Everyday Miracle

Well, I'm back from Pennsylvania. When I got to Lewisburg on Friday, I quickly visited Ella at the Geisinger Medical Center, where she was staying. She was pretty energetic, given the fact that she was sporting a tube that entered her upper abdomen, threaded behind her abs, and punctured the left-hand side of her liver. This tube was attached to an equally nifty ostomy bag, which was collecting her bile.

Bile, by the way, is an interesting yellowish-green color. It looks a little like Jagermeister.

By the time I got there, Ella was roughly Lisa Simpson yellow, and the "whites" of her eyes were sort of a canary color. Her bilirubin had dropped from 9.5 to around 7 or so. 1.5 is normal.

Ella was also having a hard time dealing with her body's failure. She was trying very hard to be brave and strong, but this is a lot to deal with at 24. At times, she was unable to sit down, take a shower, or even walk without help. It was incredibly frustrating, and she clearly felt betrayed.

I was having flashbacks to the last time this happened. Ella was about 12 weeks old, and had been diagnosed with Biliary Atresia, a condition in which her connective bile duct was severely blocked. Her bile backed up, causing cirrosis, a deterioration of her liver and duct tissue.

Ella ended up having a Kasai procedure, which was somewhat experimental at the time. In this operation, the surgeon cut away at her liver until he found bile ducts that hadn't been damaged by the blockage and subsequent bile backup. He then brought part of the liver and small intestine outside the body. We used to collect Ella's bile and drain it back into her body. Sometimes we would not do this immediately, and my mother would store the bile in the fridge. Often, my sisters and I almost would almost drink a glass of green stuff, only to realize that it wasn't limeade. We learned to be really careful.

Ultimately, the doctors connected Ella's liver to her intestine. This was intended to be a short-term solution, used to keep her going until she could get a transplant. However, the temporary fix held and Ella thrived.

For 24 years.

When we were kids, our parents often reminded us that Ella's survival was miraculous, that she was "one in a million." Ella had a scar all the way across her belly and a slightly smaller one below it. We would often look at her "cross-stitch" and try to remember the days when she had nearly died. It was difficult, because Ella was anything but frail. Simply put, she got into the same trouble as any other kid, and passed her medical checkups with flying colors. She thrived through her childhood, the death of our parents, high school, and college. She double majored in Sculpture and Painting and Printmaking, earning a Summa Cum Laude in both majors. She went on to a graduate assistantship at Bucknell, where she's been for the last year.

As it turned out, Ella wasn't one in a million. She was more like one in six billion. Simply speaking, kids with Kasai procedures usually need transplants within a couple of years. Kasai livers just don't last into the twenties.

We forgot about this, only to be reminded last week. The left side of Ella's liver is currently blocked. The tube is draining out the bile, but she's going to need a permanent solution. The next step is to explore the bile duct on the left side of her liver in search of the blockage. If that doesn't work, Ella will probably need a "Kasai revision," which involves clearing out her bile ducts directly and reattaching them to her small intestine.

At this point, everything is somewhat experimental. Revisions aren't generally done on 24-year old Kasais, as 24-year Kasais don't generally exist. Further, bile duct explorations aren't usually done on scarred, 24-year old cirrhotic bile ducts, as these are also incredibly rare. Whatever happens next, it will be somewhat revolutionary.

The Gastroenterologist is trying to determine the chances that the exploration will be successful. If it looks good, we'll pursue it after the holidays. If not, we'll start looking into the Kasai revision.

Ella's spirits are high, although the pain creeps up on her, especially since she's trying to avoid hitting the Percoset too hard. Laughter can be particularly tough, as it irritates her abdominal muscles. Ysterday, after we got her home, she tried to keep the pain at bay with some over-the-counter meds. It didn't work too well, and she got pretty ticked at me for making her laugh. I offered to discuss the Holocaust with her, but she gave me a steely glare.

By the time I left this morning, she'd gotten her pain management under control. I'm counting the days before she starts to chafe at having to lie on a couch. I give her about half a week. The doctors have told her that she can do pretty much whatever her body will handle, but that she should be careful to avoid overdoing it.

I think that "overdoing it" might have been printed under Ella's picture in her high school yearbook. To put it mildly, the kid tends to live on the edge. However, with her sisters and I all hovering nearby, I think she'll try to keep it under control. Besides, it's only a couple of weeks before she'll be up here for Christmas. How much trouble could she get into in a couple of weeks?