Give Blood...

Not just for our girl but for the many others who need your help too, thank you!

For the Love of Lucy Blood Drive

Thursday, May 9^th 2013

3PM-8PM

Please visit:   http://www.redcrossblood.org/make-donation and search by sponsor code 02215121 to schedule an appointment.

Please contact tedra.farrell@redcross.org if you experience issues scheduling donor appointments.

Donor Eligibility Guidelines: Click Here or call 1 800 RED CROSS

Donation Tips (including tips for after donating):  Click Here

Really Tough Decisions...

I want to say a heart felt thank you to those of you who continue to check on, think of, pray for, and love all of the Marletts.  We value and appreciate all of the love and support, and thoughts and prayers that are given to our family.  I am continually in awe of people!  Despite what the media portrays, people are good, they genuinely care!  They are loving and want to lend a helping hand.  People want to understand and relate, we are witnesses to all of this every day!

I have wanted to write so many times in the past few weeks, but as it should be life has gotten in the way, it happens.  The need for sleep outweighs everything at the moment.  We have also needed time to try and process all that has and is occurring, time is not something that is in abundance around here.  I am proud to say that we have fallen into somewhat of a groove with things at home, it feels so good to have a sense of routine and familiarity back - once again, we have a new normal.  That being said, we know that this is only temporary as Drew and I have been contemplating, praying, thinking about, and discussing with our medical team just what we are going to do about our girl and her failing gastrointestinal system.  

I mentioned when we left the hospital at the beginning of March (can you believe we have been home since then, with the exception of a few clinic visits :) that we are facing some very difficult choices when it comes to Lucy's life.  We all felt that with the placement of a somewhat permanent biliary stent, and by permanent I mean eight weeks, and the use of continual "big gun" IV antibiotics and anti-fungals that we had some time to go home and think about these difficult choices.  Eight weeks goes by faster than you think, and so now here we are, faced with the reality that we need to do something.

Let me just preface this post with saying that our options for Lucy are not going cure her or even make her better.  The choices that we are contemplating will hopefully reduce her risk of recurrent life threatening infections, which in turn will hopefully minimize some of her daily pain, thus improving her overall quality of life.

All that being said, we really only have two options for Lucy.  The first, is maximally invasive surgery to try and reconstruct her bile duct, and the second is to do nothing.  Both options come with a tremendous amount of risks!

If we choose to do nothing, what we are really choosing is to have the stent removed and treat the reoccurring cholangitis and pain that will ultimately ensue.  It is thought that Lucy will develop a stricture either upon stent removal or soon there after.  Stenting her bile duct did nothing to correct the reason why Lucy needed a stent in the first place, unfortunately it was a temporary solution to a much bigger problem.  Even though we would love to keep the stent in for as long as possible, it poses an even bigger risk of infection for her the longer it stays in, especially if it becomes embedded in the wall of her bile duct which is a risk of leaving it in longer than the recommended eight weeks.  We will keep her on continual IV antibiotics and anti-fungals all in an attempt to keep the "bad guys" away for as long as possible. But here's our reality, these medicines can only fight off specific types of bacteria and fungus.  Eventually, when you fight with the bug kingdom as much as we have, these meds loose their ability to fight off  bacteria because the bacteria become resistant to them.  Equally as worrisome is the fact that Lucy will be in even more pain, because an occluded bile duct hurts.

Dr. R very lovingly shared with Drew and I that doing nothing and letting her body do what it ultimately will do most likely means that she will suffer a very painful end of life in which she will need to be heavily medicated to remain comfortable.  His concern was for us as well, sharing with us that we will have to make some very tough decisions as her parents all in an effort to not prolong her suffering.  These conversations, though very difficult, are our reality these days.

Our other option we have for our girl is maximally invasive surgery in which we will reconstruct her bile duct.  Two of the brightest surgery minds at DuPont have been working hard to figure our just how exactly we can go about doing this.  One of these brilliant minds is our surgeon Dr. K who knows and loves our girl, and the other brilliant mind is the liver transplant surgeon Dr. D who has a reputation for doing the impossible.  Don't think for one moment that we don't know how lucky and blessed we are to have the outstanding medical team that we have!

The tried and true surgical method for reconstruction of a bile duct is to do what is know as a heptocholangiojejunostomy Roux en Y (not only can I spell it, I know what it is:).  Very simply, this is a complex procedure in which the surgeon takes a portion of jejunum, brings it up to the liver creating a Y of sorts.  Then attaches it to the liver creating a new conduit for bile to drain.  The bile from the liver then flows directly into the small bowel.

This option, though tried and tested, is a very very scary operation for our girl.  Her intestines are coated in every type of bad bacteria and fungus.  If we were to connect Lucy's jejunum to her liver we all fear that we would be putting her at an even greater risk for infection.  In fact both Dr. K and Dr. R believe that she will very likely develop sepsis immediately upon connection, if not then soon thereafter.  Although Lucy has survived sepsis before, this time we are fearful that the bacteria in her small bowel are more potent than the meds we could use to treat it.

Are you gasping yet?

Breath, we are not going to be able to fix Lucy's bile duct this way, the risks do not out weigh the benefits.  Instead we are seriously considering an alternative surgical procedure, alternative as in has never been done before. This procedure will involve resecting either a portion of her jejunum or using her diseased gallbladder to create a conduit which will attach to her liver creating a new bile duct of sorts.  Instead of connecting the conduit to her diseased small intestine, we will instead create an ostomy, or a hole (I know, as if the girl doesn't have enough holes in her body already) on the the exterior of her body in which the bile from her liver will drain via gravity into an ostomy bag.  The reason we want to create an ostomy is so that we can eliminate any risk of infection coming from the bacteria in her small bowel.  If the conduit or new bile duct is not connected to her small bowel then in theory bacteria from her small bowel will not be able to infect her liver, thus minimizing her risk for ascending cholangitis.

This option gives Lucy the lowest risk of infection, if it works!  However, the procedure itself is massive and as such comes with a tremendous amount of risks, the biggest being that she may not recover!  We also understand that an ostomy is no picnic, especially since we are going to have to attempt to keep her ostomy sterile.  Ostomys are typically anything but sterile.  Ideally we would all love if this procedure could be done laproscopicly which will minimize her recovery time, but that is something that has yet to be determined.  The two surgeons differ on what organ they want to use as the conduit, but both agree that no matter what organ is used this surgery has the potential of reducing Lucy's risk of reoccurring ascending cholangitis.  Any surgery is a risk, but one of this magnitude on our girl brings new meaning to the word.

Which way are we leaning... well, that all depends on when you ask me.  We do realize that there is no right or wrong decision here, what we ultimately end up choosing to do for our girl will be the right decision no matter what!  If I were to sum up our biggest fear, it would be the fear of doing more harm than good.  Our goal is and remains to give our girl the best longest life!

Just when I thought she couldn't get any cuttier.... she did!

A Walk in the Park...

Our life, it has not been a walk in the park recently, but it hasn't stopped us from taking a walk in the park...

The stars and the moon were in perfect alignment yesterday, they had to be it's the only explanation that I can think of :).  Lucy was awake and in a good mood before 5 PM, that hasn't happened in a long long time!  In all honesty, Lucy is barely awake these days, and when she is it's not necessarily a good thing.  The weather, it was beeeeautiful, unseasonably warm which had us uncharacteristically out and about with all four of our kiddos at 5 PM...wonders never cease!

The kids each took turns pushing their sister's chair while Drew and I walked a few steps back holding hands and taking in the site that is our kids, and also to protect our toes from getting run over by novice drivers.

The grass was blooming with hundreds of dainty purple flowers (Siberian squill for those of you horticulturists out there, Mom:), for which I look forward to seeing each and every spring!  We couldn't keep Sophie from running through the grass.

And we couldn't convince Lucy to put her feet in the grass...

that is until the girls talked her into it!

Megan, no one can deny that she holds a special place in Lucy's heart.

I tried to take a picture of all four them looking at the camera at the same time and before Lucy tipped over...yeah this is as good as it got.

This guy, he can roll his eyes in more directions than I thought possible, but every now and then he looks me straight in the eye, and when he does I can see straight into his soul.  Sixth grade boys, they are budding future gentlemen.  Jack is a great big brother, you should see him with his sisters but in particular Lucy, he is so so good to her.

We have a lot we are considering when it comes to Lucy's life, we are standing at a crossroads needing to make a decision to go this way or that way.  We really only have two options, both come with considerable risks!

We have not made up our minds just yet...

For now I am trying to saturate myself with moments like these, it's not every day you see her smile! 

 Your thoughts and prayers, love and support are greatly appreciated!

I promise to post a more detailed update soon, but for today I just wanted to share some of our memories with you.

A Cause for Celebration...

Today we are celebrating five unbelievable years...

Happy Birthday Lucy Grace...

we love you more than words can say!

Trying to Get Back on the Saddle Again...

I want to apologise for any confusion my last post may have caused, we were discharged from the hospital on Friday March first and arrived home at around dinner time.  We are home now and are trying to adjust to life outside of the hospital.  It is an amazing feeling being home day and night with all four of my kiddos and my husband.  Everyone is happy that we are home, but hands down the person that is the happiest...my husband:).  He makes me feel so loved and needed, just looking around my house is a reminder of that(think a much needed spring cleaning).

I'll be honest, it has not been the smoothest or easiest transition, our coming home, not that I was expecting it would be.  It has been awhile since I have taken care or our girl all by myself, all the while being a wife and mother to four of the most amazing kids (hey, it's my blog and I can boast if I want to:).  Taking care of Lucy 24/7 was a lot before her most recent issues, but it is even more now.  The minute we arrived home on Friday we were greeted by "the kids", our nurse, and infusion nurse, and also by the phone ringing.  I had to take the call as it was our infusion company calling to coordinate delivery of our weekly medical supplies for that evening....nothing like jumping in with two feet. Lucy's order is anywhere from six to eight boxes of meds and supplies weekly!  Recalling the number of meds for an entire week, TPN bags, fluid bags, med lines, lumen caps, needle syringes in 3ml, 5ml, and 10ml increments mind you, gloves, transfer caps, lab kits, dressing change kits, adhesive products, chlorascrub pads... and the list goes on and on (over 6 pages) that are  needed to take care of our girl at home is a cognitive exercise that rivals doing the Sunday New York Times cross word puzzle.  Medical inventory specialist, it's just one of the hundreds of job titles that I have.  Professional medical supply organizer, that is the job that I take on after my medical inventory specialist job has ended for the evening.

The kids, they were thrilled to see us walk in the door last Friday, they continue to keep saying "I can't believe that you and Lucy are home!".  Although yesterday, when I announced an official Marlett House cleaning day they did not sound as enthusiastic when they said, "oh yeah...Mom's home".  Each of us have had moments of difficulty, perhaps me more than anyone, but the kids have been a bit more dramatic about theirs.  I feel at times that I have divided myself into so many tiny pieces that I am going to crumble and not be able to be put back together.  Perhaps I fear more if I do crumble when I eventually get put back together I won't be the same.  Drew is always to first to recognize that I am the last to recognize the change that has occurred right before our eyes.  I think it's a survival mechanism,  I somehow attempt to try and make things go back to the way they were.  When I fail at this, because I will, it's inevitable nothing can or will ever stay the same even in the most ideal situation, I will then be able to see more clearly where we are and what has occurred.  I have processing delays, just like Lucy, only mine are more emotional delays.

It doesn't help that we are functioning on very little sleep...think newborn baby only ours is nearly 5 and has a central line infusing dozens of meds, TPN, and fluids 24 hours a day.  A heart rate/pulse ox monitor that alarms at all hours of the day and night, sometimes for false alarms and sometimes for legitimate alarms which means you can't ignore any alarm.  Connected to that baby is over fifty feet of oxygen tubing that no matter how I coil it up, inevitably, I will trip over it in the middle of the night. Our baby has over two dozen IV meds to infuse that are so strategically timed it can make even the most punctual person late if they had to keep to a med schedule like ours.  There simply is very little time in between meds to do anything else.  A GJ tube that is connected to two drainage bags 24/7 that like to pour out bile on some days and not on others which just has me going huh? most days.  One supra pubic catheter that has almost ended our straight cathing days,  that is when it does not get clogged.  This only tends to happen on the very rare occasion when I want to leave our house, this is just how our life is, we just have to learn to roll with it.  Just like with a newborn baby, you have to learn to sleep when the baby does,  we are learning.  Of course day light savings was just a cruel occurrence this weekend.

Remembering EVERYTHING, it's just like remembering how to ride a bike...not really.  I find myself having to write a lot of things down so I can plot out just how I am going to do this or that, my memory is not what it used to be, but I also have a lot more to remember .  When Lucy was born I had a labor and delivery nurse, who also happened to be the morther of four, give me some advice... "get yourself a notebook and write it down", she wasn't kidding!   I do feel like I have my groove back when it comes to taking care of Lucy, I really had no choice but to jump back on the saddle again.  As for the rest of our life, it's a work in progress.   Laundry is caught up for the most part, and we went to Costco for the first time this year.  FYI, it's better to go more often, it will save your eyes from popping out of your head when you see the finale tally at the bottom of that very long receipt.   Our house is slowing starting to look like a mama lives here too, but I don't want to give you the wrong impression, it's by no means clean.  Let's just say I am the master of the five minute pick up.

Our life is just that, our life, we are doing our best with what we have been given.  I write all of this not for your pity but to document just where we are in our life at the moment.  Coming home from a very long hospitalization with a child who is not going to "get better" but is "doing better" is a strange feeling.  I am not sure what direction life is headed most days, and sometimes I am just fine with that and other days it just plain sucks.

We're going home...

We've been here a long time...

I never know how long an admission is going to last, and for some reason I never pack for more than three days, I just don't.  In the back of my mind somewhere buried deep I have convinced myself that planning for three days is just the right amount of time.  I bet if you asked Drew he would tell you that he wishes I would plan for more days as he is the one who is on the other end of the phone when I ask him to bring me my black shirt with the scoop neck not the v-neck, and the socks with the stripes but not the thick ones the thinner ones.  Oh, and can you also bring Lucy her baby doll with the purple booties not to be confused with her purple baby, and her pajamas with the barn animals.   I say it as if she only has one pair, but seriously what little kid only has one pair of pajamas with barn animals.  So naturally Drew responds with, which pajamas with the barn animals, the pigs or the cows, or the ones that Lucy thinks are pigs but are really cows?

We have managed to live life as family in two separate locations.  Hands down the question that we get asked the most, "How do you do it?"!  The answer to this question could be a novel, or a book series if I were to write about the minute details, but the simple answer is...we are just so grateful that we can do it!  It is not easy, but we choose not to complain, and to be thankful for ALL that we have.  That is not to say that we do not feel the emotions, the pain, and the hardships, oh we do, believe me we do.  But we know that we would rather be doing all that we have to to do for our girl, for our family, than to not have her here with us at all.  It's both the blessing and curse of having a medically fragile child....you can and will never take life for granted.

Our expectations for Lucy's stent placement are very different from what is our reality.  There is no such thing as a "permanent" stent, more like long term would be a better description of the type of stent that was placed inside of Lucy's bile duct.  Metal bilary stents are typically placed in adults as a palliative measure for those who have some type of malignancy or tumor in their bile duct that is deemed inoperable.  The patency of these types of stents is approximately 6 months, the prognosis for the individuals who require a metal bilary stent is sadly often less than six months, hence the confusion with the word permanent.  There are no studies done on how long a metal bilary stent can be placed in a child's anatomy, it is assumed based on a child's size that they should stay in for half of the amount of time as an adults.  Even less is better, as the longer it remains in the greater the risk that the stent can become embedded in the wall of the bile duct, increasing the infection risk, and even worse not being able to be removed.  I tell you all of this because for our girl this means that the stent that we placed in her bile duct is a very short term solution for a long term problem.  The doctor at Hopkins agreed to placing a stent in Lucy as a palliative measure, but told us that he agreed with the Jefferson doctor in that the only "fix" for Lucy's bile duct is maximally invasive surgery.

We were disappointed when we realized this,and adding to the disappointment was what the doctor learned about Lucy's bile duct when he went into place the stent.  It is significantly damaged.  His recommendation is to have the stent removed in 8 weeks.  It is thought that once the sent is removed Lucy will most likely develop another stricture either upon removal or soon after.  This is not what we were expecting, in fact we choose to place the stent in the hopes of avoiding having to put her through major surgery.

Once again, we are now at a crossroads.  Our options for our girl are few, and are what we are describing as having to decide between God-awful and horrific choices.  We are putting off doing her gallbladder surgery at the moment as we have even bigger surgery options to consider.  I will not detail our options at the moment as we are still trying to gain clarity on the issues.  What I will say is that we need some time... time to think, time to process, and time away from this place.

Lucy is on continual IV antibiotics and IV antifungals, what we refer to as the big guns, all in an effort to keep the infections at bay.  She is and has been on a regimen of continual antibiotics to control the overgrowth of bacteria in her intestines.  We are doing all that we can at the moment to keep her infection free.  Health wise, Lucy is stable,  she is about as good as she is going to get at the moment and for that we are so very very grateful.  After having some serious yet more difficult discussions with the good doc on Monday morning, he said to me during our conversation, "do you want to go home, and take a break from this place, for a few weeks while we gather information and make some decisions...", my response was simply... "yes"!

You know the saying "it takes a village...", if you knew the number of people in our life who make the impossible possible for us to take our girl home and live life, I think you might tell me that it takes more than a village, it takes an entire city!  Lucy's level of care is greater than ever, but we are going to go home for a few weeks and do what we do best...live life!  I will admit that I am nervous, like I said we have been here a long time, having around the clock nursing care and immediate medical attention when we need it is very comforting.  We are all going to have to adjust to life together again.

The next few weeks are full of special celebrations, a certain little girl's 5th birthday is just around the corner, and another one's 38th birthday too:).  Birthdays are all about celebrating someones life, and that is just what we intend to do...celebrate life!

We're going home!

(By the way...please don't say anything to our kids, we're going to surprise them!!  We're veterans at this, we know better than to say we are coming home until we have the actual discharge papers in hand...even so we have been readmitted once after signing the paper .  Oh and don't be fooled, just because I initially only packed for three days, does not mean that we have only three days worth of stuff to take home.  It's quite the contrary, we have three months of stuff to take home :)