July 3: Day +1

Yes, that is Bella. The fluid influx for transplantation has rendered her, well, squishy.

Well, we're into the positive numbers now! It's quite surreal that it's our turn. We have read the ups and downs of 5 other EB kids that have preceded us in this experimental clinical trial, and now it really is our turn.

The good news?we picked our primary nurse today. Her name is Theresa and she is just the best. On the BMT unit, you can select a primary team of nurses that you like that the charge nurse will try to schedule just for you so you have continuity of care across shifts and rotations. This is a great concept. The very absence of this (ab-sense) was evident at CHOC when we would cycle through a different ICU nurse every day and have to start from scratch educating the new staff on all the things they had to do differently for an EB kid. Not the case here at U of M.

Theresa was our first day shift nurse, and during her first rotation was nice, but kind of quiet. Well, when her rotation came back and we got her a second time, we had been through quite a few other nurses in between, and no disrespect to any of the other nurses, but we didn't realize how good Theresa was until we got her back. It's like going first on American Idol. you're doomed because there's no one to compare you to! Anyhow, she has emerged as the biggest advocate for Bella, especially over the last 48 hours. She and I have taken on residents, fellows, and surgeons over various elements of Bella's care and she has gone toe to toe on behalf of Bella. In addition, she has a great sense of humor that is quick and sharp, but understated, so she always gets you when you're not ready for it. This element is CRUCIAL for me as I rib and jest with every single person I like to work with including Dr. Tolar.

Second piece of good news. V-e-r-s-e-d. I know Brett if you are reading this you are cracking up since you first planted the seed with me on the phone and again on your site. Let me extoll to you all the virtues of versed. Versed is a sedative most commonly given prior to administering anesthesia or or a surgical procedure. In other words, it knocks a body OUT. It is fast acting, has low toxicity, and causes loss of memory associated with the event it is sedating. That is some good.... stuff. Bella has been itching like crazy, has more saliva than a cotton candy machine or taffy machine coming out of her mouth, is swollen like a grape, and overall is in a pretty miserable state. We were trying to figure out how to do her dressing changes today. She can't lie on her back, otherwise she gags on all the fluid in her mouth and throat. The docs ordered some morphine... no effect at all. She's pretty much maxed out on ativan and benedryl and still combative as all get up. So, Theresa asks me, "Have you guys tried versed?" Then, Brett's (EB BMT alumnus of patient #8) words rang in my ear about how well it helped with his little Rafi. So, I reply, "OOH OOH OOH! Yes! Send it!" I got so excited...

...until Theresa returns to say the docs wanna give her more morphine instead.

Here we go again.

I promptly lay out the evidence that the morphine is completely ineffective, and she says, "I'll just send them in to talk to you directly about it."

Round 3, here we go. You may recall rounds 1 and 2 yesterday about the bumex to help Bella pee. (p.s. Dr. Tolar told the staff in rounds in front of me and the two gals that were fighting me yesterday that "It was the RIGHT decision (to switch to bumex)." That was some gooood satisfaction to start the day.)

A few minutes later, Theresa walks back in, and I ask her, "Where are the troops?" She looks at me and says,"They said (she shrugs her shoulders and looks down like a puppy in trouble), we'll give her versed." I guess they were still licking their wounds from the last round, and all Theresa had to say was, "Mr. Ringgold would like a word with you..."

Don't get me wrong; I am really cool up front with every staff member I meet, but when crossed, I waste NO TIME in laying down the law that:

1) I know more about my daughter than you

2) I probably know more about EB than you

3) I have no problem confronting any decision you make

We now have versed scheduled for all future dressing changes, baby.

Onto the less than good news.

One of the expectations Dr. Tolar set with us the morning we met him was that there will be unexpected situations and complications along this road, and that it will all be intense. He did not bat an eye at this, nor sugar coat his words. I respect that direct communication, because it assumes a level of respect in that he thinks we can handle it, and in fact expects us to. ( I tell you I love this guy more each and every day I work with him). So, if you are to "expect the unexpected," then really nothing is unexpected. This mindset helped us deal with the ongoing issue of Bella's Hickman line. Remember, this is the main IV line that goes right into the entrance of Bella's heart. This is the freeway that all of Bella's chemotherapy, pain meds, and transfusion as well as blood for labs travels in and out of. We have had problems with it since the day it was placed. Fluids go in, but blood doesn't come out well. In addition, it appears that her staph aureus infection is in fact in the Hickman itself. So, we have been putting alcohol in the lines every night trying to kill the bug in the line as well as IV antibiotics to kill the bug in her body, but so far to no avail. Yesterday, Dr. Tolar scheduled surgery for Tuesday to take it out if the infection hadn't cleared by then. Well, today, during Bella's thrashing at one point, she yanked it out of her chest about a half an inch. What really had to hurt is the fact that it is sutured in place! Ouch! Despite the sutures, it pulled out somewhat. Then, we noticed dried blood in one of the lines, and when we went to flush the line, blood started leaking out of Bella's body from around the lumen itself. That was a bit weird to see.

So, now the dumb thing has a laundry list of problems with it, Bella is targeting it for withdrawal herself, and it's the middle of a holiday weekend. Solid. Tuesday seemed a LONG way away for correcting this problem. So, once again, Dr. Tolar to the rescue. I left the hospital this afternoon after bandage changes for some much needed respite, and I got the call from Ang that they are going to do the surgery tomorrow morning. Dr. Tolar came in and met with Ang and asked her what concerns she had and she said, "Are we going to have anyone in the O.R. on a holiday weekend that knows anything about EB precautions?" Dr. Tolar explained that those people are on call and will be there, and Dr. Acton, the surgeon who placed the line the first time will be coming in to do the surgery himself. You can't help but feel good when the guys are willing to come in on Sunday morning, July 4th, to operate on your kid.

So, while the Hickman problems of today were the "bad news," it will spell good news going forward as it will allow the nursing staff to draw blood on the first try, and get rid of the source of the infection. The real bad news tomorrow is that they will have to intubate (stick a breathing tube) down Bella's throat for the procedure. They did the first line by blowing air across her face to avoid intubation (intubating an EB kid is highly dangerous as the tube itself can cause blistering, tearing, and scarring in the throat), but because of her mucositis, her breathing isn't as reliable as they'd like for surgery. Ironically, the mucositis also causes the walls of the throat to swell, so the risks go up even more so than for the last surgery, but this time they HAVE to. So, PLEASE direct your prayers that the breathing tube leaves Bella's body completely unharmed tomorrow morning.

This is how I get to spend my R&R... catch up on laundry!

In other news, Ali and I had Punch Pizza and DQ for dessert, followed by a popcorn movie night in our bedroom. It was good for us to bond again. I could feel her not being as close recently due to my continued absence. By the end of the night, the intimacy was BACK, and BOY it felt GREAT. I just love that little girl so much, despite her drama, and she loves me back despite mine! :)

Popcorn and a little "Go Diego, Go!" DVD... sublime...

Last but not least, we have a new FEATURED PARTNER for the month of July! Please check out the FEATURED PARTNER page to read about the great things Music For a Cure is doing in Southern California. Among other things, they help fund the music therapy program at CHOC. During the month of July, up to 50% of proceeds from Bella's book, Bella's Blessings: a Humble Story of Providence will be donated to Music For a Cure. So, if you have not yet purchased a copy, please help support us and Music for a Cure by doing so. Thanks!