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We think it’s Biliary Atresia, but that’s really bad so hopefully that’s not it… Part Three

posted Apr 19, 2011, 7:59 PM by Becca K.   [ updated Jan 6, 2012, 6:07 AM ]

At the time of Natalie’s first surgeries, we had not heard of www.caringbridge.com (the site of her first webpage), Facebook was not around, and MySpace was a name I called my bedroom.   What I am saying is that I did not document my thoughts and feelings at that time, the way that I do now.  Not that you would have wanted me to.  You see, for the first 3 weeks of Natalie’s life, we lived in our home and visited our baby in the NICU.  I cried every time I left her, which was every evening and every shift change.  I’d start crying in the elevator leaving the NICU and finish up half an hour later as we pulled into our driveway.

We did not know in the beginning that Biliary Atresia would be Natalie’s final diagnosis.  She began her stay in the NICU under the UV lights, like any other jaundice case.  We’d work on “normal” things, like trying to get her to eat.  The first day it was 15 ml every 3 hours, then 20ml, then we finally worked up to a whopping 30 ml.  I was encouraged, cheered on even, to keep breastfeeding.  And so I did.  It gave me a purpose.  A sense of control.  And when you can’t control anything, you’ll control that one thing with everything you’ve got. 

I took to it like a champ.  Strike that.  I took to pumping like a champion dairy cow.  I focused on finding ways to increase my milk production levels.  I found special teas marketed themselves as “Mother’s Milk tea.”  My loving husband was my biggest advocate.  We’d walk down a long hall, into some section of the hospital that was no longer in use, except for the rare nursing mother pumping session.  There was a room with 3 old chairs, a sink, paper towels and soap (for keeping supplies clean) and outlet.  Not much more, except for some posters donated by a La Leche League USA.  I’d plug my Medela Pump into the wall, take a seat on the old metal chair that was missing chunks of vinyl on the seat, and watch the milk rise in the bottles, feeling victorious as the ounces would climb higher and higher, knowing this was for my girl.  The aches I felt, sitting hunched over, were worth it.  This was for Natalie.  This was making her stronger, this I could do.

After a few days, they did a full blood work-up.  Her GGT level was around 1700 (normal is 5 - 55), letting the doctors know that something else was wrong. 

They’d take her for an ultrasound.  “Inconclusive.”  She was born with a gallbladder, after all, and was stumping them.  After this happened 3 times, she had a HIDA scan, and then an open cholangiogram and also a biopsy all on October 13, 2003. 

From Wikipedia:

Cholangiography is the imaging of the bile duct (also known as the biliary tree) by x-rays. There are at least two kinds of cholangiography:

  • percutaneous transhepatic cholangiography (PTC): Examination of liver and bile ducts by x-rays. This is accomplished by the insertion of a thin needle into the liver carrying a contrast medium to help to see blockage in liver and bile ducts.

·         endoscopic retrograde choledochography (ERC). -  is a technique that combines the use of endoscopy and fluoroscopy to diagnose and treat certain problems of the biliary or pancreatic ductal systems. Through the endoscope, the physician can see the inside of the stomach and duodenum, and inject dyes into the ducts in the biliary tree and pancreas so they can be seen on x-rays.

In both cases fluorescent fluids are used to create contrasts that make the diagnosis possible. Cholangiography has largely replaced the previously used method of intravenous cholangiography (IVC).

That’s when Dr. Rettig met with us and drew on a paper towel what we were looking at. 

 

 
He had opened Natalie up, injected dye, and then tilted the table to watch the flow of the dye.  And that’s when he said that he hoped that this wasn’t Biliary Atresia.  He and Dr. Burress, the Pediatric GI attending to us in the NICU, both referred us to a wonderful team of doctors at Children’s Memorial in Chicago.  And in the meantime, the biopsy slides were sent to Mayo clinic.

Mayo’s answer came back “Biliary Atresia.”  But all other local doctors disagreed.  So we got the slides back and took them with us to Children’s Memorial.

When we arrived in Chicago, it was like no place we’d ever been.  There were bright colors everywhere. There were multiple waiting rooms with lively fish swimming in tanks.  We were escorted to an examination room on the first floor.  We thought that we must have been in the wrong place.  There were no plaques or diplomas oh the wall.  We met Dr. Whittington and Dr. Kohli and told them we’d hear the term “breastfeeding jaundice.”  Dr. Whittington (awesome doc at Children’s that we’d just met) gave me a look like I’d just crawled out of a cave.  “That is a myth.  We’ve disproven it.”  OK, I thought.  Not going down THAT road with him.  I had just had my first lecture by a genius, and I wasn’t a fan of lectures.  But it let me know that we were in the right place.  They knew their stuff.  And they forbid me to feel guilty.

Dr. Kohli took the slides and reviewed them.  He asked Dr. Wittington to review them.  Their first review said that she may have Cystic Fibrosis.  They said it just did not present like “typical biliary atresia.”  Sweat tests were done = “negative” was the answer.  Genetic tests were sent away, and those took 6 weeks to get back.  6 long weeks of desperate waiting.  The results came back negative.

After we had left the NICU, we waited for the other shoe to drop.  We were still waiting.  We waited for the bad news that we knew would come.  Every inconclusive answer left me feeling more and more frantic.  Dr. Whittington had told us that for a Kasai Procedure, the procedure to treat Biliary Atresia, to be most successful it must be done by the time the child is 12 weeks old. 

The clock kept ticking.

In the meantime, we had her baptism blessed in the Church.  She’d already received the sacrament of baptism in the NICU, so Father (now Bishop) Timothy Doherty blessed Natalie on December 14, 2003 within the walls of St. Mary’s.  I cried.  It’s what I do. 

Just a few days later we returned to Children’s and during a follow-up exam, Natalie happened to have a dirty diaper.  Dr. Whittington took one look at it and re-diagnosed her with Biliary Atresia. 

Who knew the answer was in the poop?

She was admitted, and had another biopsy, it was again inconclusive.  The kid is consistent.  She then had another cholangiogram, this time a percutaneous type, which was then followed by a Kasai Operation on December 19, 2003. 

Her surgeon, Dr. Superina, called her’s a case of “correctable Biliary Atresia.”  We’d learn later that things are not always what they seem, and rarely are they as simple sounding as something called “correctable.”  But, for the moment, we had an answer. 

She got to come home on Christmas Eve. 

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