, and he blisters. Spontaneous blistering is what it's like. No matter how gentle I try to be, I still hurt him. I hate being the source to his pain. I kissed him last night and he blistered. That hasn't happened in months. Overnight, his chest looks like ground beef. He is in more pain than I can imagine. The constant Morphine drip is the only way he's napping now. This is the hardest things I've ever had to do. My precious little boy has suffered more than I've ever seen another suffer and now, he suffers more. The Dr.s say that with his form of EB, the mucositis is hitting hard and hitting early. We knew that was a risk. We knew that was a risk and we took it to try and save him. Now that's it's happening, it's hard to internalize all of it. The doctors have told us that it will get harder over the next 10 days and he'll continue to suffer for the next 20 until Caleb's bone marrow starts to produce cells. I'm not sure how that's possible. My point is, this morning I felt like I did a few days after I had Daylon. I was crying like a hormonal post-birth mother, feeling so scared and unsure of what was in store for my son. When Daylon was diagnosed, I remember walking out of Dr. Metz office and making it to the elevator before Brian and I started crying. I've never felt so lost. It was us against this horrible disease that I had never even heard of, and all we knew was that within a year, the disease would win. Nothing could be done. That is a feeling that no one should have to experience. Overtime, we've educated ourselves and tried to give our son a meaningful happy life, no matter how long. It's brought us happiness in return. Today, when that feeling began to take over my mind, Brian and I prayed together and began to talk about all of the people in our lives who offer us so much love and support. We read the comments that you all have posted and retold each other of the acts of kindness we have seen and felt over the last 11 months and the best thing happened.That awful feeling went away. I know we're not alone in this. We have you. Thank you so much. Thank you for praying for our son. Ya know, his skin has cleared up so much over the past two months that everyone around here is amazed at how he looks. His skin durability tests extremely low, but his skin looks so good. I know in my heart that it's because so many people are praying for Daylon. I also know that if his skin were to have stayed the same as it was a few months ago, the effects of the chemo would have been fetal. I do not think that the Daylon's condition as of March could have handled such a severe reaction. We really feel that it's saving our son's life right now and it's giving us sanity. From the bottom of our hearts, thank you.

TRANSPLANT DAY!! Caleb to the Rescue!

The big day has arrived!

Caleb, our little hero
Brian had to bring Caleb to the hospital at 5:30 am. The hospital has been so great about making the kids understand what is happening and helping Caleb's nerves to stay calm. Yesterday they gave him a mask just like the one they used today to knock him out and he got to pick his favorite lip smackers to scribble all over the mask. He chose the sweet smell of rootbeer to fall asleep to! Caleb's bone marrow was pretty concentrated, so they were able to get what they needed in the sample alone! Were still waiting on the blister te

st results, so we'll keep you posted.

Caleb is already playing and wanting Daddy to "fly" him through the air.What a speedy recovery!

He's a bit loopy, so we just can't help but laugh at some of what he says and does!

Daylon's transplant was at 12 p.m. The chemo is of course, still wearing on him, so he wasn't always the happiest little guy. They hooked the line up to the tip top of the central line and let it drain into him as fast as gravity would allow. The line was a little clogged at first but after a flush and moving him, it went much faster. It was over in no time! Transplant stage complete!

Ready! Set! GO! Last few drops!

The hospital is really great about making each kid feel special. Each of the kids got a soft teddy bear and the boys each got HUGE balloons and two toys. Caleb loves his new Cars toys and Daylon is really enjoying his new mobile and glow worm.

I want to get this out asap, so that's about all for now.

Oh! PS- Brian and Caleb were wrestling yesterday and Caleb hit Brian in the mouth with his noggin. Yep, he loves sporting his new look! Woo-hoo! The dentist saw it last night and will have it all fixed up tomorrow. Thanks for all your kind words and prayers! They keep us going!

GROW CELLS! GROW!

The girls showing off their new butterfly shirts in support of their "Butterfly Baby's" big day

Chemo's kicked in

The title pretty much says it all. The chemo has taken over Daylon's body and it's a depressing thing to watch. As big of a pain the Busulfan was (having to do some many frequent dressing changes) the Cytoxin is sooo much worse. He's so weak that his cry isn't even a real cry. Not to mention all the vomiting has blistered his insides making his voice hoarse once again. He's on three different nausea medications and they have seemed to help. Yesterday he started on TPN (IV nutrition) because he was down to 6 oz. a day. A far cry from his normal 40-50 oz. It's also helping with the vomiting. Nothing in, nothing out...sort of. I don't mean to sound negative, we know that this is, unfortunately,one of the hard things he has to do to get better. It's just that it's been an emotional few days. It's so hard to watch Daylon suffer his whole life and now he's suffering more. I pray this transplant works. On top of feeling rotten from the chemo and his 1 year old molars coming in, he has to deal with the pain of bandage changes all day. Yes, it was just the Busulfan that required frequent changes, but when he vomits, they get dirty and need to be changed. Thankfully, I can get away with only doing the spots that are wet. He's not gagging and doesn't know how to commuticate that he's going to get sick, so I've yet to be successful with draining through his G-tube.

One other difficult thing about the Cytoxin, is that it can cause kidney, bladder issues. To keep it under control they run a constant flush and require that his diaper be changed and weighed by a nurse every 2 hours, 24/7. He's not the best sleeper these days so I end up waking him up most of the time. Sleep is a thing of the past. The crummy thing is if his diaper doesn't weigh enough than they give him medication to make him go within a half an hour. Yep, another change. Another half hour to get him back to sleep. It stinks too that we have to be super careful about handling him (in a non-EB way) since his body is expelling the chemo through his skin and urine. Everything gets thrown away (cloth diapers and onesies, I mean). Everything is wiped down with super strong, odorful cleaner and we have to be double gloved to change him.

On a happier note, Daylon's organs are all still function properly! This is a big, big deal. His organs stand a higher risk of deveoping life threatening issues than the other EB kids, since his form EB is internal as well as external, so the chemo is super risky. The doctor just came in and said that they're all really excited to that Daylon went all the way to Day -3 before he needed the TPN and they're floored that he hasn't run a fever the whole time! Infection is really common when you don't have an immune system and Daylon's sores allow for plenty of bacteria to grow. The final great thing is that today is his last day of chemo!!!! The effects of the chemo will still be around for a few more days, but at least he's done with that stage! Tomorrow is a rest day for his body and Thursday is the transplant! It's all happening so fast now. Once you start moving, it really goes.

Father's Day was, of course, on Sunday and the kids came down for their weekly visit. The nurses let us all be in the same room (3 person limit due to the room filters) for a few minutes to watch Brian open gifts and take pictures. Since Daylon started getting really sick on Saturday, I stayed up at the hospital and Brian was back at the RMH with the kids. Keira woke him up (all on her own!) at 6:12am and brought him to the table where she had made him a breakfast of Nilla Wafers, chocolate chip granola bar, blueberry yogurt, some rasins and a chocolate pudding cup. She's not allowed to use the stove, so she worked with what she had! I'm so proud of her. Brian said it was the best Father's day breakfast he ever had. When they got to the hospital and walked into Daylon's room, he pointed and said, "Da-da". Brian was face was beaming. It was so stinkin' cute!

Ya know, there wasn't a fancy breakfast, a bunch of presents and a day filled with all Brian's favorites, but as I'm coming to realize more and more, you don't need all those things to make someone feel appreciated. Brian seemed happier in the 20 mins. we all spent together in a hospital room, than he did in the full day celebrations of the past. It seems simple enough, and I always thought that was the case, but now I know it. It's really been such a blessing in our lives to raise a sick child. We've become mindful of many parts of everyday life that were always moments and decisions we took for granted . Each day you have together is a gift. Cherish it.

Day -6 is ending!!

Daylon and Erin getting ready for a bath and dressing change...at 1:30 a.m.! Daylon has us all working graveyard!

One of the things we absolutely love about this hospital is that the patient and family pick the nursing staff that works with you. You get to pick a primary nurse who is the head of your care team and each nurse is only allowed to be the primary on one patient team. Additionally, you pick three nurses for each of the three shifts worked each day. It's such a great program. We had Erin six of the eleven nights last time Daylon was admitted and we loved her. We chose her to be are primary nurse and it's working out great. She's familiar with Daylon and his bath routine which is so wonderful!! Daylon just thinks she great, so we love that of course. She's very attentive to our family and really keeps on top of Daylon's medical status. Plus, she's willing to sing during his dressing changes!! It so nice to work with the same few nurses and build up a comfort level with them. I feel like I can go to them with any problem and because they know and care about Daylon, they work immediately to resolve whatever problem is at hand.

Brian had the kids all day today and most of yesterday. I'm so impressed with how well he's handled all four kids. Taking four little ones out is no easy task...trust me! He took Violet (and the other kids) to the doctors yesterday to get her staples removed. He said she was a tough little girl and didn't even make a peep. What a blessing! Tomorrow starts a new, more intense form of chemo. We'll let you know how it goes! Giddy-up!

So many pointy things so close to his skin!

Finishing up Day -7

Well, Day - 7 is coming to a close here in a few hours. Just one more day of Busulfan (one of the chemo drugs)! I'm particularly excited about this because the rule of Busulfan for EB kids is dressing changes at least once a day since the chemo is released through the skin. Blah! Last two changes have taken 2 hours and lots of tears on his end and frazzled nerves on mine. I don't think I'll ever get used to EB, emotionally speaking.

Daylon is still in good spirits but the chemo is starting to take toll on him. He's not been eating as well as he usually does.Really it's about a quarter of what he does normally. They Dr.s today talked about adding TPN (it's IV nutrition) to his lines soon. He's still able to sit but he's really wobbly now and tires out quickly. I've learned that the hard way when he toppled over this morning and hit his head on the crib. It turned out to be a pretty bad wound, poor baby. He actually got hurt a few times today, but we were warned that his skin would brake down even easier these days. His hand in the picture is one of them. As you can see though, he's still willing to play! What a champ!

The hospital encourages family life living, so each BMT room is nicely painted (we have a blue accent wall), but outside of that, it's a empty canvas. They want the family to turn the rooms into "bedrooms" with photos and decorations. Studies have shown that people heal more quickly in a comfortable home atmosphere, which makes sense. Anyway, I went to Target yesterday when Daylon and Daddy were getting some daily bonding time in, and I snagged these great animal safari decals on clearance for $10. There were a bunch of animals in the pack so I was able to do the whole room! Pretty cute, huh!? The Care Partners program gave us a gift bag that had some window crayons, so the kids drew pictures on the windows for him. He likes to stare and point at the touches to his room so we'll call it a hit!

Speaking of "hit", the RMH Summertime activity this week was drama camp with a special show of "Annie" on the last day. The play was sooo stinkin' cute! Keira was only there one of the three days but she was GREAT! Yep, I'm one proud mama! They even made the costumes! It's kind of funny story but every time I would look at Keira during the play she was so serious. I even tried to get her picture before hand, when they first walked out, but she wouldn't smile. After the play, same thing. I took a few serious pictures and I asked her to smile but she wouldn't. Finally, I gave up with the pictures and she asked to take off her costume. The second we did, she started smiling and jumping around. I just laughed at her apparent hatred for her costume, and asked her why she didn't smile like that before. She matter-of-factly replied, "I was being a sad orphan, remember." Way to stay in character!

Ally (Bella's sister, left), Keira as "Pepper" (center), Emily (right) Sorry about the cell phone pix quality.

The orphan cast of "Annie"...Keira's bottom right

Thanks for all your thoughts and prayers! They're working!