Sunday, December 09, 2007

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?

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  • Wow.

    And it's gonna take a while to get over the bile in the fridge thing.

    By Anonymous Franki, At December 9, 2007 at 10:25 PM  

  • Wow, poor kid!!! But she sounds like a real trouper....I'm sure things will turn out good!!!
    My prayers are with her...

    By Blogger Odat, At December 10, 2007 at 5:33 AM  

  • P.S. you are sooo bad for trying to make her laugh! ;-)

    By Blogger Odat, At December 10, 2007 at 5:33 AM  

  • Being deeply squeamish, I confess to having skimmed a lot of the detail here, but I hope all is well soon enough.


    By Blogger Glamourpuss, At December 10, 2007 at 8:34 AM  

  • Crankstah,

    Thinking about you and Ella constantly. Wish I could give you and yours big hugs.


    By Anonymous Anonymous, At December 10, 2007 at 10:14 AM  

  • My heart goes out to all of you. This is all very reminiscent of my own experiences. A sibling with a life threatening condition growing up. A parent who died of gallbladder cancer which spread to the liver. The bile duct thing....the jaundice, weakness, pain.

    I am sorry all of you are going through this, particularly Ella, but I know it's not easy for you or your other sister either.

    Keeping you in my thoughts now and through the holidays. {Hug}

    By Blogger Echomouse, At December 10, 2007 at 11:21 AM  

  • How much trouble can she get into?? How much is there to get into?? :)
    I think it's fabulous that she's done so well and I hope that the miracles continue!!

    By Blogger Claudia, At December 10, 2007 at 3:31 PM  

  • Franki-
    It's been over twenty years, and I'm still not over it.

    She's pretty amazing. And, yes, I am truly evil for making her laugh. I honestly couldn't stop myself.

    I understand the squeamishness and deeply appreciate the warm thoughts.

    Thanks. I really appreciate it!

    In some ways, it's nice--it's bringing us all a little closer together.


    And I'm sure the miracles will continue. In this I have faith!

    By Blogger Crankster, At December 10, 2007 at 7:29 PM  

  • It's impossible to read this and not feel incredible sympathy. I hope Ella will prove to be one in a billion and astound us all with her long, long life.

    By Blogger SQT, At December 10, 2007 at 8:28 PM  

  • Oh wow! I'm sending positive thoughts her way--healing thoughts.

    Who knew you could put body fluids back in after they'd been sitting in the fridge next to the eggs?

    By Blogger Lex, At December 10, 2007 at 9:14 PM  

  • It seems Miss Ella is true to form and pushing the medical community on her behalf. Very nicely done. However, I'm still thinking positively for you all. Bile in the fridge? Limeade? ew.

    By Anonymous Anonymous, At December 11, 2007 at 12:09 AM  

  • thank you very much deary my yeger meister drinking days are over even before they began

    By Blogger Nosjunkie, At December 11, 2007 at 4:07 AM  

  • SQT-
    I have my hopes. Frankly, I'm betting on the kid!

    And thank you for dropping by!

    Thank you for the warm, healing thoughts. And, not only can you re-insert bile after it's been refrigerated, but it is much more refreshing that way!

    All the healing thoughts are greatly appreciated. I don't doubt that Ella will thrive, but every bit of positive energy helps!

    Trust me--you'll thank me for this one. Jager tastes like furniture polish. At least, it tastes like I'd imagine furniture polish tastes.

    By Blogger Crankster, At December 11, 2007 at 7:09 PM  

  • OK, I'll cut this short, you need me, you call me, I'll be there. capish?

    By Blogger The CEO, At December 11, 2007 at 10:58 PM  

  • I loved this piece.

    Good luck to you all.

    By Blogger Mystic Wing, At December 12, 2007 at 5:00 PM  

  • Love and prayers to Ella, hugs and supportive energy to the rest of the family.

    She is an amazing young woman.

    Wishing for the best possible outcome soon.

    By Blogger heartinsanfrancisco, At December 13, 2007 at 11:37 PM  

  • First time on your blog, and what a post to read.

    I work in project coordination for the operating rooms in a huge hospital that does a gazillion transplants a year, not to mention many experimental surgeries that they attempt in an effort to avoid transplant. There are so many inspiring stories out there of survival and resilience, and each and every one still touches me.

    I am constantly amazed at the leaps and bounds in surgical practice these days, and I hope that your sister becomes one of the many who try something new and win.

    By Blogger The Red, At December 14, 2007 at 4:55 PM  

  • CEO-
    Capisco. Grazie.

    Mystic Wing-
    Thanks. I think we'll be okay.

    Thank you for the warm thoughts and wishes.

    The Red-
    Thank you so much--not just for dropping in, but also for your thoughtful words. I think that Ella will, as always, amaze and inspire us. She's been a miracle for so long that we seem to have forgotten how amazing she is. This is a mistake we are not going to repeat.

    Again, thank you for the warm and kind thoughts!

    By Blogger Crankster, At December 15, 2007 at 11:06 PM  

  • Wow. My money is on Ella kicking ass and taking names and continuing to be a medical miracle!

    By Blogger JamieSmitten, At December 16, 2007 at 11:24 PM  

  • Jamiesmitten-
    A wise bet, methinks!

    By Blogger Crankster, At December 17, 2007 at 9:16 PM  

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