August 31: Day +60

First off, thanks for the happy birthday wishes. I really appreciate it.

Out of the three things I hoped we would accomplish with Bella today, two were achieved. You can't really ask for more than two changes in a day around here... it's a tall order, so I'll take it.

First of all, my day started at 12:15 am when the room phone rang. By the second ring, my body was already in the air heading toward the phone as the blood in my body started to immediately freeze and my stomach squeezed into a knot. It was at 12:30 am the night Bella was born that I got the call in mommy's recovery room that Bella had coded. So, I have a little PTSD from after midnight calls from nursing. Last night, I could hear myself answer, and the person on the other end of the phone started with, "Mr. Ringgold?"

I hate when people call me that. That's what the resident said the night Bella was born.

The night nurse quickly began to relay the events of the prior hour and a half. Yesterday, you may recall, we began Bella on phenylephrine in an effort to transition her away from the norepi. The thinking was that the high dose of norepi Bella was on was causing her dynamic outflow obstruction to return. Norepi squeezes the heart and the vasculature, whereas phenylephrine just squeezes the vasculature, but doesn't get dialyzed like the other pure presser, vasopressin, did.

Well, the trick was going to be how to roll into the phenyl and out of the norepi smoothly.

It didn't go smoothly.

The docs went up too high on the phenyl without pulling of the norepi fast enough, and it basically froze her extremities. They stopped getting cuff blood pressure readings altogether, her hands and legs turned blue and cold, and the blood pressures they were getting weren't good. So, they pulled way back on the phenyl, and added nitric oxide gas to help her breathe better. Apparently that started to go south at the same time. The attending doc (not resident or fellow) spent 90 minutes in Bella's room trying to get a blood pressure with little success.

Meanwhile, as the nurse is rattling off all this information, he hasn't said, "Bella's fine now" yet, so I start to get nauseous and I could hear the body I was in saying, "okay," and "m-hm" at the proper rate, but the voice inside me was saying much louder, "oh God, oh God, oh God, oh God..." Finally, I could feel my stomach preparing to empty, so I got the nurse off the phone and ran to the bathroom. Luckily, no emptying took place (that's just the worst....), but I was dizzy and shaking from the story nonetheless.

Not a good start to the old birthday.

Needless to say, Angelique and I couldn't sleep a wink after that.

When I walked in to Bella's room this morning, a new machine greeted me. It has nitric oxide funneling into Bella's ventilator to help her breathe better, and her blood pressure was still in the toilet. She was getting yet more fluid to prop up her blood volume temporarily when I walked in. Same old same old.

So onto rounds. At least in rounds we decided to get an A line put in today, and we decided to try a new dose and type of steroids to try to actually treat the inflammation in her blood vessels, rather than just replace what she should be making on her own to handle the stress of the situation. The third item I was pushing for but didn't get? To finish getting off the norepi and flying just on phenyl for one day to at least try it. We never made it, and the doc was ready to scrap it. Unfortunately, that would still leave us with low pressures and the dynamic outflow obstruction. Too much phenyl however combined with the norepi, caused poor perfusion in her extremities. It also caused "mitral regurgitation" which is when the vasculature being too tight causes the blood to push backwards from the left ventricle back into the left atrium. Don't want that, either. The hope is that once off norepi, both problems go away, but we have to walk a razor's edge to get there, and quite frankly (and rightfully so I freely admit) no one wanted to do it until the A-line was in place. Unfortunately, we didn't get that in place to roughly 5pm, too late in the day to retry "titration (transition)."

So, the A line is in (and looks mighty spiffy - who dressed that puppy? :)), and we began the new dose of the new steroid. We'll hold off on the titration till tomorrow, and they'll no doubt give yet more fluid overnight (they do every night... they wait till I leave because they know the pain they would endure trying to do it while I'm there... just kidding), then in the morning they'll say in one sentence that they ran the Prisma net zero, but she'll be several hundred mLs of fluid up on the day. At least that is how just about every other day in the past two+ weeks has gone. Let's hope I'm wrong! This is one of those times I'D LOVE TO be wrong!

In other news, we were on our way out the door for pizza at my favorite spot when the entire staff at RMH was setting up for a Luau in the front room. It had been drizzling, so they moved it indoors, and right in the line of sight of one Ali Ringgold. Needless to say, the grass skirts, hula hoops, lights, and music were too much for a girl to resist. We never made it to pizza, but Ali had A BLAST at the luau, and watching her that happy, well, that was a better gift than pizza. Those of you that know me well know that's a big deal, as I am admittedly a certified pizza-holic, but you know, that parent gene... delighting more in your kids than in your self... this isn't the first time I've let Ali pick how my birthday went. It took me a few minutes to realize I wasn't getting outta RMH, and that the cold sloppy Joes were going to have to do. I was slow to come around, but in the end, we had a lot of fun.

Then, it was off to visit Bella and share some of the Aloha spirit with her!

P.S. Don't give 4 year olds cake at 10 pm, even if it's a sliver. It is now 11:44 and Ali is totally out of control screaming and crying like a maniac, of course claiming that's she's not tired. Epic ending to a long day.

P.P.S. The cake in question? Baked by Ali and mommy with lots of love! It was GREAT!

Good night.

August 30: Day +59

The three loves of my life...

Oh Bella... you're such a mystery.

Last night, we went to bed with a night time call-in report to hear that her pressures were "superb." Awesome! We went to sleep content. Once again, we thought we had the puzzle at least solved for the moment. Bella was hopped up on all-you-can-eat portions of steroids and vasoconstrictors. Pressures would surely hold now, and we could get back to getting all that extra fluid off...

I walked in this morning and looked at the monitor, and just like Groundhog Day, her pressures were down in the toilet... again. Chris, one of Bella's primary day nurses, just looked at me totally crushed. My heart started pacing. How could this BE? Bella was on double the highest dose of norepi she'd ever been on, and almost 3 times as much steroids. After less than one day, Bella just couldn't hold her blood pressure, despite the megadoses.

I stood in rounds with my shoulders slumped. I felt like the spine had been pulled out of me. What was left? When you have low blood pressure (hypotension), it's either the pump (the heart) or the tubes (vessels). You can give fluid to fill the pump, or squeeze the tubes to act a little smaller and hope that either or the combo will increase the internal pressure within the system. The problem is that Bella seems to have leaky tubes, so no matter how how much fluid you pump in or how hard you squeeze, the fluid leaks out, and the pressure drops.

I thought we'd played all our hands. We gave fluid, we gave steroids, we gave pressers. Nothing is holding. I asked the new attending doc today if there was a drug to fix capillary leak, and he told me that if he knew how to do that, he'd be a very rich man.

I told him someone needs to make "fix-a-flat" for capillaries.

Now THAT is a million dollar idea!

Anyhow, we ordered yet another echocardiogram, and the dynamic outflow obstruction has returned. We're hoping it's from the whopping dose of norepi, and we are replacing the norepi with yet another pressor called phenylephrine, which structurally looks like norepi, but behaves like vasopressin. What that means is that it shouldn't get dialyzed by Prisma, and it won't squeeze the heart... just the vessels. We are in the process of switching over right now. It should take about 24-48 hours. The hope is that it will relieve the heart from pumping so hard, which in turn will lower the outflow obstruction. Who knows.

There are two other theories / plays on the table, but I'm too tired to get in to them any further right now. If they keep getting talked about, I'll bring them in to the conversation. Otherwise, I won't worry you. Just pray the phenylephrine for some reason does the trick for us. We really need a miracle. It would be the best birthday present I could get tomorrow just to walk in in the morning and see strong pressures. That's all I'd need (aside from the chocolate cake I really really hope Ali and mommy are making for me... :P)

We will probably revisit the A line tomorrow again as we couldn't get good readings all day and night today despite moving her cuff from both arms to right leg over and over again.

We also had Bella's day +60 biopsies and blister tests done today.

Dr. Tolar lining up their blister machine.

Need sleep. Mentally wiped from the day.

P.S. Found out Ali is a carrier for EB today as well.

Not a banner day.

Bella's fabulous socks of the day...

Ali pinching Bella's tootsies...

August 29: Day +58

Happy Birthday today to my daddy in heaven. He would have been 82 today. He passed away in 1997, and while I know that we will see each other again, I miss him.

There was a lot of missing going around today. Ali cried all the way home from Target because she misses Lucy, our pet dog, who is staying with grandma and grandpa back in CA. Angelique and I miss Bella, and we really noticed yesterday and today... not like on any other day it isn't palpable, but this weekend just really hit us. I think it's because the three of us went out into public to do stuff, and we just noticed Bella's absence the whole time. Tonight at Target, it was really evident. Just before we came up here to MN, we had turned Bella's car seat around to face front. It was really cute seeing Ali and Bella in the back seat together whenever we went anywhere. Well, that car seat has been empty, and a catch-all for magazines, Ali's toys, etc. for faaaaaar too long. The empty car seat was what tore me up when Angelique was discharged from the mother baby unit last June. That image hit me this weekend again. The other empty item that hurts to see is Bella's crib. It's been empty for so long now, I hardly remember her sleeping in it that first week here. Man, I miss that little girl. I can barely stand it.

The good news is that we started giving Bella the max dose of norepi and hydrocortisone and her blood pressure finally stabilized! We were also able to finally D/C the vasopressin, an antibiotic, and an antifungal. 3 less meds! Yay! We also figured out that we will run Bella on the Prisma at -10 mL an hour for 12 hours at a time, then run it at even/net zero for 12, then back again to -10 for 12, etc. We realized that Bella doesn't replenish the fluid from her tissues back into her blood stream at 10 mL an hour. So, if we continue to run her at that deficit for a couple of days in a row, even though she is still fluid up, her vasculature ends up too dry. Every day, we learn a little more about finding the balance for Bella while her body works on healing.

Ali's artwork adorns the walls of Bella's room...

It is an exhausting game of titration. While we really are giving it our best, I can't help but feel like we're failing her. For example, her weight is 14 kilograms today. She was 10 kilograms 2 weeks ago. Folks, that is just under 9 POUNDS of fluid that we have put on her in two weeks. That's no fun. We are now treating this ulcer on her head daily as well, and she sure didn't come over with that either. She has 5 open wounds currently on her from staff shearing her skin off with fingertips wearing dry gloves (three are underneath those two squares on Bella's scalp above). She has had over 10 since being in the ICU. Each wound is between the size of a dime and a quarter. I don't know about you, but if I tripped and skinned my knee and sheared off that same amount, I'd notice. I think the worst part of it is that of the 10+ wounds she's been given by staff, NO ONE has copped to a single one and filled out an incidence report. The only nurse with enough class to call this out has been Chris, her current daytime nurse. She is now entering reports for every wound Bella is given. At first, I was a little hesitant, after all, it's EB; sooner or later, we've all left our mark on Bella. However, staff has to be held accountable for whenever they injure a patient, no matter what the patient's diagnosis is, and regardless of intent. I would assert that over 95% percent of med errors and injuries are not malicious, but we must be vigilant to give the best care we possibly can at all times. Patients and their families count on this. After all, wouldn't you if you were the patient?

Sorry for the doom and gloom; this journey is not a straight line, it is not a paved highway, and it is not a glass-covered pond. It is roundabout; we are clearing the brush as we go, and it feels more like we are adrift in a big emotional ocean, where the waves lift us up, then crash down on us. Remember that life preserver analogy from a few days back? That is what keeps us afloat.

It's interesting; my faith doesn't always make me feel better. It is often far more practical. It helps my brain continue to function. My faith gives me just enough peace to fall asleep at night, and just enough strength to get out of bed each morning. I am grateful that the last place I am before bed is on my knees before God, and the first place I am in the morning is right back down on them again.

This journey has literally brought me to my knees! Having said that, I have to say that dropping to my knees has been a powerful experience for me, especially since I'm not a 10 year old with elastic legs anymore. Just the physical act of getting on my knees... it is such an iconic position of surrender. I don't care if you are doing it to pray to God, Allah, or anyone; just try dropping to your knees and remaining there for 60 seconds. The submission of ego and pride to the totality of a world that is beyond my control is actually very liberating. I can give up trying to control and carry the weight of the world on my shoulders. I can GIVE UP ( pay attention to those two words... ) my troubles, my fears, my concerns, my pain. Where can I give it? UP. To God. Give - up. May you never quite hear or read that phrase the same way again.

Will he take it? He always does. Imagine my fears, worries, etc, are in a big backpack on my back. Imagine God walking up behind me, and when I drop to my knees, He grabs ahold of the pack, holding it in place, essentially lifting it off my back. It remains suspended behind me, and he places it over to the side, ready for me to grab and throw back on should I choose to. See, it's always my choice what I do with my backpack. I can give it up, or I can take it up. Being the imperfect human I am, I do a mixture of both. God's always there to take it, and it's always there for me to take back. Either way, I always have a choice.

"Visit the Doctor," By Ali...

Tomorrow morning: more biopsies. Speaking of biopsies, Ali played clinic nurse today and had me (the doctor) give her, me, Angelique, and the baby doll biopsies. Ali wrote out a schedule (see below) and drew how many biopsies each person was supposed to get and at what time their appointment was. The scribbly lines represent our names... Start saving for med school, right?

If you don't chart it, it didn't happen!

August 28: Day +57

I just had to share this... this was in the hospital pharmacy! After all, that's where I always go for a bike helmet and some antacids... not a bad price, either!

The puzzle continues...

Oy vay, folks, oy vay. I walked into the hospital this morning fairly confident that today would be a quick check in, rounds, then off to fun with the family at the falls.

WRONG...

I walked in and Bella's nurse, Chris, just looked at me. She did NOT have a happy look on her face. I walked in and saw the monitor, and understood why: blood pressure once again in the toilet. So, up up up we went on the norepi. No dice. We struggled with soft pulses on and off, which led to the blood pressure monitor not being able to read a bp on and off for a couple of hours at a time. Sam, her attending ICU doc, tried to place an A line today. No dice. After three tries, he just couldn't get her artery to hold still. So, no clear view of the bp.

After he left, Chris and I got to talking again. Remember, yesterday, Chris and I deduced the vasopressin's ineffectiveness. So, today, we got to it again. I realized that we were do to wean the hydrocortisone again. I told Chris that as much as I want to get the steroid out of the equation, I worried that Bella's adrenal insufficiency is real, and isn't gonna let us wean any further. Just then, Sam walked in.

He said, "We need to have a pow-wow. Do you have a minute?"

That's really never a good start to a conversation, so I prepared for what was coming. So, he spelled out Bella's problems. Then, he copped to the fact the the adrenal insufficiency might be the culprit, and that we need to go back up on the hydrocortisone. Thursday in rounds, he refuted my adrenal insufficiency theory flat out, but later that day I told him I didn't buy his argument for why not. That's what I really like about working with Sam. We agree/disagree/banter all with the same comfort level and hash out everything on the table without so much as a blip in heart rate going up for either of us. It's a good partnership. We're also pretty much the same age and have young kids, and so I think we just 'identify' with the other guy. Anyhow, I really appreciated him being willing to come around on this, because unfortunately, if you have adrenal insufficiency, no amount of vasopressor or IV fluid infusion is going to restore blood pressure. Hydrocortisone is the treatment, like it or not. Anyhow, it turns out that we are on the same page again today about the hydrocortisone as we were about the norepi yesterday.

So, new plan, yet again...

Now we are ramping up the norepi and hydrocortisone together in tandem and will use as much of the combo as we need to get her pressure back. Good news? We got her pressure back. Bad news? Her heart rate is now in the 160-180s. It's like whack-a-mole in Bella's room, folks. Anyhow, I had them go up on her precedex to help calm her down and lower her heart rate, and at least now we will be able to pull off some of that fluid...

... because on top of everything else, Prisma crashed again today. This is the second Saturday in a row this has happened. This is always a drag, because inevitably, Bella ends up with excess fluid on board by the end of the day due to the hours between when the machine goes down and when they can get a new circuit set up and running. It usually takes 4-5 hours to get back up, sometimes less if all the planets align.

All the while...

We got away for a few hours today to return to Minnehaha Falls with Ali. We KNEW she would have a blast there, so we put on our bathing suits and set off for some fun in the water!

Well, we had a blast, but the real treat was in meeting another family there who are from here. They have a boy, Collin, who is 6 months younger than Ali, and a girl, Aubry, who is 2 weeks younger than Bella! We noticed them playing in the water, and Ali wanted to meet "that little baby," so off we went. Well, we started playing together with them, and before you know it, we're all talking and playing and having a blast, moms, dads, kids, even aunt and uncle and their two dogs!

We used the "tag-team-time-to-go" technique (parents, you know this one) to get the kids out of the water and on our way, and as they were drying off next to us, Collin shared his graham crackers with Ali...

... and when it was time to leave, they instinctively took each other's hand and headed off together walking and talking away like best friends...

They wanted to go see the falls together, so over the bridge they went...

and up the path to the falls...

When the rest of us got there, Ali saw Aubry and gently walked up behind her and gave her a hug. It wasn't just any hug, though. It was the kind of hug a big sister gives to a little sister. Maybe the kind of hug this big sister has been dying to give for too long now....

Collin's mom was amazed at the spell Ali had put over him! He was acting so polite and calm... she asked if we wouldn't mind getting together again! I was hoping she was going to ask that because we felt the same way, but didn't want to impose. Anyhow, we exchanged phone numbers, and they offered to have us over and cook for us! The kicker was that as we were saying goodbye, Collin walks up to Ali and says something to the effect of, "May I see you again sometime?" to which Ali responded, "Sure."

I just watched Ali get asked out.

Oh, MAN, it was so cute!

Collin's mom, Deb, and Angelique both heard the whole thing go down as well, and we were beside ourselves trying to keep quiet! Anyhow, no sooner than we get to the car, I get a text page, "Hey, remember how we were going to invite you to BBQ? Well, how bout right now? We're in the park and have plenty of food!"

Before you know it, we are all sitting down together eating brats, burgers, and peanut butter and jelly sandwiches. After we said our goodbyes, we texted again tonight, and Deb reiterated their commitment to having us over and to do anything they could to help us while we're here.

When do complete strangers reach out like this to each other? Does it happen every day? Are we so inundated by bad news and tragic headlines, that we miss out on hearing about God's providence playing out in parks on Saturday afternoons? I tell you I am so unbelievably humbled to be in the middle of this ongoing story. I cannot help but end my days in utter gratitude for the world I live in. How could I not? I feel like a kid watching a magic trick for the first time. I am in awe and complete wonder at God's masterpiece: LIFE.

It's the best game ever invented.

Thanks for playing with us. Without you, who would we share today with? Without you, how could we glorify God's providence in all our lives? Who would we give our testimony to? The word that I often don't know how to use in the Christian sense is "witness." Growing up Catholic, it isn't used quite the same way. However, what it means to me today is that today I got to witness God's love poured out for my family by complete strangers. Mind you, they didn't know Bella's whole story till the end of the BBQ; they just knew we had a kid in the hospital. Good people are good people not for what's in it for them, but for what's in it for others. Another's joy is their joy. Another's comfort is their comfort, plain and simple.

We were blessed to meet some good people today.

You have to appreciate that between receiving your support from far and wide over the internet and through the mail, and the support we have received from so many on the ground here locally, we cannot go to sleep in any other state than one of gratitude.

Now, here's your challenge:

If we can got to bed tonight in an attitude of gratitude DESPITE Bella's situation, can YOU go to bed with an attitude of gratitude despite yours?

Good night.

August 27: Day +56

Hello, Happy Friday (or Saturday) to you all!

Well, we had a breakthrough today! YESSSSSSSSSS!!!!!!!!!!!!

Remember how we couldn't figure out how to get Bella's blood pressure from bottoming out? Remember how we started her on vasopressin along with the esmolol on Sunday, and her pressure has been in the toilet ever since?

You're gonna love this.

I found out in rounds this morning that the team learned yesterday evening that...

... Prisma dializes vasopressin.

Seriously. After being on the drug since Sunday, and on Prisma (continuous dialysis) the whole time, the team just found out last night about the drug interaction with dialysis...

So, we started going UP UP UP on vasopressin today. I told the team for the second straight day NOT to look at the dose, but look at the patient. Since the dialysis was dialyzing the drug off, WHO KNEW how much was actually being metabolized?

At one point, Sam the attending ICU doc, who I really like a lot, mentioned as he was walking out the door that he had half a mind to get rid of the vasopressin and go back to norepinephrine. That got Chris, Bella's nurse, and me to brainstorming. We began to think about what the days on vasopressin had been like. Sunday... okay... for half the day. Monday... bad. Tuesday... bad. Wednesday... bad. Thursday... pretty good... hmmm... what was different about Thursday? Why were her pressures so strong?

We were off Prisma till late in the afternoon! The vasopressin wasn't getting dialyzed so it could actually do its job! That was it: Vaso gets dialized, norepi doesn't. We needed to go back onto norepi and get rid of vaso. The only reason we ever switched was due to esmolol, and we were off that, so why were we on vaso if it wasn't working?

I literally ran out of the room to get Sam. He came back in and we laid out our argument. He bought it. Let me rephrase... we laid out why HIS hunch was the best lead we had. It still didn't solve the cause, but might at least treat the symptom in the meantime. Well, how can anyone refute their own idea? ;) He agreed to go back to norepi. Within about an hour back on, Bella's blood pressure jumped up and they began to wean off the vasopressin. The goal through the night is to wean it off completely and return solely to norepi.

I can't tell you how happy I am tonight. We also went down on her narcotics, and went up on her methadone and scheduled her ketamine on a regular basis. So, we have a new sedation profile that we are transitioning into with excitement. We will move off versed and dilaudid, and remain on ketamine, methadone and precedex. When I left tonight, Bella's pressures looked GREAT, and she was clearly awake, but seemed more peaceful at the alert state she was in than in recent days.

Now, hopefully tomorrow we can begin to pull fluid off poor Bella since she is an absolute giant right now. I feel like we just chased a ghost of a heart murmur for 10 days now, and we are just now getting back to where we were. I am a little frustrated by the process, but this is the nature of the beast. It is like seasons. Just because you may want summer to come right after winter, you can't force it to. You need to go through spring whether you want to or not. Similarly, you can't move any faster than a slow crawl in the ICU, regardless of how impatient you (I) might be.

I will share a cute moment today... we were taking off Bella's headgear so we could roll her over and change the dressing on her ulcer. The RT had all the bandages off Bella's face, and was just holding the tube in her mouth for a minute while the nurse was administering a drug. Bella was awake with her eyes open, so I started to sing one of her songs to her. Well, while I was singing, Bella scrunched up her face like she was trying to smile. Her whole affect brightened! Poor thing is so swollen right now that she could hardly move but she smiled with her entire face! The RT noticed also and we had an, "AWWWW, SHE'S SMILING!" moment in the room. It was really tender and sweet. Bella has endured much, and will need to endure much more, but I will say that if you just give me a moment like that to me every now and then, I'll be up for being beside her the whole way!

Lastly, Ali has been practicing her letters and impressing us with her ability to write pretty much the entire alphabet now. What has been really interesting is that she doesn't consciously know what she is spelling when she starts drawing letters... BUT, recently, she wrote HLA and TPA in my notebook. These are very specific transplant acronyms that she no doubt has heard.

Today, however, takes the cake. What word does the below picture resemble?

You got it.... PRISMA. We joke about "Rosy the Prisma Machine," but no joke here! She doesn't know that she was almost spelling prisma; she never made a comment about it, and she has no interest in trying to spell words correctly yet. What's on ALL our minds 24/7 up here? Bella.

Last, thank you all who have posted these past 2 nights. We are so flattered and humbled by your outpouring of love and support. I again say that the reason tragedy and "bad things" happen in the world is for us to have the opportunity to KNOW ourselves as loving, nurturing, compassionate, helpful human beings. Those opportunities are there every day, but tough times seem to "turn up the volume" on the opportunities to help another human being. This truly appeals to the highest parts of our nature and we experience ourselves as connected to another, whether giving or receiving, and we experience ourselves as good, and we have an innate desire to KNOW that we are in fact good after all. Bella in turn has come to earth with EB to give us all the opportunity to care for her... to CARE FOR ANABELLA. It is in doing this that we get to know ourselves as good people by wishing good upon another, and for many of you, another that you have never even met. Simply extraordinary. We get to see by all these comments, that there is a lot of good in the world, despite what the major media outlets spew every day. Miracles abound in OUR community, in our world. This is our community, and we feed each other.

Thank you (the person sitting in your chair right now - yes, you!) for making this community so special. It is an honor to be in community with you.

Good night.

August 26: Day +55

Wow. You are all amazing. Thank you so much to all of you that took time out to post a comment, no matter what you said, and no matter how long it was. Today was dreamlike reading your words. To hear what you've been feeling and praying for on our behalf... without those comments, we don't get to see just how many people are thinking about Bella, and praying for her daily. It was no coincidence I was able to handle the day at hand with such happiness.

The day started out with Angelique calling in sick with a cold. It came on outta the blue yesterday evening, and she spent the entire day today in bed. So, after a half hour in rounds this morning (seriously) to bring the new attending BMT doc on board and wrestle with the many issues on hand, I was off to the pharmacy to get cold medicine for my sweet wife.

After dropping that off back at RMH, I got back to the hospital and we did a dressing change since we were changing the dialysis circuit today. We had a wound care nurse come to look at the back of Bella's head. Not good news. Bella has developed a decubitus ulcer, or a bedsore. This is the most common hospital-acquired injury out there and is taken very seriously. Reports had to be filed, pictures taken, the whole bit. Long story short, back in July, RT gave her a harness for her breathing tube that was neoprene with a hole in the back. It was a chin strap that was turned around backward so the hole was behind her head. Even despite copious amounts of protective dressings, when she got really fluid overloaded in July (the first time), her head swelled through the hole in the strap and the skin broke down. Then, due to her prisma line being soooo tenuous that a diaper change caused problems, we were unable to really get at the wound very well except for quickly while we had 3 people roll her over. Well, given where the ulcer is, it was really hard to see clearly. Today, we rolled Bella over in the opposite direction while she was off her circuit and we finally got to see the true nature of the wound.

Now, I'm going to say what I'm going to say, and please don't rush to my aid with your comments to make it better. As the person in charge of her skin care, I felt like such a failure when I saw the extent of this injury today. How did I let this wound get so bad? Again, just let me hang out with that... it's okay. I know what the circumstances were, and while they were simply beyond my control for the cause, I'm just amazed the wound lay undetected for so long. I understand why... we simply couldn't see it, given her lines and prisma, but at the end of the day, it was and still is my responsibility to care for her skin. Everyone on the treatment team knows this and defers to me on her skin care.

Anyhow, having said all that, the wound care nurse was extremely complimentary on the job I was doing, and liked the way I had been treating the wound up to this point. The only thing we are going to do differently is roll her over every day and dress the wound with a fresh dressing. Up till now, we only changed the dressing once every 4 days when we'd change prisma. Well, it'll take 3 people a day now to do it, but we have to treat this very seriously as it is a 2 cm long hole in her head that gets no daylight, lots of body heat, and moisture from sweat... see where I'm going with this? Major priority now.

In other news with Bella, we dropped her precedex to next to nothing today. This is one of the sedation drugs that has been masking how Bella is really doing. Bella is clearly going through withdrawals; she is shaking like a leaf on a tree. She is swollen to the point where I don't even recognize my little girl any more. I won't show pictures today because honestly they are extremely upsetting. However, her blood pressure hasn't looked as good as it does today in a LONG time, so we are all very, very happy about that. It seems we found the right amount of vasopressin to support her blood pressure. We are starting her on methadone to help her with her withdrawals, and we are going to wean her as fast as she'll let us off as much as she'll let us to try to find the kid who came in here two months ago with a swollen airway.

Just before going back on prisma, we got the call that the docs all wanted to get a CT scan of Bella's head, chest, and abdomen in an effort again to see if they can spot any infections anywhere in her body that they've missed so far. She had a fever last night for the first time since chemo, so there is a concern that there is an 'occult infection' sneaking around in there that is not showing up on cultures, but continuing to be responsible for the soft blood pressures. So, we headed to CT today around 2 and did our thing. Never a dull moment.

Once back up on prisma, it was time to get back to RMH. I had to go to the store to get fruit for a school party for Ali tomorrow, then pick her up from school and meet momma back in our room at RMH. We hit dinner and bingo, and came back up for playtime at RMH.

There are so many issues on the table that are going to take a whole lotta time to undo. That's okay. I got nothing BUT time. Right now, there is nothing I want to do more with my life than completely rehabilitate my little girl. I watched parents drop their kids off at U of M today. I love this time of year. It holds such promise, such possibility. As I walked past the dorm, the hospital loomed overhead. I looked up toward Bella and promised her I'd drop her off at college one day. I don't care what it takes from me to make that vision a reality. There is nothing in my life that is worth more than dedicating my life to this little girl. I used to have dreams of financial wealth and abundance. Now, I dream of Bella. I know that God will continue to provide my family the means to allow me to dedicate all my time if necessary to being full time dad/physical therapist/occupational therapist/music therapist/wound care specialist... caregiver, whatever it takes. Now, if I could just sell a few more books... :P

So, all in all, there is still a very positive feeling in the air about "solving Bella." One thing that is happening is A LOT of communication and brainstorming, and that is a good thing. One new thing that is on the table is that Bella may need a tracheotomy. The breathing tube is beginning to cause more problems than solving, and so Dr. Tolar is spending a week talking to EB experts in Colorado and Cincinnati to discuss the possibility. It is completely reversible and is done for ICU patients when they know they will be intubated for more than a month normally. The problem in this case (why they haven't done it sooner) is that with a BMT patient, you have to wait until they are fully engrafted, and even then, they usually don't feel comfortable doing this procedure before day +90.

Remember last week, when we had to picture open heart surgery as an option for her outflow obstruction that magically disappeared? This is the nature of being in the ICU. This week's nightmare? Tracheotomy. Not that there's anything wrong with putting one in. We did the G/J tube fully expecting at some point to go back to a G, and eventually get rid of that. It's just that it is a rather invasive procedure that we never in a million years thought we'd have to wrestle with as RDEB parents. Our JEB parents in the EB community have to wrestle with this choice more than we do since JEB involves the lungs and RDEB doesn't. With RDEB, the debate is usually over G tube or not. So, we're now getting to bounce this one around as well.

Honestly, it's just another conversation. I am hoping Bella rallies so we don't have to go down that road in reality. We're getting used to going down scary roads in conversation. We've had to do that since the day she was born. Yes, it is stressful and scary, but it is also just the nature of the beast. We can't "wish" we didn't have to think about such things. EB requires us to deal with things as they are, not as we wish they would be. It's a powerful metaphor for life in general. Life is much easier to navigate through when you are relating to what is instead of what you WISH would be, or better, what you think SHOULD be. That's pretty useless. It's easy to do, don't get me wrong; I'm first in line for the award for having a 'flair for the dramatic.' That is why I am so blessed to be married to one of the most grounded people I've ever met. Angelique has taught me the simple (but not easy) practicality of dealing with the matter at hand, not the matter in my head.

Alright, I've gone on for long enough here. Thanks for indulging me. What I am reminded of each day here is that we are far from alone. There are parents fighting for their kid's lives every day in every children's hospitals in everywhere day after day after day, and some win, and some lose. I eat dinner among heroes every night of the week at RMH. When you hear the struggles another family is facing, it puts it all into perspective, and above all, despite it all, it reminds me of HOW ABSOLUTELY BLESSED I AM, AND HOW BLESSED MY WHOLE FAMILY IS. I give thanks every day for exactly how it is, right here right now.

Good night.

Fabulous socks of the day...

August 25: Day +54

I'm pretty sure today was a good day. Lots to share...

First piece of good news is the esmolol is out of the picture! They D/C'd it early this morning, and this afternoon, when they did another echocardiogram on Bella, her outflow obstruction was still gone! Her heart sounded great to the cardiologist, Dr. Pyles, so... where were we... oh yeah... It was as if 10 days had mysteriously disappeared, because while we eliminated this whole heart theory, we are still back to where we were 10 days ago to the T... blood pressure that is just too low.

The good news is that there is a buzz of brainstorming and consulting and just plain 'fire' going around the docs about Bella. The docs are so frustrated that they can't figure this thing out that they are calling everyone they know, consulting, literally racking their brains trying to find the solution. We like this.

Again, her problems are:

1) low blood pressure

2) kidney failure

3) acidosis (blood pH is too acidic)

4) fluid overload from leaky vessels

So, the next place we are poking around is in the world of sedation. We have gone up quite a bit on her sedation over these past 10 days and it could have a part to play in her low blood pressure. So today, we started by cutting one of her drugs (precedex) in half. From there, we are going to wean her back on the two narcotics (have to move slower on those to avoid withdrawals) she's on, versed and dilaudid, in an effort to wake up her body. Her mind is awake as evidenced by her pupils now remaining dilated, so we think if we wake up her body a little more, this will allow it to 'kick into gear' more on its own.

We are also going to install an arteriol, or "A" line in her wrist. This will measure her blood pressure on a constant basis and will be far more accurate than the cuff.

Here's a depressing fact:

Her right lower leg has been SQUEEZED by her blood pressure cuff over 5,000 times in the past 52 days. She gets her BP read at least 4 times every hour, and every 3-5 minutes any time we start up a new circuit. That's roughly 100 times a day, every day, for over 50 days now.

Here's a miraculous fact:

Not one of those times caused a blister.

Thank GOD! No, seriously, THANK GOD!

So, an A line will be put in by the bedside, and that will help us get a better view into her pressure.

We also weaned back the steroid hydrocortisone to 6mg every 6 hours, down from 8, which was down from 10. So, we are at just above a half dose of what she was getting, and I really don't think we'll be able to go any further, given her adrenal insufficiency. The goal is to wean 20% every 3 days if she'll let us. Dr. Pyles also doesn't think her hypertrophy is necessarily linked to the hydrocortisone. He said he could see it on the echo from July, which wouldn't have given the drug enough time to do the damage, plus she is a little old for that reaction; it usually occurs in much younger infants. So, if we need to leave a smaller dose running, I'm not as worried about it as I was a week ago. It's just amazing the pretzel you have to turn yourself into regarding whether all these drugs are good for Bella or bad for Bella, and at what doses for what period of time in combination with what other drugs... DIZZYING... almost. I am pleased that I feel like both Angelique and I have kept up with the conversation and the implications of the various drugs Bells is on. I remember when Rafi was going through her transplant and her daddy, Brett, would write about the various drugs and doses... I was really intimidated. I was intimidated when Marybeth wrote about Sam's drugs as well when Sam got her transplant. It really is much easier to follow when you're here all day focusing on nothing else... and have access to wikipedia on your laptop! lol.

SO, there is a "second wind" in the air about "solving Bella" on the unit which we feel really good about. Where's House when you need him? He would have had this whole thing solved in 44 minutes, LOL. Oh, Hollywood. Thank you for completely distorting reality for us all.

In other news...

Today, there are no pictures largely because I was in front of cameras instead of behind them. Today, we filmed a DVD for Bella of mommy and daddy and Ali. The Child-Family Life dept. coordinated with the music therapist (yay!) to assist us in shooting a video for Bella that could be played in her room when we're not there. This will be particularly good distraction material when she is out of her drug induced coma, but the auditory stimulation in the meantime will be beneficial.

After the video session, we had a photographer from Now I Lay Me Down To Sleep come and take portraits of us with Bella. Our social worker thought of this last week, and I realized we had never done a family portrait with the four of us before we left. So, a wonderful photographer turned Bella's room into a portrait studio and shot really sweet pics of Bella, and each combo of us with her, as well as pics with all of us together. It was quite the yoga session to squeeze us all in around her, but it worked. It's probably gonna be awhile before we get the pics back, but I promise I'll be sharing them as soon as I get them!

I had to bribe Ali with ice cream to get her to take some extra pictures at the end of the session, so afterwards, we all went to Punch Pizza for dinner and Dairy Queen for dessert! It was a fun family day in the middle of the week. Thanks for the ongoing prayers, thoughts, and intentions.

One last request, if you haven't commented, would you take a minute to do so? you don't need to worry about what to say. It's not so much WHAT you say, it's THAT you say anything in the first place that touches us. That you take the time out to be a part of our life makes our day, one day at a time. Also, would you be willing to share about our blog on whatever social network you are on? We would be most grateful. Thank you, and good night.

August 24: Day +53

Ali rocking mommy's reading glasses...

And the roller coaster continues!

Wow, what a day. This morning, Bella's pressures were in the toilet again (like 41 over 21 at one point), and they infused her 5 TIMES (2 saline boluses, 2 red blood cell packs, and one platelet pack) over night to try to boost her blood pressure. Think that's enough fluid? Bella is leaking fluid out of her blood vessels out into her tissues faster than they can infuse it. No one knows why. Normally, this can be a BMT issue, but is usually healed by day +30. Anyway, her heart rate also climbed up to 130. So, now the only positive effect of the esmolol had disappeared, and her pressures were 40's-60's over 20'-s to 30's no matter what they tried. They just couldn't get it back.

The plan in rounds was to get another echocardiogram tomorrow, but they moved it up to this afternoon. I have still not met with a cardiologist at this point, and when the nurse found this out, she was incredulous, so she ordered a consultation for me. So, the ultrasound tech came in and did the ultrasound, and a little while later, Dr. Pyles came in right as the attending ICU fellow was in the room. Perfect. Dr. Pyles said the obstruction was gone! The hypertrophy was still there, but when Bella's heart pumped, there was no "gradient" - no obstruction to the blood flow. He said to cut the esmolol dose IN HALF. The words were barely out of his mouth before our nurse was already changing the dose on the med pump!

Esmolol has been a controversial call, and while we had one day on it that looked okay, we've had two subsequent days on it in the toilet. When her pressures were fine, I was okay with it, but she couldn't hold it up and to be leaky with low blood pressure, it's not the best choice, especially when it was no longer holding her heart rate down. It has been debated in offices, in Bella's room, and I was told even at the lunch table in the physician's lounge today as to whether it was the right call. Hey, whatever the right call may be, the obstruction is gone, and it's wrecking her BP, so we are phasing it out as rapidly as possible. After an hour or so, Dr. Pyles had the echo team come back and do a quick scan while she was on the half dose to see if the obstruction returned. He stood across the room and directed the procedure himself, and sure enough, it was still gone, so he halved the dose again! So, now we are at 1/4 the dose we were two hours earlier, and you'll never guess...

... her blood pressure returned! Go, Dr. Pyles!

The BMT fellow came in to see me and she asked, "In your own words, how would you describe the past two weeks for Bella?" Well, I broke it down for her. I explained that we just spent 9 days chasing this heart murmur around and around with 3 echos, a cardiac MRI, a chest CT, and two new medications, when two weeks ago, we were two days away from extubation when Bella got an infection. While I appreciate all the due diligence, I still felt like we were running around in a circle/painting ourselves into a corner, whatever phrase you want to put on the whole thing. She smiled. Her response was, "Well, we feel that since Bella has gone on esmolol, she hasn't been happy, and we haven't been happy, so we're gonna get her off it." If only I could speak that concisely.

Now, you know I've turned myself inside and out like a yogi to get behind this drug. It was no easy sell for me. I was only too happy to hear that it was going away. I had no problem hearing that, believe me, despite the work I did convincing myself it was the right drug at the moment. Hey, if the obstruction was still present, things might be a whole lot different, but as long as "it" is gone, get the esmolol outta here! To be fair, I choose to thank the esmolol for whatever role it played in clearing the obstruction, and now it is time for it to be on its way.

We also had surgery come in today to re-suture Bella's hickman to her shoulder. Both sutures had been pulled out of her skin during recent circuit changes, and there is now a deep pothole in her shoulder where skin used to be. There are two teams that touch that line, nursing and dialysis, so I will be putting them both on notice tomorrow explaining that they pulled out not one, but two stitches out of Bella's shoulder, and that they need to be more careful going forward when they take her on and off her circuit.

It was a long day, but in the end, Bella is stable again, and we all feel like we've put this heart fiasco past us. we will get another echo tomorrow to see what her heart looks like on the 1/4 dose of esmolol. Please pray that it looks the same and that we can D/C it for good tomorrow.

Meanwhile, enjoy the Ali show... Good night.

Ali starring in "The Little Mermaid," again...

Ali telling jokes to Bella's nurse...

Ali posing with the therapy dogs at RMH...

August 23: Day +52

Who is that? It's Bella!

Up days and down days...

... today was not exactly up. Sorry. Bella rarely seems to give us two in a row of anything, and if that is the case, tomorrow should be great! [tired smile, followed by a sigh]

Bella was a little restless last night and needed a few bumps of sedation throughout the night. That was a bad omen for the day, because usually she sleeps pretty peacefully through the night. If she was up, it meant she'd most likely be pooped today, and boy was THAT the truth. She tanked today in just about every way possible.

Her blood pressure was through the floor at several stages today, this evening, and tonight. And when I say, "through the floor," I mean like 60 over 21. At first, I thought that since we've D/C'd norepi and weaned back on hydrocortisone, we'd have to play around with the vasopressin before we found a happy medium. The problem is that we were dealing with low blood pressures before we went on esmolol. Now, her blood pressure has an antagonist on the inside (probably CIRCI - Critical Illness Related Corticosteroid Insufficiency), as well as one on the outside (esmolol).

So, first, we cranked up the vasopressin to try to even things out. Nope. We rolled Prisma back to net zero to help. Nope. We gave her a saline bolus to increase her total fluid volume. No dice. Finally, after ordering an EKG, they infused blood, and ONLY THEN did her blood pressure respond at all. Hopefully, it will stay up through the night.

Daddy changing out head dressings with respiratory therapy...

We are still taking it one day at a time around here. There really is no "plan" past trying to get her back to a fluid-even weight and keep her stable. On top of everything else, her blood gases went really acidotic (acidic to you or me) today so we had to crack the vent back up a ways as well.

Again, I think the heart of the matter right now is that we have 2 main issues one of which is causing a third:

1) CIRCI (adrenal insufficiency). Evidenced by low blood pressure despite IV fluid and vasopressors. Main treatment? Hydrocortisone.

2) LV Hypertrophy (thickening of the heart wall in the left ventricle). Causes poor blood pressure due to inability to fully pump blood into the circulatory system. One main cause? Hydrocortisone.

3) Acidosis of blood pH. Unclear as to origin. Haven't gotten any good answers, but also I haven't asked the right questions... I'm usually asking about BP and drug/fluid interaction.

Oh yeah... and then there's...

4) Renal Failure

Bella checking daddy out during bath time...

So, ups and downs. I feel afloat drifting in 40 foot waves in a giant storm with nothing but a life preserver on. My life preserver is my faith. It is rain-soaked, waterlogged, fraying at the edges, and just plain worn down. It's the only thing that keeps me from sinking permanently into despair. There are days when I am happy, days when I am angry, and days when I am fearful. Today, I am fearful. Dr. Verneris, many many weeks ago, cautioned that one thing you don't want is to be parked in the ICU. Well, it's happened. We're parked with no view of 5D in sight. I have faith that the view will change. I can't even feel it right now. Right now, the fear's grip is just too tight. Nevertheless, it's all I've got to get up tomorrow and do it all over again. I'm holding on to my life preserver as I fall asleep tonight.

Dear God, you can read the homing beacon in my life jacket, but don't come and get me. Get Bella instead. Heal her kidneys. Heal her heart. Heal her adrenal glands. You have already healed her liver. You have already healed her skin. You have given us so many tools to work with. Thank you for all of this, and for the practitioners who wield them for Bella's aid. Please, God. We grow weary, and need to lean on you now more than ever. Please "solve the case," so that victory may be proclaimed and your power and love may be witnessed by those following Bella all over the world.

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."

Good night.

August 22: Day +51

Mommy giving Bella her evening aquaphor massage...

A good day... (yay!)

I walked in this morning and found out that we were off norepinephrine since 10pm last night. Then, I looked up at her blood pressure, and it was STRONG. Man, that was great to see. We've been on that stuff for a long time now, and it was nice to get rid of another medication! Then, I looked at her heart rate. 103. This got me thinking. Esmolol was running at 200 (don't worry about what unit of measurement), up from 175 last night. Normally, Bella's resting heart rate is 110-115. We are trying to get her HR down into the 80's-90's, but low 100's is an improvement.

Then, for some reason, I had another thought.

I asked Bella's nurse, Lori, to pull up Bella's HR over the past 7 days on a graph on the computer. Bella's HR has been spiking into the 180's which has not been helping with her heart condition (hypertrophy - or swelling of her left ventricle wall). Sure enough, as soon as we started esmolol, the spikes disappeared. Her heart rate had stayed nicely within a 10 beat per minute range for 3 days straight. Now that we were weaning the hyrocortisone, and D/C'd the norepi, the esmolol could finally do a little of what they hoped. So, While it didn't get her HR to where they hoped, it stopped it from going where they didn't want to go. Being the visual kinesthetic learner that I am, when I saw that graph, everything changed for me.

At that point, I did an usual thing for me. I changed my mind. LOL.

I realized that the esmolol was indeed working for Bella, now that it had room to. I am grateful to God for boosting her BP along with the right dose of vasopressin. They are using the vasopressin and esmolol like a compressor in the sound world to keep her heart rate in a manageable range. They have "dialed in" her meds and reached an equilibrium where we can pull off her excess fluid, and wean her vent while not working her heart a single beat more than necessary.

We had a good pow-wow in rounds and everyone (including me) was on the same page for a plan for the day. We restarted J-tube feeds, weaned her ventilator a little, and started to pull of extra fluid every hour with Prisma to make up for a few days in a row of too much fluid (mostly from two circuit changes in three days).

Dr. Tolar, Nurse Lori, and Bella...

When Dr. Tolar came in to see Bella after rounds, I told him I had changed my mind about the esmolol, and finally come around. He said he felt the same way. He reminded me just how complicated Bella's case is, and how her problems have evolved so much these past two months. Everyone involved has had to become really flexible in trying new things with her to try to get the best result given the amazing set of parallel issues occurring.

I didn't feel like such a schmuck after that. :)

I laughed to myself this morning after I changed my mind. I thought, "What will I say on the blog? I was so gung-ho yesterday!" The funny thing was, I really knew I wouldn't lose face at all. We are in uncharted waters, and I have been praying daily for trust. Well, it finally arrived. I ride the docs and nursing staff extremely hard, and I need to be willing to bend as much as the next person, if it's in Bella's best interest. Just like when they wanted to put in a new catheter and I was against it, but realized we had to... now the new one works like A DREAM and we are all so much better for it. It allows everyone involved in Bella's care to "do" more of her care without threat of her dialysis circuit crashing every time you change a diaper even!

I'm pretty sure that at one point when I was young, my dad showed me a picture of our family coat-of-arms, and it had a latin inscription that meant, "Trust, BUT VERIFY."* That is the harmonious middle ground I felt like I reached today!

* = I am too impulsive to cross reference the above statement with my brother Paul, who is our family historian, so I may have to edit this upon further clarification

Anyhow, bottom line is that Bella is off one more drug, (slowly) weaning off steroids, weaning off the vent (a little), and is currently in a good place with all her vitals. WE'LL TAKE IT!

In other news...

We hit Mall of America today! Not to shop, mind you... MOA is all about the Underwater World aquarium and Nickelodeon Theme Park in the middle of the mall! Those two will rip a whole in your pocket, but it's fun to do every once in a great while. They added a butterfly exhibit there as well, so Ali got to go to two butterfly exhibits in two days at two different fun places! The kid is definitely maxing out the fun every weekend, and rightfully so. Enjoy some various pictures. Thanks for all your love and support. Good night.

August 21: Day +50

Mostly, it was an okay day today.

Good news:

Dr. Tolar began to wean Bella off hydrocortisone today! I couldn't stay for rounds today (they were 2 hours LATE) but he met with me before hand and I laid my case out for him, including 3 research papers (2 from one of our readers in Portugal, thanks Carla! and 1 I found myself) that attributed left ventricular hypertrophy to hydrocorstisone. The good news? After the hydrocortisone was discontinued, the hypertrophy went away. When Dr. Tolar walked in, I was reading one of the abstracts, so I just handed him my computer and he smiled. I told him about Carla, and that I have a world-wide following doing research on behalf of Bella. He liked that a lot.

They discontinued one of Bella's antibtiotics today as she shows no visible signs of infection. They will most likely d/c the 2nd antibiotic tomorrow.

The Prisma crashed this morning around 6 and they got her back up on a new circuit by 11, which frankly on a weekend, I am quite pleased they got it back running that quickly.

The weaned Bella's one of Bella's other drugs, norepinephrine, to just a whiff.

The bad news?

Bella is still on Esmolol. Why? I have no friggin' idea. I still feel like I'm taking crazy pills on this one. Esmolol lowers blood pressure primarily. Secondarily, it can lower heart rate. They can't give her the dose they think will lower her heart rate, because it lowers her blood pressure too much! Does any of this seem strange to you, too? Low blood pressure is Bella's problem, not her goal.

One thing I can promise you. Bella will not be on Esmolol by this time tomorrow. Due to the weekend schedule, I missed the cardiologist that is behind this bizarre plan today. I will be hanging around like a virus tomorrow till I corner this person if they are not in rounds, whatever time that ends up being. Even Bella's nurse started hammering the resident this evening, because the plan CLEARLY isn't working. Bella's heart rate has not moved an inch, and her blood pressure has tanked yet again, and now they are having to give her more of the 3rd blood pressure drug they started yesterday to compensate for the Esmolol.

Makes. no. sense.

In other news...

Ali got to go on a play date with her friend Ellery to the Como Zoo today. Thanks, Logan, for taking them! This allowed me to actually work for two hours (I actually work still about 3 hours a month doing consulting from here).

After, Angelique and I went to Minnehaha falls and had a little picnic! There is a really cute little spot below the falls where you can wade in the water that I CAN'T WAIT to get Ali into soon enough! We also took some time to do laundry and clean the apt... things that pile up during the week. Tomorrow, Ali wants to go to Mall of America, and she'll either go just with mommy while daddy dukes it out at the hospital, or I'll 'clean house' early in the morning and get a chance to have some fun myself.

Thanks for your unending love and support, Good night.

August 20: Day +49

TGIF.

My brain is full. In another browser, I currently have 4 different tabs on wikipedia open:

Dynamic Outflow Obstruction

Cortisol

Blood Pressure

Adrenal Insufficiency

I've been spending the week trying to wrap my brain around cardiology and how Bella's situation defies all convention. Bella has left ventricle hypertrophy resulting in low blood pressure. Problem is LVH is usually a result of HIGH blood pressure. Today, we went on a drug that is usually given to lower blood pressure. Then, we added another medication that raises blood pressure. This is on top of the two other drugs we are currently on to raise blood pressure.

Sound a little confounding? Now you get a glimpse into why I have a chronic headache. If you ever want to go insane, try to understand an insane person's logic. I feel a little insane trying to understand today's call. The med we gave (esmolol - a beta blocker) was given with the intention of lowering Bella's heart rate down to around 85 bpm. Her resting HR is around 110-115. The thinking was that if they could slow down the heart rate, they would allow the heart to fully close and open and rest in between, thus somehow lowering the progression of her hypertrophy. Fair enough, but after several hours on the drug, it did nothing to lower her heart rate. Frankly, it did little of anything, despite the ever increasing levels they kept giving. Despite this, no one was willing to stop the drug. Now, she has a new drug running that isn't working, and they added a third presser to counteract the side effects of this ineffective drug.

I feel like I'm taking crazy pills.

The one good piece of news was that we at least starting talking about weaning back the hydrocortisone. We have been on a big dose of hydrocortisone (cortisol) for a month now, when the original plan was to use them for 72 hours then wean off them. The attending ICU fellow tonight mentioned that hydrocortisone can lead to hypertrophy.

Okay, in that case, 1) we've been on hydrocortisone since July 21. 2) we have hypertrophy.

Am I missing anything?

Soooo...I asked him when we could start weaning off it. The fear of God came over him and he immediately began hemming and hawing about how long it would take to do it and that we'd have to talk about it and plan it out.

Ok, big deal, start talking and start planning, right? So, I pressed on and asked when could we at least begin the wean? Then, he passed the buck to BMT and said since it's their drug, they have to make the call. Alright, I know who to bug over there in BMT...

Bella is squeezing her hands so tight, she squeezed off her last fingernail on her right hand...

In the meantime, Bella has rapidly spreading petechiae, which is usually from low platelets. Bella's platelets are fine. No one knows why she has petechiae which has gotten worse and worse over the past 48 hours. Also, her right leg is discolored and swollen, and she is now way overhydrated (again) and puffy all over.

I feel like we've painted ourselves into a pharmacological corner by stacking so many drugs on top of each other, especially because Bella flat out looks worse today than yesterday.

It's just so hard to watch her slide so far back from where she was a week ago. Ugh.

In some good news... Daylon transferred back to 5D today!!! YAY, DAYLON! This is good for Daylon and good for Bella since she copies everything he does anyway. We gave her a hard time tonight, telling her that her boyfriend skipped out on her and she better follow him over there to keep an eye on him (He's pretty cute, you know).

My brain needs a rest. Please pray that the docs have the courage tomorrow to begin weaning Bella off steroids. It's time. My gut is YELLING at me about this one at this point, and that is exactly what I told Dr. Tolar. Hopefully tomorrow he will take the reigns and get this process started.

"BEEELLLLLLLLAAAA!!!!" Holy cow, I almost dropped her!

Tomorrow will be a fun day. Rounds aren't till 9, Ali gets to go on a play date with Ellery to the zoo, and mommy and daddy are going on a picnic date to Minnehaha Falls. Don't tell Ali! tee hee. Good night.

Always leave on a high note!