Details...

I think I'm pretty good at writing about the facts and details complete with a bit of emotion, but not recently...for that I apologize.   The facts and details are not as easy to process and understand, and as such I am struggling to write sentences that make sense of all of this.

Lucy has ascending colangitis again, a serious potentially life threatening infection in her common bile duct. Ascending colangitis occurs when bacteria from the small intestine ascend into the common bile duct.  An infection tends to occur if there is a partial obstruction in the bile duct.  Lucy's common bile duct is obstructed again, most likely from sludge and stones coming from her diseased gallbladder and deteriorating biliary system.

Despite having and ERCP preformed a month ago to explore and clean out her common bile duct(CBD), things are backed up again.  An ultrasound preformed on our day of admission showed that Lucy's bile duct looks similar to what it looked like prior to her having her ERCP.  When we first discovered this finding back in November Lucy underwent an MRCP to take a closer look at her common bile duct area to help us confirm that there was an obstruction, and if possible help us determine the cause.  The MRCP confirmed that yes indeed there was an obstruction thus causing a stricture in her CBD, but why, that was still unknown.  ERCP's are the gold standard now a days for exploring and removing debris from a CBD, therefore Lucy underwent an ERCP to do just that.

The GI doctor that preformed the ERCP at Jefferson Hospital in Philadelphia discovered lots of sludge, stones, and "crap" (his words not mine) in Lucy's bile duct thus causing the stricture in her CBD.  While cleaning out her bile duct they also dilated the stricture to allow bile to once again drain into the small intestine.  Originally we all thought that he was going to place a stent in her CBD to help keep it dilated, but he opted against doing this.  The reason being was that the stricture in Lucy's CBD was not as a result of a tight muscle, in fact he found her CBD to be extremely floppy, the floppiest he had ever seen.  He felt confident that the dilation would hold, and opted against placing the stent thus saving Lucy from having to have a repeat ERCP to remove the stent, as stents are only temporary.

We also learned through all of this that Lucy's common bile duct is grossly enlarged, a very uncommon finding.  It is believed that the enlargement is due to the fact that she has bile duct dsymotility, a rare but not uncommon finding in an individual with severe gastrointestinal dysmotility, aka Lucy.  Not unlike her intestinal dysmotility issues, Lucy's common bile duct does not move bile through it causing bile to sit and collect inside the bile duct.  This leads to a build up of thick sludgy bile, that then forms an obstruction in the CBD,  thus allowing bacteria to be trapped which can then cause serious life threatening infections.

The bacteria that gets trapped in the common bile duct comes from Lucy's diseased small intestine, which we know is full of every type of bad bacteria and then some.  Lucy continues to be febrile despite being on multiple broad spectrum antibiotics, which we reffer to us as the "big guns".  We are fearful that Lucy could be growing a resistant bacteria which would mean that no matter how big the gun we just won't be able to shoot down the bacteria.  Ultimately, we will not know if Lucy will get better from this infection until we clean out her CBD helping to rid her body of the infection.

Preforming a cholecystectomy, removing Lucy's diseased gallbladder, will help to eliminate some of the sludge and any more stones from getting trapped inside her bile duct that are contributing to the obstructions.  This needs to be done, but first we need to do some more exploration.  We need to take a look at what is causing her current obstruction in CBD, and then move forward with cleaning out her CBD before removing her gallbladder.   Talks are being had with the GI doc at Jefferson, Dr. R, and our surgeons here to discuss the best way(s) to go about doing all of this.   Simply removing Lucy's gallbladder is not going to fix her issues, it will help, but ultimately as long as bile stays stagnant inside her CBD Lucy will continue to be at risk for ascending colangitits.

Here is where it gets trickier.  Our girl is sick, she has an underlying disease that we can not cure.  In Lucy's four short years of life we have intervened and provided support for the areas of Lucy's body that have needed us to support it.  A g-tube for feeds, which lead to a gj-tube, which then lead to a central line for IV nutrition and meds....and the list goes on and on.  Everything that we have done for Lucy we did to help provide her the best longest life!  The question that we are ultimately faced with is how far do we go?  At what point do we stop trying to correct the problems and allow the body to do what it will ultimately do when we stop intervening and treating every problem?

This week we have had some of the most difficult discussions of our lifetime with Lucy's doctors.  If we do nothing more to correct these problems for Lucy her "quality of life" will be very poor in our opinion as she will most likely stay the way that she is currently until the end of her life here on earth.  However, we have hope that by attempting to correct her current obstruction and fixing some of the issues contributing to her recurrent obstructions in her CBD we can get Lucy well enough that we will again see Lucy's personality shine.  This is ultimately what makes Lucy Lucy, not the physical condition of her body but rather her spirit that shines through it.