One Year Ago Today: 12/7/2012 - Progress! A Diagnosis & Lamborghini Juice

Evie and I had a great sleep last night. Really we were both so exhausted that nothing could've stopped us, but it was wonderful. Today she's enjoyed unfettered access to the Disney Channel and we've nearly finished reading Harry Potter and the Chamber of Secrets.

Our morning started off with another EKG and echocardiogram.

This time the cardiologist was right there while they did it and he showed me her left ventricle. Yesterday her left ventricle was "severely dysfunctional". After the barrage of medications that were taken last night and the less stressful type of dialysis, her left ventricle today rates as "moderately dysfunctional, nearly slightly dysfunctional." So that is all _Fantastic_ news. The cardiologist is fairly confident that the onset of the myocarditis was rapid/acute enough that the recovery should be equally quick. Once her heart returns to regular size and stays that way for a day, they will start to decrease the amount of medication she is on and continue to monitor the size of her heart to make sure it doesn't swell again.

She had dialysis all night long, and has continued on it all day. She's enjoyed a couple dozen ice chips today (about the size of really large jelly beans) to help with her dry mouth issues.

We belong to The Church of Jesus Christ of Latter-Day Saints, and early this morning I looked up the closest congregation to the hospital, and left a voicemail message with the bishop indicating I was trying to find a local priesthood holder that could help me give Evie a priesthood blessing. One of his counselors works here on campus, and was able to come over about a ¹/₂ hour later. He happened to arrive right when several doctors came in to talk to me, and kindly/patiently waited until we could give Evie the blessing, and then stayed for a bit just to let me talk, and before he left he offered to help in any other way he can. It is nice to know that you can depend on a random stranger if you need to. :-)

We added TPN to Evie's IV cocktail to compensate for the lack of food. She's mentioned several times that she plans to eat ice cream and pizza at her first opportunity. Although she has shown signs of improvement, she still is unlikely to be allowed to eat or even drink for a few more days. Poor thing.

She had an arterial IV line put in today so that they could keep a very close eye on her blood pressure. Gratefully they gave her topical anesthetic to numb the skin--then put more local anesthesia in after they made the incision, so it didn't bother her one little bit.

There is a medication that Evie's Nephrologist (Kidney Doctor) mentioned several days ago, but wanted to rule out several things before really discussing its use. Due to what transpired on Wednesday & Thursday as well as the fact that every bacterial and viral lab test has come back negative, Evie has begun taking eculizumab known in the US as Soliris. Part of the reason for the forward leaning is because the doctors are keen on avoiding long term damage to Evie's ability to produce insulin. Amongst other things that you might find particularly interesting is that in 2010 Eculizumab earned the title of the most expensive medication on the planet--a title it continues to hold to this day. At this point, they don't believe she'll need to be on it for an extended period of time. I think I'll start calling it Lamborghini Juice, because that way I can feel as though in a way my daughter has helped me accomplish a boyhood dream sort of. So at this point, for those of you keeping score, they're fairly certain that we can safely diagnose/label at least part of what we're dealing with as what I referred to earlier as option 3. Its scientific name is atypical hemolytic uremic syndrome. That link is probably the best moderate yet simple discussion. If you want to go deeper, enjoy a few links:

• Shallow: 2.5 Minute YouTube Discussion
• Shallow: Medical Abstract
• Moderate: aHUS bootcamp
• Deep: Scholarly Reading

So interesting facts you'd pick up by reading this: There are about 300 cases are known in the US. If you had thought that Evie had bad luck when it comes to OI, consider that the odds of that are 7 in 100,000 worldwide. The odds of having aHUS are 1 in 500,000 in the US. So to wrap up, Evie has been diagnosed with:

1. OI
2. aHUS
3. kidney failure
4. pancreatitis -- complication from aHUS
5. myocarditis -- complication from aHUS

Pray for:

• No infections. She is anemic and is bruising at every site they have tried to stick her--her IV dance card has too many partners for too many songs as it is. You may also read that a brutal and common side effect of the Lamborghini Juice is serious meningococcus infections. We don't need that. (What we're taking/doing to avoid/resolve: Flagyl, Ceftriaxone, Vancomycin, Fluconazole, Amoxicillin)
   
• Speedy resolution to complications. We need the heart and pancreas issues to reach a quick happy end so that the poor girl, and her body can obtain food in a more natural fashion. Obviously additional complications with either organ would be most non-triumphant. (What we're taking/doing to handle/resolve: Heart:Milrinone infusion, Epinephrine infusion, Solumedrol (steroid), IVIG (Intravenous Immune Globulin). Pancreas:Insulin Infusion, Calcium Infusion.)
   
• Kidney function. Whatever kidney function we can get back is a score.
   
• No touchbacks. Once we get this thing "Resolved" let's swing for the fences and not have any recurrence/flareups.
   

I don't want to hear anyone complain to me that my post started out positive and became a downer. Just because it is on paper doesn't mean its depressing. We had a great day. We got many answers. We're enjoying Lamborghini Juice and there wasn't even a credit check--they just assume I'm good for it. :-)

I'll end on yet another positive note that I've been setting aside for a rainy day: I have nearly four hundred hours of leave that I have stored up like a squirrel that I can now use as necessary. I have already transitioned my workload back to my team, and can rest easy that work is taken care of. Our church congregation is taking care of us. Our neighbors are taking care of us. Our family is doing everything they can from a distance to take care of us. We are blessed.

Matthew 19:26 ~But Jesus beheld them, and said unto them, With men this is impossible; but with God all things are possible.

1 Nephi 17:50-51 ~ And I said unto them: If God had commanded me to do all things I could do them... And now, if the Lord has such great power, and has wrought so many miracles among the children of men, how is it that he cannot instruct me, that I should build a ship? BLESS US?

He already has. He will continue to do so.

DISCLAIMER: Posts Labeled "One Year Ago Today" are a record of what transpired when Evie first became ill.  The slightly edited text comes from emails which we sent to family to let them know what was happening, and to keep them updated.  These posts are usually long, but if you want to truly understand what life was like for us, and what led to this point, it makes for great "light" reading.