October 7: Day +97

Heaveno!

RING RING! RING RING!

7:03 am. Nope, that wasn't my alarm clock, that was the room phone. You know, the room number that ONLY the PICU staff has - "The Bella Phone."

"Bella Hotline. Tim speaking." Just kidding.

"This is Tim," my more professional, "Give me the facts," answer.

"Hi Tim, it's (Bella's night nurse). I just wanted to let you know that Bella started getting really acidotic over night and we had to put her back on the vent and turn her rate up to 24. Just wanted to let you know so you wouldn't be shocked when you came in."

"Thanks."

I wanted to say, "So, shock me out of bed instead," but I had just been shocked out of bed, and my mouth didn't work so good. Probably for the best. Remember, phone calls on the Bella phone are never good, so I am always scared to answer. What a start to the day.

And it didn't get any better from there. Take a deep breath, here we go!

First, I walked in, and Bella was covered in extra layers and even a blanket over her hat. Hmmm... the nurse indicated she was about to send off cultures because her temp got too low. I scowled, knowing how long Bella was uncovered yesterday, thinking that it was just because we had her out from under her covers for too long.

Nope. Not quite. Her temp was 92 over night and the warmest we got it was 95.9. It was 93, 94.5 (even with the bear hugger heated air blanket back on). Her chest x-ray also showed a significant change in what looks like an infection brewing, as well as some atelectasis (collapsing) in the right upper lobe. Could spell an infection. Her blood gas pH over night was 7.05. Folks, that's really, REALLY acidotic. That temp is really REALLY low. The two KEY indicators for enzyme health and function in our bodies are... yup... pH, and temp.

So we had to crank up the vent to 28 (practically square one) to get her blood gases to appear normal again, and we had to crank up the bear hugger for the first time in a week to try to get her temp back up. The problem with the bear hugger is that it makes Bella's blood pressure TANK. At one point when we turned it on, her blood pressure was 40s over 30s. The nurse frowned at the monitor, and I asked her, "Which would you rather frown over right now, blood pressure, or temp?" We were fighting two battles at the same time, and to help one was to hurt the other. Not good.

Can you spot the good news inside that paragraph? We actually could read her blood pressure, thanks to the new A-Line we got today! That thankfully went very well. Unfortunately, we couldn't get it in till about 1:30, so we were flying blind with blood pressure from 5pm yesterday; we can't get cuff pressures off Bella to save our lives. Thank GOD for the A-line. We would be so screwed without it.

I found out something that anyone involved with EB care should pay attention to:

DO NOT INJECT lidocaine to pre-medicate a site you need a suture or stitch in. It produces a blister, and compromises the integrity of the skin. That's what happened with the A-line yesterday. The doc that came in today, Dr. Sidell, explained this, and the whole debacle made perfect sense. If you infuse lidocaine under the skin in an EB kid, it will just fill in the layer missing anchoring fibrils and just separate the skin into an instant fluid pocket/blister. The reason the stitches tore out so easily yesterday is that the surgeon who put them in used lidocaine first. So, try a different pain management approach... either a PRN or even topical lidocaine maybe.

Anyhow, we have a new A-line that works great. We also have a new BMT doc. Good guy. However, we had a pretty sobering conversation about Bella today, just like on day one with Dr. D. He was quite a bit more tactful about it at least. The BMT docs are pretty worried about Bella right now. Her liver is getting worse, and she is starting to have multiple organ failure. No kidneys, a compromised heart, weakening liver, lungs were good up till yesterday.

The one big area that he and I discussed today is Bella's brain. The one chilling thing that I've noticed lately is that we have weaned a TON of sedation off Bella. A TON. We are at .2 of precedex, and we are not seeing any more alertness or wakefulness in Bella. The ICU team has been conspicuously silent about this, and when I brought it up with my nurse today, she had a very unique look on her face; one that I hadn't seen before in the months we've worked together. It was saying, "We haven't wanted to say anything to you, Tim, but... she should have awakened by now."

I told her it isn't lost on me; the ICU team says more by what they don't say, then by what they do say. This morning in rounds, no one would look me in the eye or say good morning to me. There was a very somber feeling in the air. They were clearly uncomfortable with this change in events and when I went back into the room, I saw them immediately start talking amongst each other fervently about Bella through the window. It was as if they were waiting for me to leave before they said the things that were really on their minds.

Anyhow, let me finish up on the day. After we got the A-line back in, we had a heart echo done to measure how her dynamic outflow obstruction (LVOT) and mitral regurgitation were doing.

Then, it was on to changing her ulcer dressings and her trach. We change out her trach on Thursdays. We also had to redress two of her lines and a couple of other bandages due to her bleeding out so much from yesterday's heparin and platelet fiasco. Turns out the renal doc gave Bella a heparin bolus yesterday instead of just increasing her rate after getting a low heparin count once we went back on the circuit. This sent her heparin (blood thinner) reading through the roof and it took several hours for her body to metabolize it. Combined with a new lower platelet setting to offset any further clotting, and we had the perfect storm for wounds to leeeeeeeak. Amazing how much blood can leak out of your body when your ability to clot is AT ALL compromised. Amazing.

Ali visiting her little sis...

The only part of the day I enjoyed? Reading your comments. When I hold her hand while I read to her, I feel the energy of your words flow through my hand into her little body. Tonight, she didn't squeeze back. Folks, she is pretty sick right now, and needs all the prayers and intentions, thoughts, wishes she can get. I promise that I send every ounce of energy into every word, and yes, even accents when asked for! That part is fun! I try to imagine what all your voices sound like as I read your words. I feel so close to those of you that open up and really talk to Bella. I am just so touched that you take the time to write to her and share with her your most heart-felt encouragements, thoughts, prayers, and wishes. They are so wonderful to read; they are medicine for Bella and for me while I read them! Please, please, please, if you haven't commented before, please break out of the silence and into Bella's hospital room.

P.S. We held off on the new drug. Dr. Wagner, her chief doc here, just isn't convinced it will do anything, but it's not off the table. We are also talking about some other out of the box ideas, but I'll save those until they actually become plans.

The docs are using phrases like "our backs are against the walls," and "[if she doesn't make it], at least you know you did everything you could." Folks, I'm getting groomed by the docs, and by more and more of them. I am really scared for Bella right now, I'm not gonna lie. She is w-i-p-e-d out right now, and very, very sick. Her heart rate never climbed above 120 today, which is low for her, especially during wake states, which there was little to none of today. I am just so sad right now.

However, we all know what a roller coaster it is here, and how much goes on in 24 hours. A day from now, things could be entirely different. They were today! Thank you all for your unwavering support. Lift us up now more than ever, I humbly ask you. We are tired, and we are scared. I know God holds us in his hand through this, and despite everything happening, I find little things to marvel and rejoice in every. single. day.

For example, have you ever watched a squirrel run up a tree, then stop and turn around and just "stand" upside down, facing down? How do they do that? I saw a squirrel do that on a building here. I love those little pests!

I heard from one of my closest friends in high school whom I lost touch with after graduation today for this first time since 1990.

Ali has these awesome "Twinkle Toes" sketchers sneakers that light up when she walks. On the walk home through the park, she lit up the park like a dance floor with every step she took. Man, that was cool!

Yesterday, Judy, the RT Educator who made Bella's amazing swim cap, dropped in with winter clothes for Ali so we wouldn't have to buy a bunch of stuff we'd never use back home!

One of my FAVORITE guitarists, Wayne Jones, wrote me on facebook today after I requested to friend him. I told him that I use his music both with Bella and with many clients/ groups in music therapy. He wrote and asked which CDs I had, because he'd like to send me some more!

I had YUMMY hot turkey, mashed potatoes, gravy and killer veggies in the cafeteria. Killer comfort food for a beautiful fall day!

My belt buckle fit at the tightest rung today! Don't know HOW that's possible...

I thought I really injured my shoulder jumping in the leaves on Sunday. Today, it hardly bothered me!

I read to Bella from you all at least a dozen times, "One day Bella, you 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."

God night.

Ali hangin' with the car show at RMH tonight...

Getting close!