How My Son Got A New Liver: Diagnosis (Part 2)

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.

Pip's Diagnosis (Part 2)

So, very early on Tuesday morning, the day after Labor Day 2008, I left Bunny with a neighbor, told her I’d be there to get her in the afternoon, and headed with Pip back to Children’s Memorial.

I held him still again while he was stuck with needles and then I drove him over to an annex for an ultrasound.  There, I held him still while a woman rubbed a wand over his tummy and clicked and rubbed and clicked.  At one point she left and then came back.  Then a doctor seemed to burst in.  He said “Can you find it?”  She said “No.”  He said “Is it just hidden?”  She said “No…I don’t think so.”

At this point she looked over at me and saw that I was staring at them with my mouth hanging open…possibly with a little drool escaping one side.  She said “Don’t worry, Mom.  We’re just talking about different veins and things.  Some stuff is hard to see on a little guy.  No big deal.”

Sure…OK.

With five hours until our next appointment, Pip and I drove to a nearby Starbucks.  We sat until it felt like loitering and then walked over to The Children’s Place.  I bought Bunny two cute little dresses with matching bloomers that were on sale.  She would be starting preschool for the first time in a few days.  She needed to look spiffy.

It felt like a cloud was following me wherever I went.  It felt as if being a normal person doing normal things like thinking about what a preschooler would wear on her first day of school was something I was just pretending to do for show.

My husband had asked if he should come with us that day but I said no.  I had come to terms with what I believed would be the worst case scenario and would be able to be strong and tear-free if I heard it.  He had just started a new job.  He needed to be there.  He could take the day if Pip needed surgery – but not now.  That was my logic.  It made sense at the time.

The visit with the Hepatologist was pretty short, really.  I liked him instantly.  He was a big man with white hair and large hands and he picked up Pip and talked to him in a jovial way that made me feel comfortable.  He felt Pip’s liver for a moment and asked me if his poops were white (well, "clay-colored" is what they say but they mean off-white.)

I said that they aren’t.  They’re mustard yellow - like all breastfed infants.  Normal.  Normal!  I insisted.  I even had one with me and I showed him.  (Because they don’t let you toss poopy diapers in the hospital trashcans…not cause I’m a fecal freak or something.  I don’t normally carry poop around.  Honest.)

He said “Uh huh”

Oh, man.  When I looked at it through his eyes, it was clearly not right.  Still, I stuck to my guns.

I said “Well….they’re usually much darker.”

He said, “Yeah….if I had a nickel for every mom who said the poop was usually much darker….seriously….this is not yellow.”

In my defense, it actually was somewhat yellow…but it was being tinted by my prenatal vitamins.  Those big ole horse pills that turn your pee glow-in-the-dark neon?  Yeah.  Those.

Another moment of layman science from a theater major:
The color of normal poop has a wide range but the general rule is this:  if your poop is a color you can find in nature in the autumn, you’re good.  There are three bad colors.

Bright red means you have lower GI bleeding.
Black means you have upper GI bleeding.
White means you have no bile.
Any of those colors mean you should see a doctor.
Science out.

Back to our story.  The Hepatologist handed Pip to me, sat down in a chair across from us, and said “OK, listen, I still have more tests to do but I am 99.9% sure that your son has Biliary Atresia…

(OK. I had heard of that one.   It was the worst-case-scenario one.  He was gonna tell me Pip would need to have a blockage removed.  Surgery.  I was ready.  I could take it.)

…which is an extremely rare, fatal, liver disease for which there is no cure.”

WHAT?!

CRASH.

My entire world.

CRASH.

He said other things but it was hard to hear over my own heartbeat.  I was able to take in at least some of the information but I couldn’t quote him on any of it now.  The room was spinning too fast.

Pip was already on the schedule for a Kasai portoenterostomy that could possibly extend the usefulness of his native liver.

The procedure had a 33% success rate and was more likely to be successful if the infant was less than 8 weeks old.  Pip was 9 and a half weeks old at that point.  We're a little late with the diagnosis.  I made some sort of gutteral noise at this.  He said, "This disease is rare.  Why did your pediatrician not catch it sooner?  Because this is probably the one case she will see in her career....most will never see it at all.  She's probably a great pediatrician but this is new territory for her."

It had a 33% partial success rate (meaning it would result in some small amount of bile drainage and the child might get past two years of age before needing a transplant.)

And it had a 33% abject failure rate.  I added the word "abject."  I don’t think he said it.  I just felt it.

He said that regardless of whether the Kasai was a success or a failure, about 98% of infants with Biliary Atresia will need a liver transplant at some point in their life– most of them before the age of two.

CRASH.

I had never known a single child in the entirety of the universe to receive a transplant of any kind.  I could not imagine that the life of a child who had undergone a transplant would be a happy or healthy one.  I was unclear at that moment what my worst-case scenario truly was.  Would the worst case be death or would the worst case be a prolonged existence in a broken body?  I felt helpless and hopeless and terrified and alone.  He left the room to see if the results of the ultrasound were back yet – handing me a box of tissues as he exited.

I called my husband and said something along the lines of “It’s bad.  They say he needs a transplant.  It’s bad.  Come now.”  I made the same phone call to my sister who was just finishing up cleaning out the apartment of our father who had died six weeks earlier.  When it rains, it pours, you know.  She said she’d be hopping the first flight she could get to.  My husband somehow managed to intercept us as we were being escorted from the clinic room down to admitting.  To this day I don’t see how that is possible but he did.  I was in a bleary-eyed daze – clutching Pip to my chest and shuffling numbly down a hallway after a nurse.  He must have broken the sound barrier getting there - which could not have been easy in a Chevy Cavalier.

More from The Artist’s Guide to Sciencey Stuff:
Biliary Atresia affects one in every 20,000 or so infants.  It is not genetic and has never been known to affect the same family twice except in one case of twins who were both born with it and are also treated at Children’s in Chicago.  It is quite rare so having enough kids with it to do research is a challenge.  The most popular theory behind the cause of BA is that a fetus or a neonate with a still-immature immune system is exposed to a common virus.  The immune system attacks and kills the virus and then mistakes the cells of the bile ducts in the liver for more of the virus and attacks those cells as well.  It generally starts at the gall bladder and moves in – turning everything into scar tissue as it goes.  Once the immune system decides that those cells are the enemy, you can’t call it off.  The scar tissue stops the bile from draining from the liver.  The bile backs up into the liver, further damaging it.

We checked into the hospital and were given a room and then my husband left again to get Bunny from our neighbor.  It was her bedtime by then.

Bunny!  I had told her I’d be home in the afternoon and I'd get her.  I had told her everything was fine.

I had lied.  Nothing was fine at all.

It truly didn’t feel like anything would be fine ever again.

 

Tomorrow: Baby's First Surgery

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

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