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Moving Forward...

February 22, 2013
Lucy is currently stable, and believe it or not a plan is falling into place.  Lucy girl, now that I have just written these words please don't go and change things on us.   We hoped and prayed that Lucy would "come back" to the point where she is currently.  She is at the point where we feel that we can consider all of our options, and weigh the risks and the benefits of each with the knowledge that Lucy is probably as good as she is going to get.

In case you don't already know, our girl has a God-awful disease!  Having mitochondrial disease is like chasing a moving target, you never know when or where it is going to strike.  It robs areas of the body of the essential energy it needs to run effectively, repeating this process over and over until eventually organ failure develops, and the life of the affected individual is compromised.  We can't stop the disease process in Lucy, nor can we slow it down...there simply is no cure for mito.   Throughout Lucy's life we have medically intervened with everything that we could to support the areas of her body that the disease has affected, all in the effort to give her "the best longest life".  This being said, our options for our girl are running out.

Lucy's entire gastrointestinal system is failing, from her biliary system all the way down to her colon.  None of it functions, none-of-it!  We have supported these failures in Lucy by providing her with IV medicines and nutrition in the from of TPN.  We understood the risks involved with TPN when we made the decision to start it for her several years ago.  We believed that by supporting her body this way we were going to be able to provide Lucy with great quality of life...we have done this for our girl, given her great quality of life! Unfortunately, with this decision came the knowledge that liver disease and life-threatening infections accompanied it.  Admittedly, we never imagined that these life-threatening infections would come in the form of reoccurring ascending cholangitis.  We assumed, as did many of Lucy's doctors, that these infections would come from trans-located bacteria and fungus from her failing intestinal tract, floating around in her blood stream, adhering to her central line, and causing life-threatening infections that way.  Stasis (lack of movement) in Lucy's biliary system, TPN cholangitis, liver disease, a diseased gallbladder, and the dysmotility in her small bowel which holds a "potpourri"(Dr. R's word, not mine) of bacteria are creating the perfect storm for the reoccurring infections in Lucy's liver and bile duct.  We cannot fix the dysmotility problems in Lucy as this is as a result of her mitochondrial disease.  What we are striving for is to be able minimize the number of infections and the pain and discomfort associated with the symptoms of cholangitis. Hoping in turn that by doing this we will be able to continue to give our girl the best quality of life, right up until the end.

We have been blessed with having the best of the best doctors come together and discuss all of our options for our special girl.  After a lot of energy, thought, concern, and prayer we are moving forward with having a repeat ERCP performed, this time with placement of a permanent metal stent.  Placing a permanent stent will hopefully prevent any further strictures or occlusions in her common bile duct.  This is what we are asking the gastroenterologist who specializes in pediatric endoscopic procedures at Johns Hopkins to do for our girl.  We are hoping that by doing this it will clean out Lucy's bile duct and assist in the removal of bacteria from that area of her body.  We also hope that by doing this we will be able to eliminate her existing infection, and reduce the number of recurrent infections she is experiencing.  After reviewing Lucy's e-n-t-i-r-e medical history (I suppose this is why it took him so long to get back to us, that and the fact that he was out of the country most of January into February), the doctor at Hopkins believes that there is merit in repeating an ERCP in our girl.  He shared with our team of docs that Lucy's case is complex(as if we didn't know this:), and although he is in favor of stent placement if things are what they appear to be, he is not sure yet if placing a permanent metal stent is the way to go with Lucy.  He wants to go in and see things for himself, ugh...the uncertainty is agonizing.

Preparations are in place and plans to be transported via ambulance to Johns Hopkins are all arranged, we are leaving here tomorrow morning at 9 am and are set to arrive at Hopkins sometime around 11 am.  Lucy has a procedure time of 2:30 pm which means we are going to be busy catching everyone up to speed on our special little girl once we arrive.  We have done all that we could possibly do from our end to prepare for this adventure.  A days worth of meds are set to go with us so that we can keep her meds on schedule, along with extra bags of fluids and dextrose with additives, hard copies of medical records, medical supplies, anaesthesia records since we have yet to speak with an anaesthesiologist,  special babies, blankets, and treasures are  packed as well as an overnight bag .  Dr. R doesn't know what he is missing...or maybe he does and planned it this way :).  He'll be back just in time to help us get Lucy through the post-ERCP-pancreatitis that is going to ensue after her procedure.  We are planning to stay at Hopkins Friday night, but are are hoping to return back to our "home away from home" sometime on Saturday, depending on how Miss Lucy is doing.

Once we return, our plan is to support Lucy through the post-ERCP-pancreatitis with pain control.  As soon as the pancreatits subsides the plan is to head to the OR here to have her gallbladder removed.  Yeah, Lucy has a mountain to climb before she can get to the other side, but it is our hope and prayer that by doing all of this our girl will have a chance at living the best longest life right up until the end.

Thanks as always for your thoughts and prayers, love and support.  I will do my best to update throughout the day tomorrow.  We could never do this alone and for that we thank you!
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Debbie said...

All I know are these mito kiss are among the strongest, bravest kids I know. Lucy is such a fighter as are her parents....all of you are in my prayers.

Helen A said...

Love you Lucy! I am thinking of you and praying for you. Don't try to drive the ambulance, you know you are not a very good driver. :)