Our Days Here...

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How We Spent the Weekend…

I just finished editing what I am going to assume to be one of several revisions of Jack’s science fair project.  Long distance homework…we’re getting good at it!  I’m a mom, no matter where I am or what the circumstances, my job is to be “there”, to encourage, to crack the whip when need be :).  Drew emailed me an update on the project and what Jack has finished.  Not bad, not bad at all, but it still needs some work.  Keep going, give it your all, you can do it buddy, I know you can!

Speaking of being a mom, my mom has been with us on and off pretty much since the end of November.  She commutes back and forth from Wisconsin to Pennsylvania like she is just running an errand to the grocery store.  She knows the flight schedule leaving Green Bay and arriving in Philadelphia like the back of her hand.   She has been a rock for us to lean on, but in particular for me.  Just knowing she was “there” in my place feeding my kids “healthy nutrition”, assisting with homework, practicing piano, doing laundry, ironing my husbands shirts, making sure that everyone has what they need for each respected activity,  tailoring the kids lunches to meet each of their specific likes and none of their dislikes, enforcing our routines and our household beliefs on our kids…has been a HUGE help.  My mom went home on Wednesday, I miss her already.  Drew assures me that things are well at home, I have to trust him as I have not been home since January 2nd.  Sophie tells me that Daddy makes the bed, most days.  The kids are doing pretty darn good in spite of the circumstances, as much as it pains me they have a routine that they have fallen into without me and Lucy.  Enough time has gone by that new normals have been established.  I know when we are all home again everyone will be willing to give up their new found routines in favor of the old.  Drew is taking on things at home and doing pretty darn good as well, including the doing the laundry and sorting the mail.  Actually, anyone could probably do a better job than me when it comes to sorting the mail. Yeah, I’m that bad at it.  I am proud of all of us, but especially our kids.
  
On Friday evening we were being threatened with a few inches of snow, it only really snowed an inch at best, nonetheless it was enough of a threat that the kids stayed home with Helen.  Drew came to see us late in the afternoon and for the first time ever I left our sleeping Lucy in the loving care of the wonderful nurses on the floor and walked out of these hospital doors with my husband for a few hours.  My mission was to run an errand to Target, Drew’s mission was to get me out of the hospital.  We accomplished both and even had a car picnic in the snow.  We didn’t have time to eat in anywhere so we got take out.  No matter where we are or what the circumstances, as long as we are together life is good.

Thursday, Friday, and Saturday nights were not great nights for our girl.  She has been dealing with what appears to be episodic periods of nausea and vomiting, added with some, more like a lot of diarrhea.  Seriously, what’s up with that?  Once in a blue moon Lucy has a bowel movement, but vomiting…it’s not really her thing, or at least that’s what we thought.  Child, my head spins just trying to think of reasons for why your body is doing what it’s doing.  We keep blaming things on the infection in her body, it’s the scapegoat so to speak.  As for infections, the infectious disease doc and I had a lengthy conversation about our special girl on Friday and again on Saturday.  Dr. R cringed when I told him that it was a good conversation, good as in not good but more like a relief.   I know, I know, it’s never good when you have to consult with ID, but when you're at this stage of the game, having the knowledge of a specialist like this agree with what your thoughts are as a mother, it is good.  Dr. R and I will discuss the details of all that was talked about and hopefully tomorrow move forward with implementing the plan.

Today I gave Lucy a bath in her bath chair, in the bath tub, for the first time since, oh geez this is going to sound really bad, last year.  She has received sponge baths, but an actual bath that’s something entirely different.  She was a little lady of leisure in the tub today, and two hours later(seriously, it took that long) when all was said and done, she wanted everyone on the floor to come and see how clean and beautiful she looked in her I LOVE YOU pajamas.  She makes me smile, and everyone else too.
Drew and the girls came by to see us on Saturday night, Jack had a sleepover with his buddy Paul, and then they all came to see us again tonight.  I love having them all here.  Megan and Sophie brought their American Girl dolls to the hospital, wheelchairs and all.  Jack brought his finished science fair board to show me.  Ask me anything you want about Venus fly traps, if I don’t know it I know who will.

We are hoping to hear back from the doc at Hopkins this week.  Conversations are also being had with the doc at Jefferson.  In the meantime, Lucy is doing pretty well considering all that she has going on.  Her labs are stable and for the first time this month her calcium is not increasing, we are attributing this to the Calcijex working.
 
Tomorrow is another day, we’ll take each day as it comes and celebrate all that we can.  Jack has my computer so I don’t have access to pictures, sorry Alicia, I’ll post some tomorrow when I get my Mac back.

Thanks as always for your thoughts and prayers, love and support.   

Today...

Today was much better than yesterday, so very thankful for that.

Sleep is scarce for me these days, I am looking forward to getting some tonight.

Praise God for antibiotics,  they are lifesaving!  Infectious disease has officially been added to our team of specialists, we are in a difficult spot when it comes to all of the bacteria and yeast Lucy has growing in her body.  We are looking to the experts for guidance and suggestions for keeping the bad bugs away for as long as possible.  Like I've mentioned, Lucy is resistant to some of the broad spectrum antibiotics that we are used to using.  To be honest, this scares me.

Drew and the kids came tonight.  When Lucy saw them she said, "I'm so happy to see you guys...I haven't seen you in awhile!".  Be still my beating mama heart!

So very very grateful for the wonderful people that God has placed in our life.  We have friends across the hall at the moment.  She took this picture of Lucy and me this morning and brought me breakfast from Dunkin Doughnuts:).

My husbands "work family" reached out to our family in the most amazing way,  I want to say thank you so much for all that you have done for us, it means so much!!!

Still no word from Baltimore, but brief talks were had with our main doc this morning about what we feel needs to get done.  What we want and what is going to happen, we are still working on the details of that.  I will share when I know more.

My emotions have been all over the place today.  I am going to blame it on the lack of sleep...yeah that's it.

Thank you so much for your thoughts and prayers, love and support!

I'm so tired...

I am going to keep my words short and sweet and just say that it was a difficult day!  We finally have Lucy back on the correct antibiotic, her pain is more under control, she has not retched or vomited since noon, and her fever is all but gone.  Things appear to be heading back in the right direction, again.  We are still waiting to hear back from the doctor at Hopkins.  We are even more concerned about her risk of infection now, she is only responding to certain broad spectrum antibiotics.  I'm so tired, just as I was getting ready to call it a night, Lucy woke up from her nine hour nap.  I am relieved to see her feeling better tonight, but am hoping that she will be ready to fall back to sleep soon.

It's 3 AM...

We're awake!  Lucy woke terrified, shaking, in pain, very tachacardic, retching, and with a fever of close to 105!  We are making yet another change to her antibiotic coverage.  Labs and cultures are done.  We are waiting on orders for an increase in her pain meds.  This screams cholangitis to me again...still.  I am looking forward to talking with our main doc later on in the morning, not sure his feelings will be the same.

A little Q & A...

People ask questions because they are seeking knowledge, information, understanding...I fully respect and GET that.  I like to do the same thing, just ask any of our docs :).  We often get asked questions about this, that, or the other.  I thought I would share a few of these questions and answers with you.  Perhaps it will help you to understand, give you some incite, or possibly confuse you more.  Whatever, keep asking questions, knowledge is power.

Is Lucy a candidate for organ transplant?   I can't recall if I have ever written about this here.  The answer is no, Lucy is not a candidate for intestinal transplant, or any kind of transplant. There have been a few attempts at intestinal transplant in kiddos with mitochondrial disease, but sadly the success rate is extremely poor.  Even if it were possible, Lucy's mitochondrial disease is very progressed, she most likely wouldn't survive the immunosuppressant medications that are necessary to prepare the body for transplant.  Lucy has way too many issues stacked against her!  Mitochondrial disease is a disease that can affect everything in the body down to the cellular level. It is thought that even if transplanting her were an option, the newly transplanted organs would eventually become affected by the disease.

What about EPI743?  A while back I talked about a new study drug that has been tauted as "the newest med" for patients with mitochondrial disease.  EPI743 is a  relatively new study drug that Drew and I learned about at the Mitochondrial Medicine Conference in Chicago back in  June of 2011.  At that time the FDA had just granted orphan drug status to the manufacture of EPI743.  This allowed patients who did not meet the original criteria for the study drug to receive the study drug if they met other less stringent criteria for the med.  By less stringent, I mean that patients did not need to have a diagnosis of leigh's disease or confirmation of their genetic mutation to become eligible for the study med.  Rather patients had to meet other diagnostic criteria, one of which was being deemed ninety days to end of life.  Lucy met all of the new qualifications for the study drug, however was not a candidate to receive EPI743 because of the way the med has to be administered and absorbed.  EPI743 is a fat soluble enzyme that is administered via the GI tract with food.  In other words, it does not come in an IV form.  Lucy's intestinal failure and malabsorbtion issues disqualified her as a possible candidate for EPI.

What options do we have remaining?  Ahhh....this is the million dollar question at the moment.  The way I see things, and believe me there are many others who are looking at our girl and seeing things differently, but in the end the opinion that matters most is what Drew and I decide is best for Lucy.  Currently we have put all of our proverbial eggs in one basket yet again, that basket being the GI doc at Hopkins in Baltimore.  We are waiting to hear back from him to find out what options he recommends for trying to alleviate the symptoms associated with Lucy's cholestasis and reoccurring cholangitis.  We heard back from the GI doctor at Jefferson last week, his recommendation was  a maximally invasive surgery in which we would reconstruct Lucy's bile duct by bringing up a portion of her small intestine and attaching it to her liver.  This option would definitely get rid of her floppy bile duct, but replacing one failing organ with another failing organ will most likely create an entirely new set of problems that will more than likely shorten Lucy's life.   Here is what we do know, we cannot fix the dysmotility problem or stasis in Lucy's bile duct.  Her entire gastrointestinal tract including her biliary system lacks any motility or movement as a result of her underlying mitochondrial disease.  What we are hoping to be able to do is alleviate some of the things that are contributing to her pain and reoccurring cholangitis. Repeating an ERCP and placing a more permanent stent in Lucy's bile duct is an option that the GI doc at Jefferson is willing to consider, but expressed concern that it is not going to fix the underlying issue, and may not fix anything at all.  Like I mentioned, we understand that we are not going to be able to fix her underlying issues.  What we are hoping for are viable options that will alleviate some of the pain and discomfort associated with cholestasis and cholangitis.  Removing Lucy's gallbladder is something that our surgeon has been wavering back and forth on doing for our girl.  It will not fix her problems, but again could potentially alleviate some of the pain and discomfort that Lucy is experiencing.  Weighing the risks versus the benefits, that is what we have been doing on a daily basis.

What is Lucy's prognosis?  This is a very tough question to answer!!  She is being described as "end of life".  Mito is an unpredictable disease, and as such no two patients are the same or follow the same path for their disease.  Although there are similarities between patients, there are just as many, if not more, differences.  These differences are what make "the end" difficult to predict.  For Lucy it is predicted that there will come a point in time when we will no longer be able to treat her reoccurring infections, and as such we are faced with the unthinkable, how long and how far are we willing to go to keep her body alive.  The decisions we make will play a role in how long or short Lucy's time here on this earth will be.

Is Lucy comfortable?  Comfort has always been our goal for our girl.  I can not express to you how difficult it has been to find just the right balance of medications to keep Lucy comfortable.  Pain management has perhaps been the most frustrating component of Lucy's disease to manage.  Lucy metabolizes meds very differently than you or I, or anyone else that our medical team has encountered.  What we assume will work for her doesn't, and what we think will not sometimes does...that's just so Lucy.  We have a baseline level of pain control that is helping to keep her pain tolerable.  Tolerable is the best we can hope for at the moment until we are are ready to say to our team that we are willing to give her comfort at all costs.  Lucy never ceases to amaze us with her coping abilities, in particular how she has learned to cope with pain.  It is both heart breaking and astonishing to witness our little girl endure so much, she is our hero.

How are we doing?  This is such a difficult question to answer in one word, or even a few sentences.  We are overwhelmed with so many feelings and emotions.  There is so much uncertainty in our life at the moment that we are not sure what to say.  We are relying on our faith, family, and friendships to help guide us through some of these difficulties in our life.  Our focus is on our entire family, we are blessed to have one another no matter what the circumstances.

I know that there are many more questions that I could discuss, but I mentioned in the title that this was going to be "a little Q & A".  Feel free to ask more questions, it has always been my desire to share our story in hopes that by doing so our story might just help others.

Thanks as always for your thoughts and prayers, love and support.  We could never do this alone.

Here we go again...

Fevers, retching, vomiting, tachacardia, increasing abdominal pain...here we go again!!!  God, my baby could use a break!

On Friday we changed Lucy's antibiotic coverage and discontinued her antifungal, much to my dismay, all in an effort to keep her from becoming resistant to any more broad spectrum antibiotics or antifungals.  I have a feeling that we are going to be making the switch back to what we were doing, at least that is what I am hoping for.  Our main doc is not on call tonight so we are at the mercy of the resident and senior resident.  Hopefully they are willing to listen to my pleas.   Labs and cultures have been ordered, again.

T.G.I.F.

Hurry up and wait...

Things with Miss Lucy have been somewhat...sorta...kinda... stable this week.  We are continuing to try and figure out her hypercalcemia issues.  Not sure if that is possible, but we have all the best minds working on it.  Despite all of our efforts to lower her calcium levels, her serum calcium as well as her ionized calcium are continuing to rise.  Our best guesses at this point are that her poor renal function is playing a role in this issue, as well as her low vitamin D levels, her poor metabolism, her parathyroid hormone function, and also the fact that Lucy is osteopenic.  I think that just about covers any and all reasons for hypercalcemia...Lucy happens to be dealing with them all!  We started Lucy on Calcijex today, an IV form of vitamin D given to patients who are on dialysis.  All of the literature about this med says it can raise calcium levels in a person by pulling calcium from the GI tract.  Lucy does not have any calcium in her GI tract so we are taking a chance that this could help to raise her vitamin D levels enough so that it will help with her bone density issues, thus helping to prevent calcium from leaching out of her bone into her blood.   We shall see...

Lucy has a few hours a day in which she is awake and interactive; however, she continues to sleep a majority of the day.  We are waiting to hear back from the GI doctor in Baltimore before we make any decisions about what we are going to do next.  As for now we have plenty of issues that we are attempting to resolve, or at the very least address.   We are in that hurry up and wait mode, and for the first time in Lucy's life I don't mind staying in this mode for just a while longer.  The decisions we have to make are not easy and will be life changing no matter what we decide.


Hurry up and wait...we are good at this, we have had lots and lots of practice over the years.

Priceless...

Her Expression when she heard "the kids" were coming to see us tonight

...priceless!

How was your day...

Today we saw a lot more of these baby blues!

We did a lot of hanging out today.  

We are in the hurry up and wait mode, waiting to hear back from the two GI docs who are reviewing the results of Lucy's most recent MRCP to make any decisions about what we'll do next. 

Unlike the past few weeks, Lucy struggled to find sleep today.  We are weaning her down on some of her dilaudid which is making her nervous system a bit jumpy.  She is hypersensitive to everything, the slightest noise seemed to wake her...  

thus making her a wee bit more irritable than her norm.  I'm so not complaining though as I was begging God this time last week for any opportunity to see my girl again.  We describe Lucy as being mostly irritable and sometimes happy, that is exactly what she was today :).  

We had art therapy this morning.  Lucy wanted to cut paper, seriously.  Her vision is not great and neither are her cutting skills, but we managed. By managed I mean we did this all before Dr. R saw her blindly cutting paper.  We hung her artwork on her door for all to admire.

Daddy came to see us today, as well as some friends from church, and the ultrasound tech who was not a welcome sight.

I feel like I spent the entire day trying to get Lucy to sleep soundly.

She finally succumbed to sleep just before eight o'clock tonight.  

Good night sweet girl, see you in the (not so early) morning, not before I see you in my dreams.

Where things stand...

We are not quite sure where things stand...we are living one day at a time at the moment.  Trying desperately to figure out Lucy's new baseline.  The results from Lucy's MRCP found that "there is persistent significant dilation in her common bile duct. The distal common duct again tapers markedly just above the Ampulla of Vater".  In other words, the MRCP detected that there is a blockage, again, lower down in her bile duct this time.  This narrowing is in a different location than her first stricture that we discovered in November.  The findings from her most recent MRCP have been sent off for review to two separate GI doctors who preform pediatric ERCPs, the one at Jefferson who recently preformed an ERCP on Lucy, and another at Hopkins in Baltimore who apparently sees more pediatric cases as well as many of the liver transplant kiddos here at DuPont.  Our hope is that one or the other will be willing to take a look at Lucy's case and make recommendations that will hopefully improve Lucy's current situation, be it repeating an ERCP, placing a stint, surgical options, or all of the above.  We are seeking as much information as possible so that we can make the best medical decisions for Lucy's life.

The MRI of Lucy's brain did not show us much of anything we didn't already know, mild atrophy blah, blah, blah...most likely related to progression of her mitochondrial disease.  They did find that her left jugular has no blood flow which may or may not be causing some issues.  We are going to do an ultrasound on Monday to investigate this further.  Seriously, this kid is an anomaly, everywhere we look they find something "remarkable" about her.  But we already knew this about her, that is that she is remarkable :).

Lucy is holding her own :).  It turns out that intubating her and putting her on a ventilator for a short period of time helped to improve her lung function by inflating her lungs fully, something she doesn't do well because she is such a shallow breather.  Antibiotics finally started doing their job on Wednesday, Lucy has not had a fever since that time!  Her vision issues are just that, still an issue.  Although she is still pretty sleepy, she has had more periods of time in which she is awake and interacting with us more than we have seen in awhile.  This is so amazing when you consider that we have been discussing "end of life" issues with our medical team since the day of admission.

I don' t think I need to tell you that things have been difficult, we have had to discuss the unimaginable, the way we envision our little girl's end of life.  What we do, the decisions we make, the choices we are presented with, ultimately will determine how long or short her time here on this earth will be.  Medicine can do a lot to keep the human body alive, but the question we are faced with is how much medical intervention should we allow to keep her body going. There is no easy answer to this question.  You can not imagine what it is like to be in our position until you are faced with it personally.  I would never wish for anyone to be in our shoes...

We have had a quiet, boring, non medical drama weekend...just what we were hoping for!  The week ahead brings new hope and lots of decisions.

Thank you all so much for your thoughts and prayers, love and support, it means so much to know that so many care.

Tomorrow's another day...

So very, very thankful for another day...Lucy showed us that she was still in there today!!!

We have so much that we need to figure out, but to see this face so full of life today was an answer to so many prayers!!

She's back...

We are back up in our room on our regular floor, I suppose someone should cancel our reservation in the PICU. Lucy is breathing on her own, with the help of a litter of O2...huge sigh of relief!  Dr. R commented to me when I told him that she was breathing on her own, "...of course she is, breathing is her strong suit", then I saw him take a big sigh of relief too :).

We have too much to try and process, I have no energy left  to go into any of the details tonight.

We are sensing a shift in our life, are scared, very emotional, and in need of some time to process things.

Thank you all so much for your thoughts and prayers, love and support...we could never do this alone!

Update...

Lucy just got out of the scanner at 2:45pm, it took a little longer than expected because they wanted some more imaging studies.  We are going to keep her sedated and intubated while our urology team changes out her suprapubic catheter.  They are even throwing in a shampoo and hair do for us. We are praying that our girl will go back to breathing on her own once extubated.  Thanks again for all of your thoughts and prayers. 

MRI...

I left our sleeping beauty in the wonderful and capable hands of the doctors and nurses in the MRI suit at 11:31am. We added an MRI of her brain in addition to an MRCP...we are praying that the information we receive today gives us the ability to make the best decisions for our girl.

Thank you all for your thoughts and prayers, love and support!  Please, please continue to pray for Lucy.

Our girl...

Our girl is in the fight of her life...please keep praying for Lucy, for all of us.  Our hearts are hurting as we continue to watch our little girl decline.

We are desperate for answers, Lucy is scheduled to have another MRCP tomorrow morning around 11am.  Our hope is that we will get some definitive answers from this test so that we can make the most informed decisions for our girl.  I am terrified, Lucy and anaesthesia will meet once again, only this time she has never been sicker.

Our day...

The Mount Rushmore of Doctors...that is how our wonderful social worker referred to the meeting in Lucy's room this afternoon.  Three of DuPont's finest came to see us, all at about the same time, to discuss the good, the bad, and the ugly.  There were a lot of tears, and also a lot of laughter.  Laughter mainly because these docs are down right amazing and childish all at the same time, that is what makes them awesome pediatric docs.  If you knew the serious nature of the issues we were discussing, I'm not sure you would laugh, but these guys know how to lighten the mood.

I am going to revert to a list format tonight to touch on the topics of our day.  Things are not looking so good at the moment and I am too tired to try and write about all of it in nice paragraph form.

Here goes...

Lucy's calcium levels are finally coming down, which is helping to contribute to her lucidness.  She is having brief moments in which she is not sleeping.  I hesitate to use the word awake to describe her as that is not befitting, more like aware is what she is.

We discovered last night quite surprisingly that Lucy is unable to see.  She can see light but is unable to see things.  Ophthalmology has been consulted and after discussing things with them, we suspect that Lucy is dealing with cortical vision impairment.  Yes, this is quite shocking, and no, we don't know why this is happening.  We can only speculate that she has suffered a set back neurologically.  I will write more on this in a later post as I am only just beginning to process this.

Her kidney function is not great but improving slightly ever since we started flushing her with extra fluids, reducing the volumes of her medications that are known to cause renal issues, and cutting back on the use of vancomycin.

Lucy underwent a HIDA scan today to see if we could get a better idea where in her biliary system things are backing up.  We were hoping for clarity with this very long and very difficult study, I think the results have caused more confusion.  We are going to repeat some of the imagining studies again tomorrow to see if we can get clear picture of things (pun totally intended).

Lucy received a blood transfusion yesterday. Sadly she was not sporting that post transfusion glow that we so look forward to, she just looked puffier today with liver numbers that indicated that she was having a difficult time processing all of the new blood she received.

We are continuing to assist her kidneys by pumping her full of fluids and then emptying her of fluids.  All of this is in an effort to flush the dangerously high calcium out of her body.  This process has resulted in some even more serious electrolyte issues, Lucy's potassium was critically low again today which had us making yet even more changes to her TPN and fluid bags.

Dr. R has a theory as to why Lucy is hypercalcimic, we are going to be doing some blood studies checking her iron levels.

Like I mentioned above, I had the opportunity this afternoon to discuss "things" with three of the most amazing doctors in this hospital.  Each of them has their own unique approach to how they see things, but all of them share the same desire to give Lucy the best quality of life.

Our afternoon meeting lead to a serious evening meeting with our main doc...which concluded with lots of tears.

(I can't imagine being any place else at the moment...)

Drew is here with us tonight, we need time to process all of this, together.

Thank you so much for your thoughts and prayers, love and support.

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.

Even tougher times...

Our goal for Lucy has always been to give her the best longest life.  This goal has helped guide us through the toughest of times.

Today very difficult conversations were had about whether or not we could fix Lucy...and if we should fix Lucy.

Drew and I believe that in order to give Lucy any hope of "getting better" and improving her current quality of life we need to move forward with the cholecystectomy and cleaning out of her bile duct.  Just how and when this is going to be done is the question.

As difficult as all of this has been, we have been overwhelmed with the love and support from all of those who know and love our girl here at hospital.  God has blessed us with the most wonderful people in our life, for that we are so thankful!

Lucy continues to be so very very sick, please keep praying.  We increased her fluids today in an effort to flush the excess calcium out of her body, her levels have continued to rise.  Her renal function has continued to decline which makes every change we make to her fluids and meds even more difficult.

I was able to spend some time with the birthday boy and the girls tonight.  Celebrating life...isn't that what living is all about!

E-cards...

I have received several emails form friends asking if I could please post how to send an e-card to Lucy again.  Here is the link and the information about how to do that.  I can' tell you how much your thoughts and prayers helped carry us through our last admission.  Lucy received over 200 cards from friends, family and blog readers all over the word.   

http://ecard.nemours.org/welcome.act;jsessionid=dHj2sk77FBkCCpT1kZ-S7Q**

The hospital website has a link to send a patient in the hospital an e-card.  They will then print the card and deliver it to the patients room via "bear mail".  Just click here  or on the picture above and follow the directions if you would like to help brighten Lucy's day.  Lucy's room number is 3704.

TImes are tough...

Things are not looking so good for our girl today.  She is still febrile, has not been awake at all today,  and clinically is worse than she was yesterday.  She is a mess, one hot mess!!!!

Lucy's labs came back this morning looking worse than yesterday.  All of her electrolytes are off, in particular her renal function labs, alerting us that she is renal failure.  She is hypercalcimic again.  Unlike the last time her levels were critically high, we are unable to flush the calcium out of her body this time because her renal function is so poor.  We are afraid of upsetting the delicate balance of things going on inside of our little girl's body.  She is in need of a blood transfusion, but we are holding off on doing this because her electrolytes are so unstable.  Adding any extra fluid, even in the form of packed red cells, could upset things even further.  We are looking into a new med for Lucy that could potentially help with the calcium issue.  It is a med typically given to dialysis patients, and as such is not something that is commonly used to treat hypercalcemia in a patient like Lucy.  As for now we have removed what little calcium we added back into her TPN.   

Pain is increasing, we are once again weighing Lucy's need for pain control with her need to breath.  We met with our palliative team today, surprisingly they had no recommendations for us at this time. Everyone is worried about our girl and as such are afraid to "rock the boat".  About the only thing Lucy has going for her at the moment is that she is breathing.  To quote the good doc yet again, "breathing is good, not breathing is bad". 

We had some very difficult conversations with our surgeon tonight which has left us needing to process a lot of things before I can adequately express them in writing.  I will say this, we are so blessed to have a team of docs who care so much about our girl and her quality of life that they are willing to discuss the really tough stuff of life with us.

Tomorrow is our firstborn's 12th birthday, where has the time gone!?!  I am looking forward to seeing him and the girls tomorrow night.

Please, please pray for Lucy, times are tough...

Mommy, Lucy is Sick....

(This photo was taken on New Years Day.  Oh how I love these girls!)

While eating dinner on New Year's Day Drew and I were talking to the kids about life when Megan said to us....

"Mommy, my nose is dripping."

"Why?"

"Because I need to cry."

I looked at Megan and told her that is was OK to cry and then asked her what was making her feel like she needed to cry.

Her response, "Mommy, Lucy is sick..."

We along with so many were happy that we were able to spend the holidays at home, but it caused some confusion in our kids.  They too were happy, but they could clearly see that Lucy was not well and I think that they were afraid to talk to us about it for fear that we would not be so happy to be home. 

If you could please pray not only for Lucy but for our other three kiddos, that they too can find clarity and peace among the chaos.

This is so hard!

A quick update....

Currently Lucy is one sick little girl.  We are in the right place, she needs to be here right now.  Her labs look about as bad as she does.  She is non-responsive and has been sleeping pretty much for days.   When we brought her home for Christmas, she was not well but our hope was that things would improve.  Sadly they have not.

It is suspected that Lucy has cholangitis again.  We did a repeat ultra sound of her common bile duct area this morning and found that it is once again blocked.  Simply removing her gallbladder is apparently not going to fix this problem.  We are going to need to discuss our options with surgery tomorrow.  Pain continues to be an issue, I am relieved to be here as we could really use help with staying on top of this for our girl.

We are being well taken care of by the wonderful team of doctors and nurses who know and love our girl.  We are settling back into room four, "our room" on "our floor".  I will update more tomorrow, I am exhausted and am going to take full advantage of having around the clock nursing.

Thank you again for all of your thoughts and prayers, love and support.

Happy New Year?...

It was good while it lasted...we are packed up and getting ready to check back in to the "hotel DuPont".  Things with our girl are not looking well, and we're not sure what is going on with her.  She has a fever, heart rates are higher, and she is very lethargic.  Overall, she just plain looks sick.  Lucy is scheduled for surgery on Monday to have a cholecystectemy, her gallbladder removed.  Before we do this, we need to figure out what is going on with her.

Once again your prayers for clarity and better days for our girl are much appreciated.