How My Son Got A New Liver: Baby's First Portoenterostomy

April is National Donate Life Month and Bunny, Pip, and I want you to be very aware because without organ donation and transplant, Pip wouldn't be with us today.

More than 115,000 men, women, and children need life-saving organ transplants right now and every 10 minutes, another name is added to the list. An average of 18 people die each day waiting for organs.

This month, I'm gonna tell Pip's story - a little bit every day. We need to improve these statistics. Learning more and talking more and sharing more is the first step.

Baby's First Portoenterostomy

They called in a chaplain to sit with me that first night after the diagnosis.

I’m not religious and I didn’t ask for one but I’m not sure they quite knew what to do with me.  I was pretty inconsolable, I guess.  I actually remember that evening very strangely – probably inaccurately.  I remember sitting in a chair in a very dark room with only a bit of ambient light from the hall seeping in, making a rectangular patch on the floor…dimly illuminating half the chaplain’s face.  The truth is, a pediatric hospital never gets all that dark - but I was certainly in a dark, dark place nonetheless.

My mother had died suddenly a year and a half earlier.  That was followed in rapid succession by two miscarriages and my dad’s lung cancer diagnosis and then his death three weeks after Pip was born.  It had been a rough couple years.  But nothing even remotely compared to Pip's diagnosis.  Or maybe the horror of his diagnosis was compounded by the tragedies of the previous year and a half.  Either way, I found myself in a pit that night and I couldn’t climb out and it seemed to grow deeper by the minute.  I could not wrap my mind around what was happening.  I wanted to wake up and find out I was dreaming.  I wanted to rewind.  I wanted to grab him and run and forget what I had heard so that it would all go away because while they kept talking doomsday, that bright-eyed, happy baby was smiling and trying to eat my hair.   Maybe they were making him sick.

I wanted to know how in the world something like this happens to US.  This is a story you hear on a radiothon.  This is a story you listen to during your morning commute and say “Tsk tsk.  That is so awful.  That poor family.”  And maybe, if you remember, you send money and you count yourself lucky that you are not a person these things happen to.

It is a mind-blowing, life-changing moment when you find out that you are, in fact, a person these things happen to.  Because we all are.  All of us.  Yes...even you.  Your world can change on a dime and, at some point, it probably will.  You are more likely to be struck by lightning than to be born with this disease but Pip happened to be the one this time.

Random.  The world is so random.

But daylight eventually comes even when you are sleepless in a green hospital chair questioning the nature of the universe and cursing the seemingly endless night.  And with it, a baby who has absolutely no idea where he is or why such a fuss is being made, wakes up, looks around for his mama, and gives her a big, toothless grin.  And you step out of the pit.  What other choice do you have?

If there is a 99.9% chance that your child has a fatal liver disease, then there is a .1% chance he doesn’t.  Parents will hold out for that .1%.  That’s what makes us so special and it’s why you hear about those moms who perform extraordinary feats of strength like picking up entire automobiles to pull their child from underneath.  We believe we can defy the odds.

So that was the focus that following morning.  Not the threat of a nearly inevitable transplant because that notion was simply too much to digest.  The task at hand was to hope that the scheduled biopsy would reveal that the problem with his liver was far less devastating than Biliary Atresia.  And when the results came back and it still looked like Biliary Atresia, the task was to wait for the tiny chance that they would open him up and find a healthy, intact gall bladder and they would shoot dye into it and the dye would flush through the biliary tree and prove that it was not Biliary Atresia after all.  Because that’s the only way to be 100% sure and there was a chance that Pip was the .1%.

Kasai Portoenterostomy For Dummies
A Kasai Portoenterostomy is a procedure that is used to prolong the usefulness of the native liver of a child who has been diagnosed with Biliary Atresia.  It has been around since the 50’s and no one has much improved upon it even though the success is really fairly limited.  The procedure removes the gallbladder which has generally pretty much been destroyed by the disease and cuts away the damaged part of the liver until healthy bile ducts are found through which bile can flow in the direction nature intended.  A segment of small intestine is removed and one end is sewn to the liver so that the bile can drain into it and the other end is sewn back into the small intestine (forming a “y” shape) so that a new drain is created to replace the gall bladder.
BAM

So, Tuesday afternoon Pip was admitted and Wednesday morning was the biopsy and Thursday morning I handed him to an anesthesiologist who carried him into surgery.  He turned 10 weeks old that day.  He didn’t cry when the doctor took him and carried him through those double doors.  I did, of course, but he just looked at me sort of quizzically from around the man’s arm as he walked away.   My chest hurts when I think about it.

My husband and I went and sat on a bench in the lobby of the old Children’s Memorial Hospital that is no longer there.  We sat there, numbly staring straight ahead, waiting for a phone call that would let us know, after they opened him up and injected the dye into his gallbladder, if he was the .1%.  We sat…and we sat…without speaking…which is how we both noticed, simultaneously, that one of the nurses who was involved in his surgery was walking out of the hospital.

She saw us see her and circled back.

She said, “Pip is doing well.  Someone came in to relieve me but he’s still there and the Kasai is underway.”

I said, “It's underway?  No one called.  So it’s for sure Biliary Atresia?  The dye didn’t move when it was injected into his gallbladder?”

She said, “Oh, they didn’t even bother injecting the dye.  There was no gallbladder to speak of – just scar tissue.  So they started on the Kasai immediately.”

Oh…well…there it is.  Pip was not the .1%.

OH!  The ultrasound!

The doctor had said, “Can you find it?”  She said “No.”  He said “Is it just hidden?”  She said “No…I don’t think so.”

His gallbladder.

OHHHHHHH…….

The Kasai went well.  They were able to find some decent bile ducts that hadn’t been affected yet and Pip was strong and stable.  He recovered well and five days later I took him home – smiley and wiggly – with a huge incision that stretched halfway across his abdomen that was stitched up with thick, black thread and a hole in his side that shot copious amounts of a sticky, yellow fluid out his side whenever he moved and a bag full of medications and vitamin supplements.

I took him home to wait.  It takes three months to determine the success of a Kasai.  You wait for the swelling to subside and you wait for signs that the bile is starting to drain and it can take a while.

I took him home and we tried to behave like regular people and give our children a normal life while watching and waiting for one of our son's vital organs to start functioning properly...or stop functioning at all.

That’s easier said than done, ya’ll.

 

Tomorrow: Waiting

To learn more about organ donation and to make sure you're on the registry for your state, visit www.donatelife.net

Wanna get an email alert when a new post comes out? Put your email address in the box below and create an amazing and affordable (free!) blog subscription that is spam-free and that you can cancel at any time.

 

 

Leave a comment