July 31: Day +29

Tim Ringgold reporting to you live from West Twin Lake in Minnesota. After a day that started in rounds in the ICU, I feel a thousand miles away as the quiet, still air off the lake permeates through the open windows of the cabin. Today I saw a mother bald eagle perched on a tree limb overlooking the lake, and I saw her son, now essentially a teenager, fly right by us with his 6' wingspan. Turns out the family have a nest two doors down.

I started my day in rounds since I left my guitar in Bella's room last night, and really wanted it for the cabin. Things are mostly unchanged in Bella's world since yesterday. I will post more tomorrow night when I am back at RMH. In the meant time...

Here are the numbers:

Weight 12.1 (down .1)

I/O +210 (circuit was down for 3 hours, so that's how we added 210)

WBC: 8.4 (okay)

ANC: 5.8 (okay)

BILI: 1.7 (GREAT! continues to drop!)

CSA: 214 (MUCH better... Dr. Tola's new target is 150)

Today was filled with fun on the lake. Jet skiing, waterskiing, tubing were all on the menu with fishing slated for tomorrow after breakfast. IT's been a good break, and Ali is having a BLAST. She gets along FAMOUSLY with our friends' boys, Jack (6) and Ben (3).

I am beyond exhausted, and have endeavored to leave as much stress out on the lake as possible. I know I will be sore for a few days from this "stress exorcism." At the end of the day, I played "Amazing Grace" on my native American flute on the dock, and let it ring in thanksgiving for such a wonderful day. Then, after an AWESOME dinner cooked by my buddy Bob, I sang a couple of fun kids games for the three kids while I also played some guitar.

A great time is being had by all. Thanks again to Bob and Lynne Boschee for taking me in under their wing yet again when I needed it so badly, and thanks to the boys for welcoming Ali! God is good, all the time!

Good night.

July 30: Day +28

TGIF!

Tomorrow, Ali and I head to the lake to stay with our dear friends, the Boschee's, at their cabin for some much needed R&R. I'll bring Ali up tomorrow, spend the night, then come back Sunday. Ali will spend the week with the Boschee family, then Angelique will head up next Saturday, spend the night, and they'll return together Sunday.

I lead with this because tomorrow looks different than today for the first time in a long time! I alluded to "the Groundhog Day Effect" a couple of nights ago, and living into that quantum sameness has really sapped my energy tanks, as you have no doubt detected in my writing. We humans live for the future; that's why TGIF is not TGIS where S equals Sunday! LOL. So, that new future really did two things today: it made today (mostly) fun, and it made it go by FAST.

Today was a dressing change day, which now has also become a circuit change day for the PrismaFLEX dialysis machine. We have to coordinate the two, because in order to change the dressings on the back of Bella, we have to be off the continuous dialysis machine, otherwise rolling Bella on her side to treat her wounds would kick us off the circuit. The circuit (the Prisma machine) is unbelievably sensitive, and it shuts itself down for the most minor of offenses.

Anyhow, because it is quite a procedure to stop the circuit and restart it (I will spare you the details as to why that is the case), AND it is hard on Bella's already fragile blood pressure, we kept the circuit running since Monday and put off dressing changes for 5 days. It was tough to say what her skin would look like after going that long, but at THIS time, her skin is in better shape than her kidneys, so it had to take a back seat to the needs of the kidneys.

The one area I was nervous about was the upper back, neck, and back of head. This area seemed to be breaking down on last inspection, so last dressing change I thought I covered and protected the area well with enough transfer, with a little Alwyn cream underneath to help it heal. Unfortunately, the skin in these areas was for some reason too macerated, or wet, and it just sort off, well, rotted. I actually cried while trying to clean it. There I was, standing on one side of the bed, with the RT and a nurse holding Bella on her side on the other, with another nurse in the room prepping the Prisma. I said something like, "Um, I will keep moving here, I promise, but I think I just need to cry. No one should have to see their baby's skin look like this." and with that, the tears just came out. It was really awkward; the juxtaposition of this tender moment cleansing my daughter's wounds and absolutely horrible looking skin with the sterility of the three nurses in their yellow contact gowns and blue latex gloves under the oppressive fluorescent light and beeping sounds of machines. Somewhere in background, quiet piano music was playing on my boombox... too quiet. Life in a fishbowl.

Fabulous socks of the day

So, we will try changing the circuit on the 4th day this time. So if today is Day 1, Sat = 2, Sun = 3 Mon = 4. On Monday, Bella will have her fist skin biopsy since transplant and a professional photographer will come in and take pictures, so it is perfect timing in that regard. Other than her back, the rest of her skin hasn't shed or broken down like I feared it would. Our main problems from chemo are internal, not external. With that...

Here are the numbers:

Weight: 12.2 (down .1)
I/O: +178 (had to give Bella some saline yesterday to boost her blood pressure above the drugs I described yesterday)
WBC: 8.5 ( YAY! up from 7.9 yesterday)
ANC: 6.1 (6.2 yesterday, no biggie)
BILI: 1.9 (YAY! Drop, Bili, drop! Her baseline was .5 before she started showing signs of V.O.D.)
CSA: 388 (after emailing Dr. Tolar, he emailed the BMT team and they and pharmacy were ON THE STICK this morning! Good. New target is 150 and Pharmacy is finally on the ball.)

After dressing changes, we restarted the Prisma machine and Bella tolerated it well. Prisma is our lifeline right now until Bella's kidneys start working again. To that end, there is a hint that they are still not completely beyond repair....

... today Bella started leaking the tiniest amount of urine on several occasions! This is HUGE, folks. The best indicator of if and when her kidneys recover in the short term is that she starts peeing. So, to see drops just leaking out during diaper changes was so exciting! I can't believe I am so excited about pee. I'll live to regret this at some point in Bella's future, I'm sure, but for tonight, we'll take all the pee we can get! HAHAHAHA.

Ali on the slide at her Pre-School Open House

Open Houses are for... cupcakes?

On the nursing front, we had a new nurse as I knew our primary ANGEL Lori would be off today. I walked in and Amy greeted me with a smile, and a GREAT attitude. As I knew it was her first time with Bella, I quickly gave her the pep talk. Because today was going to be a circuit change and dressing change day, it was gonna get pretty intense from about 8-2, so I explained to her that it took a second nurse being in the room for about HALF the shift on Monday to handle all the complexities of Bella's care on a day like today. I told her to call her backup early and often, and don't feel the least bit bad about it; Bella requires an extremely high level of care. She took it all with a smile, and did great. It's not whether or not a day nurse is going to fall behind with Bella, (it's a given), it's how they conduct themselves WHILE they are behind that makes all the difference. Amy kept a positive, interested, and connected attitude the whole time, despite how stressful it got (and it was pretty stressful from 8-2 nonstop). Now, when I am working along side someone like that, I am HAPPY to train, teach, and share. I kept cheering her on throughout the day when I could see her getting a little beat up by all the meds/labs/care she was juggling, and complimented her repeatedly on how well she was keeping it together, and took the time to share the same with her charge nurse while she was covering Amy on a break. Amy worked a 12 hour shift, and it was an easy, fun environment filled with partnership, good conversation, learning, and a few laughs.

Last but NOT least, please double your prayer efforts not for us, but for Daylon tonight. His parents got some pretty intense news today after they did an MRI to assess the damage of the brain hemorrhaging he suffered. Daylon's life lay in the balance right now; it's really touch and go. THANK GOD Jennifer's mom is in town taking care of the kids so Brian and Jennifer can be with their beautiful little boy through these darkest of times. You can read more (so you can pray more clearly) at their blog:

http://lovingbabydaylon.blogspot.com/

We love them like family, and humbly BEG for every last ounce of your spiritual energy to be POURED OUT for Daylon right now.

Thank you. We know there are so many out there following this journey our families are on. Your prayers keep us afloat. This is so far beyond anything WE could comprehend, and given that we've just endured a year with EB (which already contained events far beyond our imagining), we are simply in uncharted waters of fear, pain, and suffering.

BUT... we are also in uncharted waters of love, humility, passion, devotion, commitment, perseverance, resilience, strength, and duty. So, remember that pain and suffering often give us opportunities to break through into something new and wonderful, just remember to look and ask for the lesson or blessing in all things. It's there, it's our job to find it.

Good night.

Ali pretending to be mommy at work...

July 29: Day +27

And another chapter of "Groundhog Day Part II" concludes...

SCENE 254: "Run in with Dr. Tolar in the Hospital Lobby"

Jakub: "Hi Tim! How's Bella?"

Tim: "Stable."

Jakub: "That's what I've heard. That's why I haven't been around."

Tim: "And that's why I haven't emailed you!"

[mutual chuckle]

Jakub: "How are YOU?"

Tim: "Tired."

Jakub: "Yeah, you look it."

Tim: "Hey!" (Tim hits him in the shoulder, pretending to give him a hard time.) "Yeah, I know I do. You know you're tired when you look in the mirror and scare yourself!"

[another mutual chuckle and they depart in opposite directions]

[end scene]

Later that day,

Dr. Wagner appears in Bella's room while Tim is finishing changing the dressings on Bella's Hickman and PICC lines...

...okay okay, that's enough. I'm new to screen writing... in fact, that was my first attempt up above. I know, I know, don't quit my day job.... uh, too late.

Forgive the above ramblings, I get bored with myself after writing so much over the past 2 months!

Alright...

Rounds were relatively uneventful except for the fact of meeting the new attending BMT doc and fellow. They are nice. There are some pretty complex things happening inside Bella right now, and I'll do my best to explain it in laymen's terms, but first...

Here are the numbers:

Weight: No weight yet today (will be done tonight and will have that data in rounds tomorrow)

I/O's: -22 (YAY! We were shooting for a net of zero, so that's pretty close!)

WBC: 7.9 (YAY! almost DOUBLED since Tuesday!)

ANC: 6.2 (YAY! MORE than DOUBLED since Tuesday! Go Ali's cells!)

BILI: 2.4 (still hovering in the <2.5>

CSA: 431 (I give up on CSA. No one seems to have a clue about these levels, the lab is CANCELLING orders in the computer on their own, Bella is on a tiny amount, and it had been lowered even more, and she's still gone up from 297 on Tue to 389 yesterday to 431 today. Again, with her in renal failure already, the LAST THING her kidneys need is too much of an extremely toxic drug hanging around. Goal is 250, max is 400, average over last 10 readings is 436. I put in a "strongly worded email" to Dr.s Tolar and Wagner tonight... hopefully they can get this under wraps by Monday)

Now to the more detailed stuff... wish me luck here.

Bella's blood pressure has been on the low end for over a week now, the result of vaso-dilation, or expanding (more specifically, inflammation) of the blood vessels. In fact, she has been on (now) 2 different vaso-constrictors, or 'pressers' to squeeze her blood vessels to help increase her blood pressure. Dr. Kooy, the attending ICU doc, gave a great analogy tonight to understand what's happening inside Bella:

Picture a garden hose. Turn it on a few turns so the water is flowing, but not gushing. Now, if you made the hose thinner, with that same volume moving through it from the spout, it would SHOOT out the end, like when you have a nozzle and just let out a little at a time. The pressure inside builds. Now, imagine that you made the hose much wider, and sent the same stream of water through it. It would lose its punch when it cam out the end, right? This is how it is with Bella's blood vessels right now. They are inflamed, so they have expanded. Now, that same blood volume she has flowing through her loses it's punch because it has to fill a larger tubing throughout.

The big 64,000 question is...

...why are Bella's vessels inflamed?

We don't have a clear answer. Infection is a common reason for inflammation, but Bella is on great antibiotics, and all her cultures keep coming back negative. They are looking behind and under every rock, and can't seem to find the smoking gun. V.O.D. causes inflammation, and Dr. Kooy felt that it may cause systematic inflammation of all her vessels, rather than just the localized inflammation we have seen in the liver ultrasounds. So, could it be the V.O.D.? Could be. Dr. Stephanski thinks it could just be BMT (chemo) related, but Dr. Wagner thinks if it was BMT related, it would have receded by last week. So, like I said, we don't have a clear answer.

This vasodilation throws a wrench into our dialysis plan to get the remaining 1.5 kgs of fluid off, because dialysis lowers blood pressure as it squeezes the excess fluid out of the blood. the theory again is that as you pull fluid off the blood, it will draw fluid out of the vessel walls, and as they "dry up" a little, they will draw the excess fluid out of the neighboring tissues, or "third space," where the excess fluid is sitting. Once we get that excess fluid off, we can then begin to talk about getting Bella off the ventilator. So, it's kind of a case of dominoes. The V.O.D., or some other cause, is lowering her blood pressure, which makes it harder to take fluid off from dialysis, which delays how fast we get off the vent.

We are now at a rate where we are pulling off all the fluid we put in by the end of the day, but we can't seem to pull off any extra without wreaking havoc on Bella's blood pressure. The body evaporates a certain amount of fluid through the skin naturally every day. This is called your "insensible losses." The docs calculate that Bella is roughly burning about 200 mL a day on her own through insensible losses. To get 1500 mL off at 200 mL a day means we are looking at 7.5 more days on Prisma IF we net zero every day for the next 7.5. Luckily, we have netted a +88mL over the past 7 days, so it is possible. The other piece is that Bella's blood pressure needs to hold its own during that time.

Soooo... we are on two key meds right now to try to 'urge' the body to shift into a more favorable environment. The first is called Norepinephrine. This is a 'vaso-constrictor', or vasopressor that squeezes Bella's blood vessels to try to keep that garden house from getting too wide. In addition, it does so while avoiding s queezing the heart, which had been squeezed a little dry by its predecessor, dopamine. So, we are off 'dopa' and on 'norepi.' The second is hydrocortisone. This is a steroid that has an anti-inflammatory quality to it. The idea with this is to stimulate the adrenal glands to produce more steroids in the body to combat the inflammation internally.

Hopefully, these two drugs used in concert will continue to keep the symptom of vasodilation at bay while we gradually pull off the rest of this fluid.

Well, I have killed my battery again trying to work this post out in as clear a way as I can. Tomorrow, I promise to bring home my plug so I can finish a whole post and get you some much needed pictures!

Pray for no more swelling, no more leaking, and

July 28: Day +26

Groundhog's Day...

...is the best context for what life is like up here. The days just roll by one after the other and it is hard to tell what day it is. The past looks the same, and no one is giving us any future past "one day at a time." So, we live in the eternal present, moving from morning to afternoon, to evening, to bed. Wake. Start all over again.

Within that sameness there are little changes that are like blips on a radar screen. Bella has grown tolerant of her sedation meds and has become a bit of a jumping bean on her bed. It's a little freaky to watch, because it looks like she's getting electrocuted, which in fact is not far from the truth. Signals to move are reaching out through the fog of sedation and muscles are firing in the most haphazard, uncoordinated way. When you watch her vitals, they don't appear elevated, so it's hard to say that she's agitated, upset, or in pain when it occurs, but it is scary to watch. I asked about seizure activity as well, but the muscle spasms don't resemble seizure activity, her pupils look good, and her vitals are stable, so THAT is a big relief.

The good news about this is that as she moves through awake and sleep cycles, her activity is appropriate. There are hours at a time that she is "deep" and resembles the peaceful slumber we've observed over the past 3 weeks. However, when she's "light" she has been progressively more and more physically animated over the past 72 hours. So, the docs upped all her sedation meds by 10% today after we gave her close to 20 combined boluses yesterday on top of her continuous drips.

Here are the numbers:

Weight: 12.3 (good... down .5kg from yesterday)

I/O's: +124 mL (not sure how she's up on fluid and down on weight... but it's only up a whiff)

WBC: 5.8 (YAY! up from 4.0 yesterday)

ANC: 3.8 (YAY! up from 2.6 yesterday)

BILI: 2.3 (holding steady in the 2 - 2.5 range - a good thing - means V.O.D. is not progressing)

CSA: 389 (up from 297. again, no one can make any sense out of this as they have been lowering this drug over the past few days)

They switched 'pressers' from dopamine to norepinephrine. This drug will squeeze more fluid out of the blood vessels throughout the body without drying out the heart any further. We've also had to go back up to the full "stress dose" of hydrocortisone to attempt to keep Bella's blood pressure up.

On a good front, we went up on J tube feeds again today, so the more formula she gets in her j tube, the less TPN and saline she needs in her PICC.

On a sweet side note, it was Ali's first "Open House" tonight. It was really cute having mommy and daddy getting to see Ali's art on display at school. She was so proud. Awesome parent moment amid a lot of not awesome parent moments.

Gotta run; my battery is about to die. Thanks for your ongoing love and support. More tomorrow!

July 27: Day +25

This beautiful quilt arrived in the mail as a gift with a matching one for Ali from one of Angelique's high school mates. Thank you, Heidi!

Here are the words:

A quiet day for some,

but not our new nurse,

annoyed by the care

that she had to give,

despite her job title:

INTENSIVE care nurse.

The manager? Off on vacation.

Her cover? The one who paid no attention

to the last time I asked

for a nurse to steer clear,

yet through Bella's door,

recently in walked this dear.

Left with only one option,

the charge nurse umbrella

she came through for me so

with the right nurse for Bella

A seasoned professional

even keeled and cool jets

Please, no more rookies,

just give me the vets

who talk less and do more

with a smile and an ease

that allows us to rest

I beg of you please.

I'm left unto verse

to spend my words freely

For the prose that precedes

today's post is clearly

not enough to persuade

the powers that be

so I turn unto you

please Lord can't they see?

It's not automatic

to care for EB

I know this, you know this

so how about we

stop for a second

take a breath, maybe two

then find your self present

with what there's to do:

Listen,

Learn,

Follow,

Expand,

Relax,

Breathe,

Smile,

Make eye contact,

FAST

For my patience is low

and my fury is high

I am not here to teach you

but maybe I'll try

to be more forgiving

patient and nice

if I could be willing

maybe that would suffice

because to wish any different

is fantasy and lore

your here on you "shift"

like it's one big chore

never mind that a life

may hold in the balance

just as long as you get

your break allowance

you'll check all your lines

and empty your bags

but don't touch my daughter

unless you have asked

what works and what doesn't

the right way to treat

a helpless mute child

whose skin is so weak

that fingertips tear,

tape shears right away

she can't tell you how

you have hurt her today

It's left to me,

on this little page

I have to release this

my soi disant rage.

Here are the numbers:

Weight: 12.8

I/O: +236

WBC: 4.0

ANC: 2.6

BILI: 2.1

CSA: 297

Creatinine and BUN are being dialyzed so they are no longer relevant numbers.

BMT docs are not worried currently about WBC and ANC numbers. CSA is back on track. Bili continues to drop which is SUPER.

The goal is to end up at ZERO for I/O's right now as Bella's blood pressure is a little soft and her echocardiogram suggests we might be pulling fluid out of her blood faster than it can infuse back in from the areas it has built up.

Nanny leaves early tomorrow morning, and we say THANK YOU, NANNY for helping out so much these past two weeks.

Docs are overall pleased with where everything is at right now with Bella. Now, if I could just get some headway with the new nurses Bella keeps getting. Now that she is on Prisma, there are only certain nurses that are trained on it, so the applicant pool gets smaller. The more experienced nurses are where it's at. They aren't freaked out by EB. I hate that I am so worn out and it is more from the staff than my daughter's condition, and P.S. she's in CRITICAL condition.

I'm sure many of you have faced this dilemma in your lives at some point. 23 days in an ICU and I have no buffer left. The difference between getting a good nurse for Bella and a bad one makes or breaks the day, and I thought I had my fill of nurse manager conversations. Guess not.

I need some rest. Thanks for listening; it is stormy outside tonight, and apparently it's a little stormy in here as well! Good night.

Ali catching a "daddyfish" with her Barbi fishing pole. Don't ask what kind of bait she used.

Ali decorated cupcakes for us for when we came home from the hospital tonight! Sweetheart!

July 26: Day +24

Well, tonight is a change; I am posting from the room because we are having some Prisma issues, and I don't really want to leave.

Anyway, Monday morning rounds began with a new resident and a new attending ICU doc. Luckily, Dr. Verneris and Christina, the BMT fellow, were present, so I felt like we had a couple of heads in the circle who could offer context and perspective on Bella. It was more like the three of us bringing the two of them up to speed on Bella.

Here are the numbers:

Weight: still no weight, though tonight we think we figured out what was causing the crib to report false weights, so when things slow down, we'll try again and see if we solved the great weight caper.

I/O: -244. YAY! Even better than yesterday! We finally hit our goal of -240 or greater.

BUN: 11 = GREAT

Creatinine: .40 = GREAT

WBC: 5.4 = slight drop from 5.7, but no biggie

Hemoglobin: 11.5 = Good

Platelets: 26 am / 26 pm = we now check 2x a day to stay on top of this. 20-30 is good, but anything less than 30 and we give Bella more platelets, so she got platelets twice today. Okay with that. Adequate platelet counts help prevent any bleeding from defibrotide.

ANC: 3.7 = YAY! Up from 3.4 yesterday

Bilirubin: 2.4 = YAY! down from 2.6 yesterday

CSA: 532 = BAAAAAD. We took another reading this morning and it came back 726 and that's when we officially knew that someone is not doing something right. We drew 2 more labs at 3pm, one from Bella's PICC line, and one from the Prisma machine to see if somehow the PICC line was contaminated, and we got 368 from the Prisma and 297 from the PICC line so WHO KNOWS WHAT THE HECK IS GOING ON... NOBODY HERE SEEMS TO. Turns out, people were making a simple mistake when drawing her blood to get this number. Simple, like make sure to clamp the second line before you draw from the first one.

That's it.

Seriously, you would be amazed. I think we live in an "ivory tower" fantasy about health care in this country. Remember when the Walter Reed scandal came out? I remember thinking, "Mildew runing down the walls of the patient's rooms? WHAT? That just CAN'T HAPPEN IN AMERICA!" Well, I remember when one of the orthopedic surgeons operated on the wrong leg of a patient a few years back at my local hospital. Last time I had knee surgery, I was given a sharpie in the pre-op and was told to DRAW AN ARROW on the thigh pointing to the knee that the doc was supposed to operate on. Why was I the one determining this? Because the doc at my local hospital apparently wasn't the only one operating on the wrong leg.

I spew all this because I have to remind myself, and everyone reading this that we "practice" "health" (sick) care and patient care. And yet, the reality is also that from 2006-2008, just under 100,000 Americans died in American hospitals from direct patient care errors, according to the 2010 HealthGrades Patient Safety in American Hospitals Study. I remember learning about this during my own hospital orientation. That's a sobering number, folks. So, when nursing staff get flustered because there is a lot to do, it bugs me, because I know mistakes are made when people get flustered.

CSA is an incredibly toxic substance, and I don't need people pumping unnecessary amounts of this drug into Bella for no good reason. This is the second time CSA levels have gotten weird, and when you're kid is already so sick and is listed as in critical condition, the buffer is ZERO in my book.

Alright, alright, that's enough. Thanks for listening.

We did rounds, dressing changes, had a liver ultrasound, had an echocardiogram (like a heart ultrasound), stopped and restarted Prisma, all before 2pm! The afternoon was nice and quiet, but things have been busy since 7. It's now almost 11 and things are starting to smooth out. As soon as I finish up with you all ( ;-) ) I will head home. The liver ultrasound came back the same as last week which means the V.O.D. has not spread at all! Combined with bilirubin numbers leveling out right now, these are really good signs for Bella.

We ran into Dr. Wagner tonight as well, and he also said that he is confident that Bella will make a full recovery from all of this. He said, "We're gonna get you back over [to 5D the BMT Unit]!" So, again, if Dr.s Tolar, Somani, and Wagner all feel good about Bella, I guess we should, too. Still, there is a strong feeling inside me to remain VIGILANT. Why? Because they assume that no errors or mistakes will be made along the way to her getting back. I feel like that's where I come in. I feel like the Quality Control Manager around here; like it's my job to make sure people are bringing their "A-Game" into Bella's room. Coach Tim, at Bella's service!

One other thing we are seeing is an adrenal gland insufficiency which is why we had to go on steroids and dopamine. Dr. Wagner wonders if it isn't an EB thing. More research.. wait, ANY research is needed on this as this might be a very important piece of every EB kid's makeup wherever they are. It may explain why EB kids are so small, as opposed to the GI absorption insufficiency theory that is kind of held as gospel right now. If RDEB only affects mouth, upper GI, and peri-anal, why would absorption issues be to blame, particularly if the child has a G-tube? So, more will be revealed on this front. Stay tuned.

Thank you to those of you who promised to contact your local RMH today. Did you follow through? How 'bout the rest of you out there?

What one new action will YOU do to

RAISE THE BAR to

MAKE A DIFFERENCE in

YOUR COMMUNITY?

There are people in your community who need your help. Please answer the call.

Thank you.

Good night.

July 25: Day +23

Daylon's mommy Jennifer got this for Angelique for her b-day... poignant, eh?

Where is the summer going?

Last summer we were holed up in our condo learning how to care for Bella, and this summer we're holed up in a room that maybe measures 10' x 10'. I was just typing the date and I simply couldn't believe it is July 25 already. Are you partaking in and enjoying this summer? Please don't take it for granted. We are doing what we can to catch a whiff of summer, but it's not quite fully available to us, for as long as Bella lies in an ICU, how can we really enjoy summer without her? So, make plans. Do something that you can only do or would only want to do in the summer. We live in Southern California, land of endless summer, and many of those where we live don't understand seasons like those who live up here. I watch people up here genuinely delight in a blue sky, or in a warm day, because it may not be that way tomorrow. For that matter, remember to delight in those you love on a daily basis, because you may not have that chance tomorrow.

Today, a bittersweet moment occurred for us when I opened the door to our apartment, and the little boy who went in for transplant the day before Bella stood in his doorway... home. He was on a day pass and will be discharged tomorrow. It was so nice to see him look so good (except for being totally bald of course), and the look on his mom's face... the relief and joy... it was wonderful to see. She and I ran into each other on many a late night during our two kids' chemo and I remember how tired she was as she was up here alone with her son. To see them back in their RMH apartment meant a victory within his battle, and you could see it on their faces.

What made it awkward was when another neighbor came by right then, and after a sentence to the mom, turned to us to ask us how Bella was doing. Upon hearing that she would be in the ICU for another 2-3 weeks, the space got really heavy, and we kind of retreated back into our room so as to not spoil the moment. Our kids' treatment took very different paths and ended up being almost polar opposite each other, and while we are happy for them, we are sad we didn't have the same luck. It just hammered home how far away it feels before Bella will be sleeping in her pack and play next to mommy's side of our bed. Man, that vision seems far, far away right now.

HOWEVER, having said all that, it was another good day for Bella. It would appear that for the moment, we are moving in the right direction, albeit unbelievably slowly, but hey, we'll take it! We met with the ICU attending doc, Dr. Somani, and he was "quite pleased" with Bella's progress in the past 48 hours. He feels we have come quite a long way this week, and his facial affect was lighter and brighter today than ever before when discussing her. Today was his last day on rotation and I told him how much I was going to miss him. Seriously, I think so highly of him. We also lose our attending resident tomorrow, Chris, who has been on the case since Bella was transfered to the ICU. He has intelligence, confidence, and wisdom far beyond his status as a young doctor, and I must admit that it is going to take one extraordinary resident to fill his shoes tomorrow morning. I didn't get to talk to Dr. Verneris, the BMT attending doc, today, and if he rotates tomorrow as well, I'm gonna to have to lead rounds myself. Needless to say, I will be in early just in case this is the case to make sure by the time everyone walks away from Bella's door, they are FULLY BRIEFED on her status, trajectory, and my role in her care.

Here are the numbers:

Weight: still no accurate weight. The crib is being weird.

I/O (fluid in versus fluid out): -224 mL !!!! YES!!!!! Best to date! The goal is to be -220-240 per day so that at the end of the day, dialysis has not only pulled off everything they've put into Bella over a 24 hour period, but some of the excess fluid she has been retaining. She is still up at least 3,000 mL, so you can see this is going to take WEEKS to get all that fluid off her at this rate. That's okay. Everyone is okay with it taking that long if it has to in order to keep things moving safely.

Creatinine: (protein waste in the blood) .36 !!! Record!! Her Creatinine was .29 at admission!

BUN (Blood urea nitrogen... waste): 11 !!! Ditto here. Bella's BUN at admission was 9.

WBC (white blood cells) : 5.7 inched up 1 tenth from yesterday. We'll take it.

ANC (absolute neutrophil count) : 3.1 inched down from 3.3 yesterday... no cause for alarm.

Platelets (blood clotting agent) : 19 (needs to stay between 20 and 30 or else gets more)

Bilirubin: 2.6 LOVE THAT! Down from 4.3 yesterday!

CSA(toxic immunosuppressant): 500 Bad. Too high. Target is 250 optimum, and 400 at most. Doesn't make sense. We'll be figuring this out tomorrow in rounds.

Bella is receiving formula on a s-l-o-w drip through her j tube. This goes right into her small intestines and keeps them functioning. She is tolerating feeds well so far, and they have been increasing her amounts in little increments. We like this because the more she poops, the more bilirubin she excretes from her body, and this will help in her fight against her V.O.D.

She is still on 2.5 to 5 of dopamine to help keep her blood pressure stable during dialysis, and so far, it seems to be working. They will start to ween her off the hydrocortisone (steroid) tomorrow to see if she can hold her pressure up on her own. We really don't want Bella on a steroid if at all possible.

Dr. Tolar came to visit this evening as well. He was very pleased with Bella's skin. Her skin has improved over the past 48 hours as well. Her wounds/scars on her thighs that were puffy, raised, and purple 2-3 days ago, have receded and are pale pink. When he asked me if I had any questions, I asked him if he thought we'd make it back from where we are. I told him that we knew he warned us it would be "intense," but 6 weeks in the ICU was NOT what ANYONE had in mind when he used that word! He responded by saying that many, even most kids who have been where Bella currently is have made a complete recovery. He said that he or I would never make it back ourselves, but that infants are far, far more resilient than we are. That was good news to hear because Dr. Tolar is eastern Eurpean... stern, precise, and not one to blow steam at all. So, when he is hopeful, I am hopeful. Dr.s Somani and Tolar have impressed me the most in my stay here thus far, and to hear both of them give a positive assessment of Bella today made the pain in my chest and head go away for the remainder of the day! That was a welcome relief!

In other recreational news...

We took Nanny on another retail therapy pilgrimage... this time to "T-1," the very first Target, which has been rebuilt as a Super Target. Man, is it... SUPER! The problem was that all three of us ended up with shopping carts, and stuff you buy at a Target resembles water in that it will fill whatever sized container you give it. 3 carts empty at the start equalled 3 carts FULL at the finish! That one hurt a little, but we needed a whole mess of "stuff", so whattayagonna do?

A monument to suburban shopping history...

So, all in all, a good day for Bella, and a good day for us. We'll take it with much gratitude. Thanks for holding us in prayer these past dark days. It has made all the difference. I know I wouldn't be half as strong at this point without you praying for us and literally cheering us on. I am running out of analogies or metaphors, and I feel like a broken record, but as you keep blessing us, I keep thanking you. It's the mutual admiration circle! Hey, it's working, so please, keep it all coming!

In addition to charging you to maximize your summer and delight in your loved ones, please say a prayer for parents who go to bed tonight fighting similar battles we are here, but who don't have friends, family, or faith pulling for them, who aren't blessed to be in a Ronald McDonald House, who don't have the right insurance to get the treatment their kid needs to stay alive. Man, there are so many that have so much less than we do, and yet are fighting similar or worse battles every day, all over our country. If you live near a children's hospital, plug in. Find out what little thing you could do to help out. If they have a RMH, sign your church group up to cook a healthy meal. I'm not interested in what you already do, either. I am challenging YOU (the person in your chair reading this) to

DO ONE NEW THING to

RAISE THE BAR to

MAKE A DIFFERENCE in

YOUR COMMUNITY.

How will YOU answer the call?

July 24: Day +22

Weekends are nice... aren't they?

On the one hand, they are nice and quiet on the unit as long as things are going stable (Bella). On the other, it's tougher to get all the right minds together should things NOT be stable (Daylon). For those of you that don't know, Daylon has been having seizures due to pressure building on his brain from bleeding due to defibrotide. They started last night, and have continued this evening. They can't operate to empty the blood and relieve the pressure; it's too big an infection risk at this stage. They just have to wade through it and wait it out.

I had just hit PUBLISH POST on last night's post when my cell phone rang. It was midnight, so instantly, my heart jumped in fear. Sure enough, it was Brian, Dayon's daddy, explaining what happened. He needed to get over to the hospital and join Jennifer and he asked me if I could babysit the (4) kids in their room for them. So, I got a crash course on who was sleeping where and what they needed and off he went. I just sat on his couch crushed with sadness and fear.

I fear that we are in some sort of nightmare that just keeps getting worse. Our two families are so close that what happens to one is felt by the other. In addition, the two kids have followed an almost identical trajectory down down down throughout this BMT process, so as Daylon goes, usually so does Bella. Our kids are both in critical condition in the ICU and we are far from home. Something just feels wrong. How did we end up this far off course? BOTH of us?

Brian came home at 3:30 in the morning while I was up wrangling one of the twins (Violet) back to bed... good timing, because I was failing "tucking-Violet-into-bed-with-her-blankie-spread-UNDER-the-big-blankie-first," and she was NOT happy with me! LOL. He was like the Cavalry saving the day!

This story is so interconnected with us because Bella just went on defibrotide, and its main risk is bleeding. What makes this scary for us is that she is also on continuous dialysis, which increases her chance of bleeding, since both are anti-coagulants. So, we have double the risk, and to watch Daylon bleed with essentially half the risk is scary. To be fair, it doesn't tell the whole story; the two are not identical, and have a different subtype of the disease, but to have witnessed so much of their stories coincide so closely, it is pretty hard to not be ten times more scared about defibrotide today than yesterday. Daylon was on day 19 of 21 of defibrotide when he started bleeding internally from it. We are only on day three of 21.

Beyond that piece of the story, here are today's numbers:

Weight: (didn't have one... scale was malfunctioning on Bella's bed today)

I/O's: +330 (Prisma was down for 11 hours from 11pm the night before to 10 am yesterday)

Creatinine: .43 (best yet! YAY!)

BUN: 13 (best yet! YAY!)

WBC: 5.6 (same as yesterday... we'll call it a draw)

ANC: 3.5 (down one tenth... no biggie)

Bilirubin: 4.3 (up .9 from yesterday... booo)

CSA: 421 (high due to being off dialysis for so long)

So, another mixed bag, but hopeful because if CSA and I/O's were up so high due to being off dialysis, why did her creatinine and BUN drop to record lows? Remember, those are measures of waste products in the blood. Could it be a hint that Bella's kidneys were doing a little filtering of their own? Hard to say, especially with the continued absence of pee. Hopefully, Prisma stays up and running through the night so we can have a solid, 24 hour period on it to see what kind of impact it can really have if it would just stay running!

In other respite/recreation news...

Ali had an all-day play date with Logan and Erin's kids, Owen and Ellery. Logan took them to the park in the morning and Ali got to go swimming. Then, they hung out at their house playing together till 9pm tonight! Thanks again Erin and Logan! We are so grateful to have you here.

Mommy and Nanny went to... Mall of America for some retail therapy!

Daddy had a man-date with Logan and Mark at Mark and Lisa's house. They are the good friends of Logan and Erin we met at Logan's BBQ before Bella was admitted. Mark is a whiz of a cook, and a guitarist as well. So, after some amazing chicken and vegetables on the grill, we retired to the basement where Mark let me play his prized Gretsch Tennessee Rose guitar while he laid down the rhythm on a snare. We began with a little Johnny Cash (Folsom Prison Blues), and then I took off into a blues and funk revival from my youth. Then, I dialed in his delay petal and stormed through an 8 minute solo I call "21st Century Mojo" that I haven't played in over a year. I finished with a latin-inspired song I wrote when Bella was born called "Providence." It felt SOOOOO GOOOOD just pouring out my pain and my joy through his beautiful instrument. I was so grateful to be given such an amazing instrument to let loose on; I treated it with due reverence, and allowed it to receive every thought and every feeling I had. It was cathartic, to say the least. I'm gonna leave you with the words to Providence...

Looking at the situation

Through the wrong set of eyes

Wanting someone else's life - not mine

Angry at the cards I drew

Who dealt me this hand?

Trying to hit the UNDO switch

and turn back time

and I'm here to

try to understand

Providence

Providence

Providence

Kneeling down upon my knees

The burden was lifted

He took it with a smile

And with music He said,

"So, you're here to

try to understand?"

Providence

Providence

Providence

So you keep on walking

But you keep on falling

So you keep on trying

But you keep on failing

And you keep on hoping

But you end up swearing

That there's just no way for you

To have just what it is you want

But I'm here to

Help you to understand

That the key is already

In your hands

Providence

Providence

Providence

Providence

You can check out a new photo collage to a scratch track of the song HERE.

Sometimes it's easy to lose sight of the light in the middle of the darkness. If you can remember to find the light within you, you never lose sight of it. However, sometimes, it takes so much to keep that light burning, you can only see a few feet ahead. That's okay. Your headlights may only shine a few hundred feet up the road, but you can drive across the country a hundred feet at a time. And so it is.

Good night.

July 23: Day +21

mommy and daddy in Bella's room...

Another quiet day... mostly...

I arrived this morning at 8 am to the mixed blessing news that the dialysis machine had crashed at 11 pm. This was a mixed blessing because on the one hand, no fluid was being taken off Bella for 9 hours. On the other hand, it gave me the chance to change her line dressings while off the super finicky PrismaFLEX (don't know what the flex is for...). When I had to do her line changes while on the machine two days ago, I felt like McGuyver dismantling a nitroglycerine bomb or some such outlandish thing with some gum, toothpicks, duct tape, and a lighter. Every time I would so much as move Bella's arm, the machine would start alarming... DANGER DANGER - BOMB WILL EXPLODE IN 5 - 4 - 3 - 2 - 1 ...

So, I got my window to change dressings. Problem was that it was during rounds, and Murphy's Law dictates that the moment I had on gloves, a mask, and had exposed the line to the world, I'd hear the knock on the door signaling that the herd had arrived and off they'd go without me. Amazingly, for some reasons they couldn't say, rounds got held up for 2 hours today, so the knock never came. So, I got clean dressings on both her lines. Normally at 8 am, I am not fully alive, but given the anxiety "Rosie-the-Prisma-machine" gave me on Wed, I was ON FIRE this morning maximizing my "mess-with-Bella" window! Who needs coffee when the threat of a big expensive machine crashing due to you hangs over your head?

Prisma got up and running nicely, and Bella was all but off Dopamine in the process! She hovered between 5, 2.5, and 0 Dopamine throughout the day while on the machine, which means the 3-day (today is day 2) Hyrdocortisone therapy she is on is working well enough to maintain blood pressure while on Prisma. This is good for now, but it remains to be seen why her adrenal glands aren't working well enough that she now requires steroids to keep her up and running.

When we finally got to rounds, things actually went fast, which meant there wasn't a lot to discuss regarding any changes to her condition, or changes in the plan for the day. That's a good thing. Here's why...

Here are the numbers:

Weight: 12.7 (down a whole kilo from yesterday! YAY!)

I/O: -152 (the first time we've actually been net negative in fluid volume in FOREVER! YAY!)

Creatinine: .67 (up a little, but no cause for alarm)

BUN: 21 (up a hair, but no cause for alarm)

WBC: 5.6 (up from 3.1 yesterday! YAY!)

ANC: 3.6 (up from 2.8 yesterday! YAY!)

Bilirubin: 3.4 (down from 5.6 yesterday! YAY!)

CSA: 300 (down from 392... target is 250...YAY!)

So... as you can see, on paper things were good this morning! It was a strange feeling having rounds go so smoothly. Each morning, I enter rounds with anxiety for which numbers are good and which are bad because as you've read in prior posts, it's usually a mixed report at best.

At this point it was 11 am, and the major work of the day was already done. Nanny came to relieve me again so I could get out a little. My friend Logan took me to lunch and we sat on a park bench in the shade in the midst of a beautiful sunny day here. Then, I went back to RMH and took another nap; I'm still feeling like there's a bug trying to take hold. I'm on massive amounts of vitamin C and sleep, and that is helping. After my nap, I returned to the hospital so Nanny could go pick up Ali and get dinner together. Angelique brought me dinner and we hung out with Bella till about 9.

Mask time, courtesy of our good friend Sara back home... thanks, Sara!

We really miss Bella.

She feels so far away.

The docs told me today to brace for 2-3 more weeks in the P.I.C.U.

I am tired, and I am sad. I was anxious for this procedure before we got here, but I am scared of where we are. I always thought that because Bella was so healthy going into this, that her organs weren't at risk, and that her life really wasn't at risk. Sitting in my hospital chair being unable to even SEE Bella because she is now completely obscured by machines... my heart is heavy in my chest. I force a tired smile when with family, and Ali genuinely lights me up inside, but I just feel, well, heavy. This too shall pass. I know this. Yet here in America, the land of feel-good-no-matter-what, this feeling, while perfectly appropriate and natural... I feel like I'm supposed to "do something" with it. Most people medicate it, and I usually transform it, but not tonight. It is just too heavy. Tonight, I just go to bed with it, and that's okay, too.

My view from my chair of Bella...

I could rejoice in the numbers today, but numbers aren't Bella, and Bella is far more than numbers. I have been managing the numbers and the skin, but what about the girl? As music therapy professor and author David Aldridge so eloquently says, "Who is treating the SELF inside the patient?" Our vibrant baby girl hasn't moved, made a sound, or opened her eyes in just under 3 weeks (19 days now), it has worn on us, despite what the numbers say.

We read to Bella, talk to her, I play guitar for her, sing to her, kiss her forehead, mommy kisses her hands, and each time I do so, I know she can hear and feel us. After all, her ears are still working and her brain is still processing everything she hears; she is just paralyzed from responding. She is trapped inside herself. She won't remember any of this due to the drugs she is on, and for that I am thankful. We just miss our little Bella. Nothing to be done about it, it just is. I am just so scared to lose Bella. Ugh, it hurts.

Good night.

A photo essay of the machines keeping Bella alive right now...

"Rosie" is the dialysis machine that is pulling the fluid out of Bella slowly and doing her kidneys' jobs for them until they recover...

"Huggy the Bear" (its real name is the 'bear hugger') inflates a heated 'blanket of air' over Bella to help get her temp back up. Having all her blood leave her body and flow through Rosie cools her blood, so Huggy tries to counteract one of Rosie's side-effects.

"The Vent" is the breathing machine that controls Bella's breathing for her while she is paralyzed.

The EKG machine follows Bella's heart rate, blood pressure, central venous pressure, respiratory rate, and oxygen saturation. This is how we "see" how Bella is responding to her many therapies and protocol.

"The Pole" manages all Bella's IV intake. Let's see how many I can remember: Vecuronium, Fentanyl, Versed, Dopamine, TPN, Lipids, Platelets (as needed), Formula, Calcium, CSA. That doesn't include the drugs she gets through her G-Tube...

July 22: Day+20

Stable and Cute... continues!

It was a quiet day for Bella (at long last). We had a little action in the morning with unfortunately yet another new respiratory therapist who was on autopilot. Some day, I feel like we'll have met and worked with everyone on the unit. On that day, I'll either be happy because there will be no more teaching, or sad because we will have been on the unit so long that we had the occasion to meet everyone who works there!

Morning rounds was another case of good news / bad news.

Here are the numbers:

Good:

Weight: 13.7 (DOWN .2!)

I/O: +100 (closest we've come to net zero or fluid loss since who knows when)

Creatinine: .53 (We've finally reached our target of .58! YAY!)

BUN: 16 (best level to date - yay dialysis!)

Bad:

WBC: 3.1 (still falling, but to be expected)

ANC: 2.8 (still falling , but to be expected)

Bilirubin: 5.6 (has doubled in two days)

From BMT point of view, things aren't the greatest with counts, but no signs of GVHD, and wound cultures continue to come back negative for any fungus or bacteria, so, we'll call it a draw?

From GI point of view, Bella was stable enough to start defibrotide today. PLEASE pray that:

a) it works

b) it doesn't give her a stroke

Her right leg changed color overnight to a greenish yellow from the increase in bilirubin and she officially looks jaundiced. What kills me the most is that it is day +20, and I'm pretty sure it was day +12 that we first heard about defibrotide. By day +13, we were ready to add it, and we had to wait 7 LONG days and watch this disease get worse before being allowed to treat it with THE ONLY KNOWN drug that can prevent it from killing her. That is so hard to deal with. It just bites at me heels all day long. I would punch the desk in anger, except I am simply too exhausted to do anything more than type right now.

From nephrology (kidneys) point of view, we are happy in that neither the dialysis machine not her Hickman line are not clogging, and Bella is tolerating continuous dialysis. I think we will finally see some fluid off of her in the next 48 hours should all things remain stable. Nephrology is happy that she is on defibrotide because, as an anticoagulant, it lowers the risk of the machine or line clogging even further... so long as it doesn't give her a stroke. The sad part of nephrology is that her kidney condition was escalated from "acute renal insufficiency" to "renal failure" in rounds this morning. She is totally dependent on dialysis to survive right now. Not gonna lie, it's pretty scary.

The newest tube in Bella's life... a heating blanket to offset dialysis cooling her body temp...

By about 11:30 am, I could tell I was running out of "health points," (like on a video game!), so I called nanny to come in and relieve me since it looked like it would be a quiet rest of the day for Bella. Wound care was done yesterday and doesn't have to be redone till Saturday at the earliest. We redid her headgear completely this morning, so that will be fine for a while now. So, I came back to RMH and promptly climbed into bed for a nap. 3 hours later, my body told me that if I wanted to be at rounds tomorrow morning healthy, I better not plan on leaving this room. So, I listened, and I haven't. I can feel a cold right outside the doorstep, and I am not opening the door to let it in! Nanny watched over Bella during the afternoon, and mommy went over after dinner while nanny and Ali played BINGO at RMH. I'm really grateful to have nanny's love and care around at this time.

Now it's time to get back to bed. Just didn't wanna leave you hangin'...

Story time for Ali!...

Finally, Angelique, Nanny, and I are repeatedly moved and touched by the beauty and generosity of your comments. They're like a V8 for our spirits! Thank you for them, and please keep 'em coming. They help us more than these words can convey.

Good night and God bless you.

July 21: Day +19

Bundled Bella in fresh sheets, dressings, and blankies...

Holy cow.

I need some dramamine... for the sea sickness... from the roller coaster... that is Bella... that is BMT... in the PICU.

Okay, bottom line: we had an otherwise pretty rough day end with a pretty amazing turn of events. Here we go:

Rounds: Here are the numbers:

Weight: 13.9 (highest yet... Bella, no going over 14, it's not very girly)

I/O: +354 (not great but half of the day before)

Creatinine: .95 (WOOOHOOOO! Less than HALF of the day before!)

BUN: 31 (WOOOHOOO! ALSO less than HALF of the day before!)

WBC: 5.6 (another dip... to be expected in this first week off G-CSF)

ANC: 3.6 (again, another dip...might have to get some more G-CSF... not uncommon)

Bilirubin: 3.3 (still moving up even faster... sign of continued liver slowdown)

No pee from Bella for another day in a row. I think it's been at least 3 days, could be 4 since she's peed anything. Again, it's not her bladder; her kidneys aren't producing any urine to go into the bladder... all the fluid just backs up inside Bella.

Can you count the wires and tubes going in and out of Bella? How many?...

The PrismaFLEX (name of continuous dialysis machine) was still running this morning, despite the ominous report from the prior night nurse. That was a good thing. However, overnight, Bella had to go up and up on dopamine. Dopamine is a "presser," which means essentially its job is to raise Bella's blood pressure. We have needed increasing levels of dopamine to maintain an already low bp (like 60's - 70's / 20's - 30's). As the dopamine went up, the blood pressure didn't go up with it, in fact it kept sliding downward.

So, when Christina (BMT fellow) came in this morning... she had that look in her eye, and I knew something was up.

"Because of Bella's hemodynamic instability, we can't begin her defibrotide today..."

Last week, Bella wasn't sick enough to get defibrotide. Today, she was too sick. Talk about a small window to give a drug. I understood the explanation and it made total sense to not compound Bella's blood pressure problem with another anticoagulant that would further thin her blood and lower her pressure. timing IS everything, both for positive AND negative outcomes. So, it will have to wait till hopefully only tomorrow. The good news is that we are still at the early early stage of V.O.D., so burning a day to wait to see if her bp would stabilize was a prudent move.

As the day progressed, the need for dopamine increased. The max dose we could give Bella was 20, and we got to 19. There is a second presser, epinepherine, but using two pressers automatically disqualifies Bella for the defibrotide study, so we were really backing into a corner today. This big question was, "What is behind this DROP in bp?" I asked every doc who came to see us, and told them not to worry about scaring us, but let me in on their thought processes. We had really great conversations, and at one point, while the BMT doc, BMT fellow, and ICU doc were in the room together, I had them all give their 2 cents, and the two docs started brainstorming about what it could be, and it triggered some decisions on preventative meds they could give Bella to head off what they were thinking about... 'just in case.'

I have to tell you what a great job I think the attending ICU doc, BMT doc and fellow, and nephrology doc and fellow are doing working together and brainstorming together on Bella's behalf. Dr. Somani, the ICU attending doc, apologized to me today in the most heartfelt way because we've had such a rough 48 hours. You can tell he is "all in" when he is thinking and discussing her case. He is just so 'thought-FULL.' Thank you for all your prayers blessing the staff; the 3 docs overseeing Bella right now are ON THE CASE.

Anyhow, Dr. Wagner was due over at our room at 3:30 to observe wound care so he could see Bella's skin. It was great to get his eyes on her, but I was petrified to roll her over due to how sensitive the PrismaFLEX was being. I knew that if I rolled her over to see her back, the machine would crap out. Luckily, overall, Bella's skin is doing well, so we can hold off on dressing changes as often right now so as not to disrupt the main treatment: the dialysis machine.

However, the real serendipity occurred when the dialysis machine died on its own just before I was considering whether or not to attempt dressings. So, we were given a couple of hour window to get as much "messing with Bella" done as all of us could do before she went back on dialysis yet again. So, Dr. Wagner looked at Bella's problem areas, and his comment was:

"That's nothing..."

...in turns of BMT complication, Bella's head and back look great by comparison to other kids in the study! That was great news indeed because now we can leave Bella alone for a couple of days of straight continuous dialysis without risking running the machine amuck in the midst of my dressing changes.

So, we got ready to start the new machine yet again, and the most wonderful thing happened!

Bella's blood pressure DOUBLED.

It got up to 120's over 80's! This allowed the nurses to cut Bella's dopamine in HALF, and allowed nephrology to set the machine to actually lower her overall fluids! When we left her tonight, she was STABLE and CUTE!

Bella's fabulous socks of the day!...

I have to go to bed as I am EXHAUSTED by the day's events. Rest assured. YOUR PRAYERS AND THOUGHTS AND INTENTIONS ARE WORKING. Pray tonight that this was no fluke. Pray tonight that this is a corner turned to better things. Pray tonight that tomorrow we can start defibrotide, and that her body can handle it. There was much more, but I can't keep my eyes open a minute longer. I love you all, and we thank you again; your energy is being felt up here!

Before I go, I have to mention that Ang and I got to finally meet Sam and Marybeth Sheridan tonight at RMH when we got back from the marathon day! Sam is patient number 7 in the study, and the first patient we followed online through transplant. WE really connected with them pretty deeply during their transplant, and it was such a treat to finally be hanging out with the two of them face to face. They are a lesson in strength, perseverance, and faith. Marybeth posts updates on Sam's journey at:

http://www.caringbridge.org/visit/samanthasheridan

Instead of "Where's Waldo?"... "Where's Bella?"...

Good night.

July 20: Day +18

Bundled Bella and her Butterfly gift from Ali...

Hello world.

What's it like out there? I live in a fish bowl. It feels like the Bill Murray flick, "Groundhog Day," where he keeps waking up and it is the same day again. Well, it's like that but with an evil twist. Each new day brings a new level of stress and frustration. As they say at the Wellness Community, "What fresh new hell is this?"

Before getting into the details of the day, it is important to remember that this is the time in transplant when the sh*t hits the fan, sorry for the slang. Whatever bad things the chemo does, the effects rain down HARD during this phase (days +7 to +28). We thought that since Bella was as healthy as an ox going into this that organ failure wasn't a main concern. My main concern was what was going to happen to Bella's skin during this phase, and the risk of infection over the next YEAR.

So, on the plus side, Bella's skin is behaving much, MUCH better than I anticipated. I honestly expected her numbers to come in well since she is so young and has a young, 8 out of 8 matched sibling donor. I just felt intuitively that her counts weren't going to be the cross she would have to bear on this journey. To be honest, I hoped that she would simply do well and not get snagged at all by any major side effects or risks. I hoped and prayed she'd be a banner baby; a poster child for a successful transplant story for EB... a source of hope. It's not naive to hope my kid will slide through unscathed; other kids have, why not mine?

One important point to make is that on the one hand, usually, the younger you are, the more chemotherapy levels you can survive. On the other hand, most kids who go through BMT usually go through AFTER They have had an unsuccessful run with chemotherapy, so their immune system is already compromised when they begin their protocol. As such, they do not require the same dosage of chemo to "clear out the existing tenants," as I previously alluded to in an earlier post. With Bella, she was 100% healthy, so they had to use A LOT of chemo on her to clear out her existing marrow and immune system. I didn't really think of it that way until talking with Dr. Wagner after the fact. So, the likelihood of major organ damage really goes up in Bella's case. Didn't really think of it that way, did I mention that?

Sooo, on the negative side, her skin is a moot point if her kidneys and liver don't turn around.

Let me tell you how the day went...

To begin, I had a little "come to Jesus" talk with the herd at rounds. Dr. Verneris got to rounds a little early and I told him how frustrating yesterday was due to everyone changing Bella's plan of care without me. So, at the end of rounds, he wanted to address it on behalf of me, but was unsure how to articulate it. Since I knew EXACTLY what I wanted to say, I 'invited' him to let me take over. This is what I said:

"At this point we are looking at 4 teams caring for Bella: ICU, BMT, surgery, and Nephrology. Well, there is a fifth team: ME. I am her daddy, I am her advocate, and I am up on what is going on with her care as you can tell. I was on the floor all day yesterday, and during the one hour I left for lunch, you all got together and changed her plan of care without consulting me. This CANNOT happen again. You are not to make any decisions on her plan of care going forward without consulting ME FIRST. I have a cell phone; you know how to reach me. You get each other on speaker phone when you need to; I expect the same courtesy going forward. Thank you."

They nodded their heads appropriately and that was that. I was so nervous that I was nauseous ; not because I was afraid, but for how stinkin' hard it was to keep my wits together and deliver that address in the same clinical tone that the rest of rounds were discussed in. I don't want to be labelled an emotional basket case... reason and intelligence are paramount up there (on the unit), and these grant you their attention, and when they are supported by passion and love, the message thereby has the power to get through. I looked around the group as I was talking, and the BMT fellow that used to like Ang more than me was clearly moved by my spiel, and for the rest of the day, we had a new level of partnership that I am happy to now have. I really feel like we are on the same team now, and that makes me very happy.

So, here are the numbers...

Good:

WBC: 8.5 (up from 7.5)

ANC: 5.6 (up from 5.2)

Remember we got rid of that fertilizer drug G-CSF on Sunday? It only took one day for Bella's counts to resume climbing! YAY!

Bad:

CSA: 201 (still too low for comfort)

Creatinine: 2.02 (up from 1.53... target is .58: means we're still in "acute renal insufficiency")

BUN: 70 (.100 is okay, but jumped up from 53... indicates more toxins in Bella's blood)

Bilirubin: 2.4 (up from 1.6... now we are in V.O.D. territory... now need a liver ultrasound)

I/O: +760 mL (total net volume in/out at the end of the day... means dialysis failed yesterday)

P.S. Bella has been anuric (hasn't peed) in over two days. Not because her bladder is backed up, but because her kidneys have stopped producing any.

The first plan of the day was to begin continuous dialysis first thing in the morning. Bella needed blood before dialysis today, so we had to wait for that. Then, the machine needed to prime itself, so we had to wait for that. Then, once we got all the planets to align, there were literally 7 medical staff in the room to oversee the 'initiation' of the new machine. Within about 10 minutes, things looked good on Bella's part. It is quite a balancing act to begin continuous dialysis in such a sick kid. It is a fine tuned concert between nursing who is managing blood pressure medicine to keep Bella's blood pressure safe, and nephrology, who manage the removal, filtering, and replacing of basically ALL of Bella's blood. Bella's blood pressure was holding steady and people were excited...

...then the software in the machine froze, and the machine simply stopped dead in its tracks.

No kidding.

7 medical staff in a tiny ICU room, and NOT ONE of them can override or reboot the machine. It has to be scrapped and a new one brought in.

Do over.

This was 4 hours into the process, mind you. It took 4 hours of coordinating and prepping to launch this new machine, and in 12 minutes, we were back to square one.

"Take TWO!"

Number 2 in action...

So... out goes number 5, and in comes (literally) number 2. We set up in record time and the nephrology team leaves. The dialysis nurse, Grace, (whom I LOVE) works hard to get the new machine ready to go within 1 hour. Now, we just have to page Nephrology because they have to be in the room when it launches.

Ten minutes goes by.

Twenty minutes goes by. Another page.

Thirty minutes goes by. No answer.

Right about at this point, I tell the nurse who is training under our main nurse that Bella's mouth needs to be suctioned every hour. The main nurse is out of the room, so the nurse in training gets on it her self. I am seated on the window ledge, and my view of Bella is obstructed by the giant new dialysis machine. I hear her beginning to suction, but have someone else in my face asking me questions while I am trying to type an email. After a few seconds, I notice how loud the suctioning is, so I lean my head around the machine to see why, and what do I see?

I see the "forbidden sucker" that tore up Daylon's mouth 2 weeks ago going in and out of Bella's mouth like a dagger.

A scream leaves my mouth, riding on the sudden exhale that launched out my lungs. Within a second, I am airborne and yelling to STOP STOP STOP!!! WHAT ARE YOU DOING??????

The poor nurse in training is shaking in her crocs, holding "the scalpel" above Bella's face.

For a little background on this, there is a type of suction device called a "neosucker" that produces 'too much' suction power to be used in an EB mouth or nose. It is the suction device that sucked the skin right out of Daylon's mouth 2 weeks ago, and has now been outlawed on the two kids. It had been sitting in a wrapper in the bottom of the bag that held Bella's "Red Robinson," a foley catheter that is long and soft and is safe for oral and nasal suctioning on Bella. The nurse-in-training, or NIT(WIT), took the red robinson off, pulled the other sucker out of the bottom of the bag, pulled it out of its wrapper, stuck it on the hose and started jabbing away through Bella's mouth guard into her mouth.

Now, you are probably wondering, if it was outlawed, why was it so close to the action? Good question. The answer is that it MAY be used if the red robinson has pulled a clot OUT of the mouth or nose that is still stuck. You can then take the neosucker and touch it to the end of the red robinson to help pull out a thick clot. This may sound a little confusing, but when in the room and explained clearly, it makes sense to the nurses.

At this point in the day, I literally (no lie) start banging my head into the walls and window.

I am D-O-N-E.

The room is now filled with nurses and staff, myself, and my mom, all sitting, standing, and SQUIRMING in the most awkward moment of silence you can imagine. I just turn and look at Bella. Frozen. Helpless. Mute. I barely hold back the tears as I start apologizing to her and talking to her. I page respiratory therapy because we have to change the mouth guard anyway; it was soaked from the night nurse NOT suctioning every hour like she was supposed to. We fixed her head gear and checked for bleeding, and thankfully, there was none. Here's the thing however; when you pull Bella's skin off, it doesn't bleed. When her skin shears off, it still leaves a basement membrane that is above any capillaries. So, I tell the NIT and the staff that it looks like she didn't do any damage, however, I can't see in her mouth to truly determine this.

I quietly pull her trainer aside when she returns to the room and ask her if she had explained the suctioning to her NIT at the beginning of shift. She confesses that while she 'mentioned it,' she didn't emphasize it since things were so chaotic when they came on. Then, she made the fatal mistake of trying to defend the fact that with "all their other kiddos..." they don't have so many precautions. Bad move. So, I have to start training this trainer on how to be PRESENT in the room with the actual patient in front of you, NOT all the patients IN YOUR PAST in order to get out of "autopilot mode."

I am done with training.

By 1 pm, a full 6 hours after Grace began setting up, machine number 2 was up and running. Remember how we paged nephrology at noon? Her pager was turned off. We only found this out when SHE CALLED US to ask why it was taking us so long to prep.

I swear to you I am not making any of this up. I wouldn't - I couldn't - imagine things could go this bad. Grace told me this was the longest set up she'd ever done. Grace is not new at this. Another award I don't want us winning.

We move at the speed of mud in the PICU.

Bella bundled up since dialysis cools her blood and lowers her body temp...

By 4pm, it is time for our liver ultrasound. It goes relatively uneventfully (amazing). Then, at 5pm, the BMT fellow, Christine, who I told you about earlier, walks into the room by herself clutching some "paperwork."

Here we go.

The only other time Christine has entered the room that late in the day was the last time we had an ultrasound done to talk about V.O.D. Remember, we needed a bilirubin of more than 2 to qualify for the drug therapy? Well, the other component they wanted to wait for was to see if the liver got so swollen that it started pushing blood in reverse through its own vessels. Well, the ultrasound saw that today.

The paperwork? The informed consent to participate in (yet another) clinical trial. So, we go over it, and she leaves it with me. Angelique and I read it, talk about it, sign it. The defibrotide begins tomorrow. The benefit? It may safe Bella's liver. the risk? It is an anti-coagulant. No problem, but the continuous dialysis is as well. Two anti-coagulants running simultaneously present a possibly life threatening situation. Bella could essentially stroke from trying to save her liver and kidneys.

I told you, EB is just one big case of choosing between crummy and awful. If she goes into renal failure, she could die. If her V.O.D. spreads to a second or multi-organ, it's 80-90% fatal. To not treat her for fear of a stroke and watch her die of one of these complications, well, that doesn't sound like much fun. She HAS to be on dialysis, and if we let V.O.D. progress, it'll most likely kill her, so we feel, as do all her BMT docs, including Tolar and Wagner, that she NEEDS to be on defibrotide at the same time. We've run out of options. We've been trying to avoid continuous dialysis and defibrotide, but we lost.

So, tomorrow, she begins her first dose. It is a 21 day treatment with no immediate results to hang our hats on. It's more like, "If she's still alive, it's working."

Before we left at 8pm tonight, our night nurse explained that machine number 2 was beginning to fail, and that it won't make it through the night.

Onto machine number 3 in the morning I guess.

You all know that I am a man of deep faith and passion. You have read me work through these issues and arrive at a positive end. After yet another grisly report, you might be left with, "Why are things going so abysmally bad throughout this journey for us?" Only God has the answers, and I frankly don't need them. Yes, things are unbelievably stressful right now, but we were told this was how it was going to go. The details of what pitfalls we might find ourselves in were unclear, but it was LAID out in front of us that this was no easy task, and that peril lurked (and still does in all sorts of forms not yet discussed) on all sides.

Don't get me wrong. (pardon my upcoming French...) It TOTALLY SUCKS that this is where Bella is. We knew this was going to probably be a bad period, but again, for different reasons.

Here's also what I know. I have personally treated patients in FAAAAAR worse shape, who were far closer to death than Bella, and met them after they made a complete recovery. This too shall pass. I have no evidence that this is the case at this point, and so I am relieved that I can fall asleep tonight lying in the care of my faith.

One day, Bella WILL look back on all this and say, "Yeah, I was born with this rare skin condition, but when they brought me home from the hospital, it went away."

I believe this. I believe this because I choose to believe this. Belief and faith are a choice. More than that, they are a gift. They are a gift you can give yourself, because only you get to choose what you believe in and what you have faith in. If you have no faith, you chose that. If you do not believe in anything, you chose that. If you believe in God, you chose that. If you believe in no God, you chose that. No one chose it for you. Your heart alone is the final vote in accepting what comes from without, and from within. My heart POUNDS for Bella. My heart POUNDS at the thought of dancing with her on her Wedding day. All that I am, all that I say, all that I do, all that I write POUNDS in dedication to Bella, and in dogged alignment with the vision I will once more recreate:

One day, Bella will look back and say, "Yeah, I was born with this rare skin condition, but when they brought me home from the hospital it went away."

Life is good.

See you tomorrow.

Home being goofy with Ali... who took this shot...

Ali scolding nanny telling her she was talking to me... SASSY!

Ali called out the following commands:

"Cheese!"

"Sausage!"

"Pepperoni!"

"Wine bottle princesses!"

Awesome. I love you, Ali.