Milk and Molasses

I'm trying to write a nice, detailed summary of the week, but it's going to take longer than I had hoped, and I know many of you are waiting to hear more about how Tsvetomira is doing.

Today has been a very quiet day for her. She has spent most of the day sleeping and running a very low heartrate. Because all of her other vitals are looking good, and she *looks* good, no one is concerned by this. When I confirmed that a new seizure drug they started her on last night was at a high loading dose, and does indeed (like every seizure med, I believe) make you sleepy, it all makes sense.

Especially coming after a day full of excitement like Saturday was. I'll go over all of that in more detail at some point, too.

We met with a neurologist this afternoon, nicely at the same time that Matt, Owen, Bogomila, Gloria, and Rinnah stopped through for a visit. She learned from just the first little bit of Mira's VEEG (video electroencephalogram) that she shows all of the markers of Lennox-Gastaut syndrome, which is a severe, relatively rare form of epilepsy. Valproate, which is one of Mira's meds from the orphanage, is a common first choice medication for someone with Lennox-Gastaut syndrome. However, the Tegretol she had been taking is actually contraindicated in Lennox-Gastaut. Even though Adam was not working with the resource of an EEG, the combination of his assumptions from the blood work, and just his gut, he was spot on when he talked with us about dropping the Tegretol. (Score 1 for Adam!) The neurologist told us today that if Adam had not already had us stop the Tegretol, she would have had us drop it last night after seeing the first bits of Mira's EEG.

(I should note here, too, that Adam was very careful throughout all of his work with us to maintain his proper role as a nurse and not a doctor. He absolutely took the lead in all of Mira's medical care, but it was always done with careful consultation with me about what he was confident about, and where he was making his best guess, and together we worked to the conclusions that drove her care.)

Along with the confirmation that Tegretol was contraindicated, the neurologist recommended we start Mira on a different second seizure drug. This just happens to be a drug that we had tried (and hated with a passion) for five weeks or so with Reuben when he was first diagnosed with epilepsy six year ago, but is also one that Adam had been talking about with me on the plane ride home (during the boring part of the flight!) as he has had some very positive experiences with the drug. What beautiful timing.

Also noteworthy during the conversation with the neurologist was her reactions as I was describing what I was seeing in the ER yesterday that had me so very concerned. After I had started talking, she started asking me questions ahead of myself: was she arching backwards? (yes), were her cheeks very flushed? (yes), were her pupils dilated? (welll, no, but that's really the only one that didn't apply, and what she was looking for only requires four of six indicators to diagnose.) She began talking about something called dysautonomia. Dysautonomia occurs when the autonomic (or automatic) nervous system does not work properly, often due to injury to the brain. The autonomic nervous system controls body functions that we don't have to think about, such as heart rate, blood pressure, body temperature, and gut or bowel activity (to name a few!) Symptoms can be mild to severe. Mira's were definitely severe. What I was describing is what our neurologist calls a "dysautonomic storm." Treatment of dysautonomia is not one of curing as much as managing. If you're cold, get a blanket. If your bowels don't move well, develop a regular bowel program. But people with severe dysautonomia might require more active intervention (as per the hand out we were given). Mira's absolutely fit into that category.

She was experiencing a dysautonomic storm for nearly four hours yesterday afternoon. As you can apparently see, she lived through it. What began soon after Adam left, continued with increasing intensity and duration until about 7:50 when an EMT came to help me roll Mira up to Gillette, was a dysautonomic storm that was simply let to run its course. By the time we were in her room in the neurology unit at Gillette, she was slowing down. Still, rather than the "observation" she had been sent up for, even in her calming state, the wonderful doctor here took one look at her and told the nurse to not even bother with the admittance papers in front of her, because we'd need the one to admit her as an inpatient, and not the ones for observation that they had been prepared for.

And that's when I finally knew that someone was listening to me, and aware of the seriously complicated little girl we had here.

I'm guessing, too, that it's also the time when many of you had heard our various cries for help, and were lifting this tiny no-longer-an-orphan up before the Father who loves her more than an of us, because he MADE her. And with that, the storm was over.

And when he got into the boat, his disciples followed him. And behold, there arose a great storm on the sea, so that the boat was being swamped by the waves; but he was asleep.  And they went and woke him, saying, “Save us, Lord; we are perishing.” And he said to them, “Why are you afraid, O you of little faith?” Then he rose and rebuked the winds and the sea, and there was a great calm. And the men marveled, saying, “What sort of man is this, that even winds and sea obey him?” - Matthew 8:23-27

I longed for Adam as I pretty much had to start over with the history of what we knew and what we had been doing for the past week. And it wasn't just me - the doctor in charge of the floor last night also wanted to know what input he had for us, and as soon as his plane landed back home, she was talking with him, and sharing her thoughts, and he called me after they finished their call, and you could tell he was finally satisfied that Mira was in the hands of someone who would take care of her.

They are continuing her feeds using the formula we brought from Bulgaria, mixing up the feed with 140ml of pedialite, and 4 scoops of formula - exactly the dose we'd been giving her under Adam's guidance (that puts Adam up to 2 for 2). Even the pedialite was what he'd been wanting her on, and we had her on for a day and a half in Sofia, until her labs came back with her sodium going down again, so we switched back to Dad's hand-crafted saline. But guess what - they are also adding a dose of sodium to her pedialite mixture once a day, so now Adam's at 3 for 3. ;) She is on a pump, with the rate slowly increased from 10ml/hour up to 45ml/hour which has her getting pretty much exactly the same amount that we were giving her in a 24 hour period, but at an even more constant rate than we were able to manage on our own.

I am delighted by the relatively low-tech approach they're taking. Nice and slow, nothing unneccesary, but at the same time, nothing necessary is being left undone. Tomorrow should be another day of talking with some specialists, and making a plan for what should be done while she's inpatient, and what things can wait.

We still don't know if we're going home in a few days or a few weeks - partly depends on how they decide to feed her. They will not send me home with an ng tube, so we'll either need a j-tube and go home earlier (but have to come back as inpatient some time in the future), or a g-tube, and need to wait until she's stable enough for surgery, which is a very different kind of "stable" than where she is now.

And the milk and molasses? Well, in keeping with the very low-key stay we're having here (well, relatively!!) after the first try at an enema did not work; they came in with a bag full of, well, whole milk and molasses! That's it! Roto-rooter syle they go in until they can't anymore, and then the milk/molasses mix does its thing. And its thing it sure does! It's rather incredible that tiny little body had been holding that much volume of backed up stool, but I'm pretty sure she's going to feel better. Regardless, she's back to sleep, and I am so fatigued that I've to erase three nonsense sentence from this paragraph. My typing skills are good enough that my fingers can keep up with whatever flow of consciousness hits my semi-sleeping brain, and that's not really a good thing! I'd better quit. Looks like she's mostly done with the hard work of it, and they don't need me awake to change the final diaper when they think she's really done.

There's my girl - a tiny bit of her European formula on the left, and milk and molasses on the right!

So, tomorrow I will try to get more photos and more of the story up - this one is mostly just information, but hopefully helps some of you sleep easier, and pray more specifically.

Precious girl is still ours!!