The sun is shining, the birds are singing, the weather is perfect, and Annika has developed hepatic encephalopathy.
Hepatic encephalopathy is such a fun word to say! And I especially love that, after you practice saying it in your head maybe 82 times or so, you still stumble over it when you pull it out in the course of casual conversation. But once you have it down, it's a great word to drop at the first sign of altered mental status. Pretty soon, you find yourself using it in a totally insensitive and inappropriate fashion, which just goes to show that you are totally owning liver disease. Your husband tries to convince you that covering the front yard over with pebbles is a reasonable solution to end the twice-weekly lawn-mowing grind? Your response: "What?! Are you encephalopathic!" If you want to sound really hip (hip-atic!), you might shorten it to "You are such an encephalopath." Always good for a chuckle. (But be careful! In the adjectival form the stress shifts forward one syllable - encephaloPATHic - you don't want to ruin your punchline with an awkward delivery!)
So Annika took two naps today. After 12 hours of sleep last night. And she will just fall asleep in the strangest way: like she'll be sitting in a chair, watching me blow up the wading pool, and then she'll just be asleep, bam! She's also saying weirder stuff than usual: incomprehensible, mumbly stuff. And when we ask her about it, she'll try to repeat herself, then stop and look confused, finally just saying, "Oh. Never mind."
She's not like that all the time. She has long stretches of time during the day when she seems perfectly normal. But I think that her non-normal stretches are becoming more frequent. At least I'm certain she's sleeping more and more. It's always hard to tell on the weirdness end of things, because Annika is a tiny bit on the weird side almost all of the time, which is, of course, one of her most endearing qualities.
Hepatic encephalopathy, if you haven't gathered as much yet, is when you go a bit cuckoo thanks to liver disease. One of the functions of the liver is to filter toxins out of the blood, and if your liver is impaired by cirrhosis or if a good portion of your blood supply is bypassing your liver (hello, spleno-renal shunt!) then those toxins remain in your blood supply, where they can eventually pass through the blood-brain barrier, causing neurological symptoms. Ironically enough, hepatic encephalopathy can often be mistaken for intoxication. ("Whoops! You mean I wasn't supposed to let my 5-year-old with a liver transplant have that Pabst Blue Ribbon at the BBQ? OK, but that wasn't in any of the discharge orders.")
So she'll have an ammonia level drawn with her next set of labs to see if she needs to start taking drugs to reduce the amount of ammonia in her system. Not all cases of encephalopathy are accompanied by a high ammonia level, and it's not exactly the easiest test to get accurate results for: there are all sorts of rules for how it has to be drawn and handled at the lab, like no tourniquets should be used and the lab tech should be wearing only green and the patient needs to tilt her head to the left and repeat the Pledge of Allegiance during the needle poke. With a needle fashioned from a bone sliver of the rare golden-headed langur of Vietnam.
If the ammonia level comes back high, then she might need to start taking lactulose, which many pre-liver-transplant parents know as the "I scoff at you, Pampers!" drug. Yes, it causes diarrhea, with a capital "D." But at least it might stop Annika from weeping for hours because she spotted a cracker in her bowl that wasn't perfect and why can't everything be perfect? Why? Why?!
Hepatic encephalopathy is a perfect example of all that's wrong with googling your child's medical diagnosis. The first hit that comes up, once you spell the word correctly which is almost as hard as saying it correctly, is this little jewel from the National Library of Medicine and the National Institutes of Health. This article shares with you some of the symptoms of encephalopathy, including forgetfulness and coma . Plus it offers up the fascinating tidbit that encephalopathy-induced coma is 80% fatal. What might be missed, however, is that encephalopathy associated with fulminant hepatic failure, when your liver fails suddenly and catastrophically, is much more dangerous than when you have a kid like Annika, who is chugging along with her normal 5-year-old life, slowed a bit by the sputters of a liver that's taken quite a hit this past year, but chugging along nonetheless.
Don't get me wrong. It's no fun seeing lethargy and an altered personality in your preschooler. After all, for me it's not the workings of Annika's liver or her lungs or her heart that make her Annika. It's the amazing and wondrous workings of her mind that make her my own child, the one I know and love. Luckily, our transplant coordinator in Chicago told me that encephalopathy in a child like Anni can wax and wane. And, anyway, we now get to hear knock-knock jokes from an encephalopathic 5-year-old. It's like being the one responsible for holding the wallets at Glastonbury, but with less body odor and better toilets. And much more Billy Joel.