Stenting her is the right thing to do...

I just ate my breakfast at 3:45pm, or maybe that's considered lunch, or maybe even and early dinner.  Needless to say my head is pounding from ignoring my hunger all day.

It has been one of those weeks already, and it's only Tuesday.  If I'm being completely honest, it's been one of those years...

It's possible that I have too much to do in a day...just saying.

After getting everybody off to school this morning I had the monumental task of packing up the things Lucy and I are going to need to survive for the next few days while we are in the hospital.  Notice how I wrote a few days, I am being optimistic here :). I think the only thing I forgot to bring was food for me.  Yep, I left the bag with my lunch and then some sitting right on the counter in our kitchen, hence the reason for my not eating until 3:45pm.  It's alright, I promised Lucy a dinner date in the cafeteria tonight, that's how much I love her :).  One grilled cheese on 12 grain bread for me and one french fry for Lucy.  She doesn't eat the fry, it's so that she can use it to lick the teeniest tiniest bit of ketchup, if she is feeling up to it.  More often than not she just likes to hold the fry and feel like she is "eating" dinner too.  It's the little things in life :).

Lucy is going to be transported tomorrow morning from our home hospital down to Johns Hopkins, again, to have another stent placed inside of her common bile duct.  She is scheduled for this to happen at around 2pm for all of you prayer warriors out there.  This time this will truly be a permanent stent, the plan is to keep this stent in for the rest of Lucy's life.

This decision was really not a difficult one for us to make.

Without a stent she is miserable, with a stent she is more comfortable.  It's really that simple.

Above all we want our girl to be comfortable!  Of course we do, all parents want their child to be comfortable, but saying we are choosing comfort in this situation means that we are choosing comfort no matter what the circumstance.  We are choosing to accept all of the consequences that accompany this decision - infection, possible rupture of her bile duct, pancreatitis, the stent becoming embedded in the wall of the bile duct, sepsis...These are all very real possibilities, but when compared to Lucy living the rest of her life in pain, this was not as difficult of a decision as you might think.

Do we wish that Lucy's circumstances were different, absolutely!

How we wish we could fix this and so many of the other problems for Lucy, sadly that is just not our reality.

Fixing Lucy's bile duct issues will not fix Lucy, her mitochondrial disease has reeked havoc on too many of her major organ systems for us to even consider putting her through a 20 plus hour surgery.  Fixing one failing organ and replacing it with another failing organ is not going to fix our girl.  This is where Drew and I and our medical team have had to come together and discuss the really tough stuff of life.  Just because we can doesn't mean we should, that has been the gist of the conversations that we have had with our team, in addition to discussing what we as her parents ultimately want for Lucy.  Our goal has always been and continues to be to give Lucy the BEST longest life.  We are hoping and praying for Lucy to have as many good days as possible, which is why we believe that stenting her is the right thing to do.

A long day...

We are home and exhausted, it was a very long day!  We have an amazing medical team, a truly amazing medical team!  This afternoon we had four of DuPont's finest all come together at the same time to discuss the really tough stuff of life with me, yet again.

Lucy's cholecystostomy tube was in the correct position and did not need to be replaced today.  This was both a relief and a disappointment.   A tube change would have been an easier solution to her problems.  Lucy's biliary system is very diseased!  We suspect that Lucy has cholangitis, again.  Due to all of the antibiotics that she is on she is not presenting the same way that we are used to seeing in our girl when she has had cholangitis in the past.  Lucy is not feeling well and is in pain, it is so difficult for everyone who knows and loves her to see her this way.  It is excruciating for us as her parents to see her this way.

We also suspect that because of this infection her entire body is not functioning as well as it should be.  We took a closer look at Lucy's bladder issues today via ultrasound and concluded that we need to get Lucy's current systemic infection under control because we suspect that her bladder spasms are related to this current systemic infection.  While we are working on making yet another antibiotic switch we are also going to try instilling Lucys' bladder with medication that will help control her bladder spasms. Topical medications typically used to control bladder spasms are not an option for our girl anymore as we used to use them, but we discovered that they caused severe vision loss in our girl.  Therefore, instilling her bladder, putting the medicine directly in her bladder, is our only other option.

Due to all of these recent findings we have decided to place another stent back inside of Lucy's common bile duct next week.  Only this time we are not planning on removing it, it will remain inside of Lucy forever.  Placing a stent and leaving it in comes with it's pros and cons.  The biggest pro is that Lucy will be more comfortable.  The biggest con is the risk of infection.  No one knows for sure how long a stent can stay inside of a bile duct in a child as young as Lucy before it becomes embedded and ultimately infected.  Placing another stent will give Lucy the most comfort, and that is why we are choosing this option for our girl.

Thanks as always for your love and support, thoughts and prayers.  I did not not intend to post about the Energy for Life Walkathon and these medical issues all on the same day.  Please consider joining our team and raising funds for a disease that very much needs research to help fund life saving treatments and ultimately a cure.

It never gets any easier...

I am  staring at the monitors watching my little one's heart rate, oxygen saturations, and blood pressure, the wave length is constant and slow...so the opposite of what we are used to seeing with our girl.  Something is going on with Luc.  Whatever it is it's sucking the life out of her at the moment  We're just not sure yet what it could be.  I've probably written/said these sentences a hundred or more times in Lucy's life.  Just ask our main doc, he worries every time I write/say it.

Lucy is about to undergo another cholecystostomy tube change, we suspect that her tube has migrated out of position causing her to be in even more pain.  At least we hope that is the cause for her increase in pain, if it isn't then we will need to investigate further.  In addition she is complaining of constant bladder pain.  From a culture stand point her urine is looking OK at the moment, but something is going on that we need to try and figure out.  Our urology team is going to take a look at things while she is under anesthesia today.

As you well know we are very familiar with hospital routines, but no matter how many times we have been in this position before... it never gets any easier.

Your thoughts and prayers, love and support are greatly appreciated.  I will try and update when all is said and done.

She's worth a cure...

(Picture from Last year's walk.)

I know, I know...it's been a while.  I promise a more detailed update really soon, because believe me there's a lot I have to write about.  But for today I have very important matter to talk about...

I am sooooo behind in announcing this, but better late than never...right!?!

It's that time of year again when we join together to show our support for those affected by Mitochondrial Disease...

Please Join us for the Annual UMDF Energy for Life Walkathon to be held on 
Saturday, September 28th, 2013
at the Camdem River sharks Stadium
Registration begins at 7:30am with the walk to start at 9:00am

You can sign up here, or by clicking on Lucy's team logo or the Energy for Life logo on the left sidebar of our blog.  Any of these options will link you to our team page "For the Love of Lucy".  There you can sign up to join our team, or be a virtual walker if you live far away from us.  Either way we would very much welcome your support.  Note you must register as an individual, no family registrations please (this allows us to count everyone!).

She's worth a cure, don't you think!

Wanna get away...

And take a jump in the pool...

Sophie, Lucy... is that you?

And feel the sand in between your toes...or not!

And dip your toes in the ocean...

And feel the sun on your face....

And the wind in you hair...

We're making memories...to last a lifetime!

Wish you were here!

A little about today...

Our day did not go exactly as we had hoped it would....

We are home, which is a good thing, but I am on fever patrol at the moment.  Lucy is currently sitting right at our threshold for what we consider a fever.

We are making an antibiotic switch in just a about an hour, I am hoping that this med will do what it has done for us in the past and kill the bad bugs, if indeed that is what is causing this rise in body temperature.

Lucy's procedure took much longer than any of us expected today, almost three hours.  There were complications, multiple complications.

Our IR doc and surgeon were both unsuccessful in placing a different type of drain in Lucy's gallbladder.  Her gallbladder is simply too diseased.

They were able to place the same type of tube, a Malecot tube, inside of Lucy's gallbladder, but a smaller one.  It's not ideal.  She is leaking around the tube.  It is sutured in place again, but the tube will most likely come out sooner rather than later.

Even worse, we learned that Lucy has a significant sized stricture further down in common her bile duct that is preventing the flow of bile from leaving.  We knew that her bile duct had restenosis a few weeks ago when we replaced her tube, but at that time there was a narrow opening in which bile could still drain. This is no longer the situation.

Due to the back up of bile and pressure inside of her, her entire biliary system is becoming more diseased.

We are faced with having to make some REALLY TOUGH decisions.

It is only a matter of time before our girl gets sick, really really sick again.  We described her as being a bacterial time bomb today.  We live with the fear that her next infection could be her last.

Our surgeon came out of the procedure to "talk" with us, by the way he has the worst poker face.  He called our main doc to come and join in on the conversation.  Three different scenarios were discussed, none of which are good.

We can't fix the problem, we can't fix Lucy.

What we are hoping to do, and what we believe we have done all of Lucy's life, is to choose the option that will give Lucy the best longest life.  For us this means choosing the option that will give Lucy the most comfort.  We could try and keep Lucy alive for a long time, but she will suffer and that is not what we want for our girl.

We all agreed that we need to think about things, but quickly.  Our surgeon is going to reach out to the doc at Hopkins and inquire some more about our girls unique biliary system.

With the blessing of our entire medical team, we are moving forward with our beach vacation.  The good doc is even reaching out to the nearest children's hospital for us, you know, just in case.

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

No news does not mean NO news...

No news unfortunately does not translate to mean that there is NO news.  Life is BUSY these days, and   unfortunately illness does not understand the concept of summer vacation, medical dramas abound!  We have been to the ED at least twice in the past two weeks, have had multiple clinic visits in the same two weeks, and have spent hours on the phone/email communicating with our knowledgeable medical team all in an effort to try and figure our girl's latest medical issues/mysteries.

Which brings me to today, Lucy is scheduled this afternoon to have another cholecystostomy tube placed in IR under general anaesthesia.   While Luc is under we are going to maximize our use of anesthesia and give Lucy all new plumbing - a new GJ-tube and a new suprapubic catheter too.  Normally, we do not change these tubes out under general anesthesia, the risks do not out weigh the benefits, but that's not to say Lucy wouldn't prefer to be "knocked out" for these tube changes.  They are uncomfortable for her and she hates having going through them, but they are generally very quick procedures that afterwards ends with many mommy hugs and and kisses as well as a promise of something I would not normally agree to :).

In my last blog post I wrote that our IR team was successful in dilating and placing a new drainage tube in Lucy's gallbladder, the same type of tube that she had in before.  Unfortunately, that tube has shifted position and has migrated out of Lucy's gallbladder causing fluid to leak out and around her stoma site.  Fortunately it is still inside her stoma tract keeping her stoma dilated which hopefully means that today's procedure will be smoother.  We are going to attempt to try using a balloon type tube inside of Lucy's gallbladder as opposed to a Malecot tube.  Our hope is that the balloon inside will keep the tube in position longer.  On the flip side our fear is that the balloon inside will take up a substantial amount of space inside of Lucy's gallbladder which could affect the amount of bile drainage that comes out of the tube.  What we do know is that we need to attempt to keep some type of drain in for longer than a week and a half.  Yep, that's how long her last tube lasted.  Lucy's common bile duct has restenosis again, we learned this during the last tube placement when they did a repeat cholangraphy.  We need some type of tube to drain in order to alleviate the pressure inside of Lucy's biliary system, it's the pressure inside which causes her so much pain.

Along with our medical life we have a family life which includes four kids, two parents, nurses, and one goldfish.  It is summer and we are bound and determined to enjoy some of it!  If all goes well today and everything else in the universe aligns, we are packing up(I write it like it's just that simple -ha) and heading to the beach to enjoy some fun in the sun and to make those all important memories.  The preparations for this trip alone could be a medical drama of it's own, but just because something is complicated doesn't mean you shouldn't do it!

Your thoughts and prayers, love and support are greatly appreciated.  We could never do this alone, and are so grateful that we don't have to!!!  For all of you prayers warriors out there, Lucy's procedure time is 2 pm.  I will try and update later.