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.