Tuesday, December 19, 2006

We counted twice and there were indeed two hundred million billion blankets on her in this picture.

























Everyone loves snuggling with the baby! Even cats!















No idea what she's looking at in this picture, but you just know it's gotta be fascinating.

























"Jonathan, is that you?"

"Yes, webpage, it is. How have things-"

"My goodness, I hardly recognized you! The last time I saw must have been, oh gosh, years ago."

"Okay, okay, i get it, i haven't visited or posted in a while, i'm sorry. I guess i just-"

"Oh no, that's fine, I understand. So many more important things to do in this world, I'm sure."

"Well, um. No. Not really."

"So would it be safe to say that you're just lazy, then?"

"Well, um. Yes. Pretty much."

And that about sums it up. I've just been in an almost vegetative state of laziness. If it would not cause the utter collapse of our lives, i'm pretty sure that Christine and i would seriously consider sitting at our computers all day and generating nothing productive whatsoever. I wish i were kidding.

There is news, and i will share it with you. Before i do, though, i want it to be known that there isn't much good or happy in any of it. I just wanted to put that out there, set it on the table, before i get started.

Where to begin? Much has happened in the weeks since i last posted, a lot of it not worth relating.

We were approved for overnight hospice care; a health professional to come spend the night with Tuesday so that Christine and i could attempt sleeping for the majority of an eight-hour period. The first week we got three nights in a row of sleep, wonderful sleep. The next week we got two, then two more, and now it's this week and we haven't heard anything yet but we're suspecting two more days. These nights of sleep are really wonderful, even though we're all on vacation from school at the moment and can, if we sleep in shifts, get a lot of Zs anyway, because it allows me and Christine to do something we were starting to miss and that's sleeping with each other. Tuesday seems to have backed off a bit from her previous sleep-all-day-then-party-all-night schedule, but she still cries pretty consistently, needs food every three hours and also just doesn't sleep for extended periods throughout random nights. I remember the third night we had the nurse over i came out in the morning to relieve her so she could go home and it was readily apparent that she'd had a hard night. "She wouldn't sleep all night, she just cried and cried and cried if I tried to put her down." Well, i thought to myself, that's why we needed help. Now perhaps you understand.

At the suggestion of her pediatrician, we scheduled an appointment with the cardiologist who had previously diagnosed Tuesday with pulmonary hypertension. Mostly we wanted to get a better idea of what kind of time frame we were looking at; we had little if any hopes of her condition having improved. We already knew that there was fluid in her lungs and that her heart was exhibiting an audible murmur, but we wanted to know if it would be worthwhile to look into getting her started on possible bottle feeding or getting her a stomach tube. If she's going to be with us for several months then we'd like her to experience things like suckling and not having a tube in her nose. The diagnosis was bad, essentially, but not necessarily worse than we were expecting. I don't honestly know what we were expecting.

The high pressure area of Tuesday's heart is much larger than it should be because it is having to work so hard. This is a sign of heart failure. Nor is that side functioning properly, there is some leakage occurring due to the abnormal stress on that area. She is not yet exhibiting what is called cor pulmonale, which is, from what i understood, the terminal stage of her condition. At least, not clinically, which would include an alarming swelling of the liver, which she does not have. On the other hand, she's not doing well either. Her breathing is labored, alarmingly so at times, and the cardiologist informed us at our visit that the pressure on the left side of her heart was 160 (i'm thinking millimeters of mercury since that's what i was told they used last time i asked). When she was diagnosed with her terminal condition the pressure was only 80.

In the midst of all that, she continues to grow and develop. She weighs twelve pounds six ounces. She loves wiggling around on the floor and having someone play with her feet. She likes putting her hand up to her mouth and tonguing it. She spends a great deal of her time hanging out in a swing that two wonderful and brilliant people got for her. And she continues to display new symptoms of her genetic disorder, the latest one being a hyperactive gag reflex. Which brings me to her new regimen of medications.

We first noticed the gagging when Christine went to put a pacifier, something for which Tuesday has shown a lot of enthusiasm, in the baby's mouth and instead of sucking like there was no tomorrow, Tuesday instead started to choke and gag. Our first thought was perhaps the feeding tube was in wrong since the last person to put it in was me and i may be many things but a medical professional isn't one of them. Her pediatrician said she doubted it, that more likely our baby simply gags more easily for reasons we may never know. So now she gets something called cimetidine, which is primarily for acid reflux. This helps to keep her from spitting up, which was our main concern since occasions of her doing so are amongst the most stressful and frightening that we go through. She'll go anywhere from one or two to several minutes without breathing consistently.

She is also getting lasix once a day to encourage her to get rid of more fluids. There is the possibility that her body might expel some of the stuff in her lungs and thus make her breathing easier. This is something that is completely at our discretion. We can give it to her if it seems to be helping or not give it to her if it doesn't. We're still on the fence at the moment.

Her pain medication has been switched from morphine, which is intended for short-term use, to methadone, which is in the same family (not structurally so much as functionally) as morphine. She gets a massive dose of the stuff once a day and that's it. And i mean massive. But it seems to be doing the trick. In fact, Tuesday is awake and making noises even as i write this. A week ago she might have been awake and crying or awake and screaming but almost certainly not awake and making contented noises unless someone was holding her. The going theory is that her body was simply too used to the morphine and it was losing its effectiveness while simultaneously agitating her, something that morphine can do to a person.

She's also still getting ativan, or lorazepam (drug names, sheesh), to help with anxiety but that's old news.

As we move into true winter we enter a zone of increased risk of infection for Tuesday. One of the body's main protection methods is the simple act of expelling air from our lungs, we force numerous microbes out of our bodies with every breath. Tuesday lacks the full potential of this mechanism, and her body is generally weaker than most people's. We were warned when we took her home that if she lived until winter we should prepare ourselves to lose her to something like pneumonia or the flu. How one prepares themselves for that i don't really know. Am i prepared for it? Probably not. I doubt i ever could be.

This update is officially over. I'll be more on my own case about posting so that you all don't have to be.