Change of Plans...

(This picture was taken the morning of Mother's Day, it's not everyday that we get a picture of all four of them, all in the pj's:)

Home sweet home!  After a difficult and emotional week, we came home late Friday night.  After several emotional discussions, we are postponing Lucy's stent removal for a few more weeks.  When this was decided, we quickly made plans to come home.  Lucy is not great, but she is doing better.  We can do for Lucy everything that they were doing for her in the hospital,  with a lot of help.

During surgical rounds Wednesday morning there was an interesting discussion between the docs about Lucy's stent, in particular its size in relation to her bile duct, it's "ginormous" according to many of the docs.  Our surgeon had some thoughts about the entire procedure and wanted to find out more specifics about the stent in Lucy's bile duct, so he called the doc in Baltimore who did the stent placement on Lucy to discuss his concerns.  Research shows that these types of stents in adults begin to embed into the wall of the bile duct after six to eight weeks.  However, there are no studies done in pediatrics to determine how long it takes before it embeds into the wall of a child's bile duct, simply because they are not commonly used in pediatrics.  After doing another cholangiogram on Thursday to check for leaks we all felt that even though the study showed that there are none, her site needs more time to heal before we stress it by putting it to use.  After weighing the risks vs. the benefits yet again, we all felt that it was best to wait a few more weeks before testing and using her new drain.  If we use it too soon the risk of it leaking, making her very sick, is very real.  The risk of her stent embedding into the wall of her bile duct is very real too, but is worth taking when considering the alternatives.  We need this to work!

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

Our Life...

On Tuesday I coordinated a pick up and drop off schedule that makes international travel seem easy.  Despite having a daughter who is laying in a hospital bed fighting for her life every day, life continues to go on around us.  It just dose, and some days, well... it just hurts that it does.

I have yet to figure out how to be in two, three, or even better four places at once.  When I do I'll let you know:).   I had a heart felt conversation with our palliative care counselor as we ran into one another in the hall.  I shared with him how God-awful difficult and painful it is for me to leave Lucy so that I can do any one of the millions of other things that I need to do for our family, our three other kiddos.  If I am being perfectly honest here, I am not good when I am not near or around Lucy, I'm just not!  I think I do a pretty good job of keeping my feelings to myself all in an effort to be able to do all that I have to do, but if you could visibly see all of the pain and the worry that flood my veins and weighs heavy on my heart when I am not with her you would cry with me, for me.  The flip side of all of this is that I feel a tremendous amount of guilt for my other kids.  Guilt that even when I am physically there with them, if Lucy is not with us then neither am I entirely.  Our counselor very lovingly shared with me his thoughts on this dilemma, "when I can't be in two places at once, I find that I have to try very hard to just be present with where I am".  My sentiments exactly!  At times it can be very emotionally and physically exhausting for me to try and be present in the moments when I am not with Lucy.

Drew and I attended an orientation meeting for families of students entering middle school on Tuesday night...yes, gulp, middle school.  As we sat and listened to the principal talk about the transition to middle school and how in two years our kids are going leave middle school a different person from when they enter, I leaned over and whispered in Drew's ear, "This is so surreal!  Are we really old enough to be the parents of a middle schooler?".  Drew sweetly whispered back to me, "I am, but you're not babe ;)".

Can't they just stay little, that was the thought running through my head as I was driving back to the hospital after leaving the meeting.  The tears, they just flowed.  Nights like these are reminders of all that our kids have been through and need to go through in order to grow up, naturally my heart and my mind wandered and thoughts of Lucy came flooding in... I think of how very different her growing up process looks.  It hurts, oh God it hurts.

Decisions about life and death are everyday conversations in our life.  I really don't know how to live any other way anymore.

***A quick update on today, the cholangiogram showed NO leaks, this is good!  Her incision site is still healing and no doubt needs more time to heal.  I will write more about this tomorrow.  I am waiting to discuss some things with the good doc in the morning..

Mama, I awake...

Look who's been awake a little more, not a lot more, but a little more.  If you want to see those baby blues open, you had better come and see them right when they open because they are quick to close and stay closed for hours.

When she has been awake she is a bit of a craby pants, not unlike her pajamas, but who can blame her.

Tomorrow we are going to take a trip down to IR (interventional radiology) to do a cholangiogram.  We are looking for any leaks in and around her new tubie.  As is our usual prayer, we are praying for clarity from this study.  

Sleeping Beauty...

This picture pretty much sums up our day.  

Lucy was only awake for brief periods of time today.  I have to keep reminding myself that she is healing, and that takes time.  Our pain management plan had some glitches which means we are working on it.  Her incision site looks good, we removed the dressing and replaced it with a clean one.  Today was also spa day in Lucy's crib, nobody this cute should smell so sour.  Much to my surprise Lucy cooperated and dare I say even enjoyed getting a shower cap shampoo.  Getting clean was exhausting and so Sleeping Beauty closed her eyes and went back to dream land.

E-Cards...

Back by popular demand here is the link to send Lucy an e-card.

Lucy's room number is 3407 3707.

We love hearing from you, your thoughts and prayers, love and support mean so much!

An almost quiet Sunday...

Lucy is about the same as yesterday - translation, she is stable, stable is good.  I wrote these words in an  e-mail earlier this afternoon.

Lucy has some healing to do and as such her body is tired, she slept comfortably for the most part today.  However, she did wake up bright and early this morning so that she could play.  One of her favorite nurses worked with us last night and Lucy wanted to make sure she had some extra play time with her this morning.  Mommy wasn't quite ready to wake up at the crack of dawn, and thankfully I didn't have to because Lucy was given her very own blood pressure machine to play with.  Yep, you read correctly, Lucy is obsessed with the portable blood pressure machines, obsessed I tell you.  Play imitating real life, some kids play school or house, ours plays medical babies.  She was so happy to have the machine to play with I was able to get two more hours of sleep, two-whole-entire hours more!  I'll give you one guess what she is sleeping with tonight :).

Pain team came by and wanted to take Lucy off of her dilaudid PCA, what can say, BIG mistake.  She was one miserable little girl when the kids came to see us this evening.  Megan even said, "I hope Lucy is nicer tomorrow".  We have been playing catch-up on pain control tonight, not fun, not fun at all!  I wish we could just hang her PCA back up, but instead I think we came up with a pretty good compromise that is pretty close to a continuous infusion.  Let's just say I'm just glad I'm not the one administering all of these meds tonight.

Slow and steady, that seems to be the pace.

Today...

After a rough night, I'm happy to report that our day was much better.  Lucy is comfortable, which is amazing, considering the amount of holes in her belly.  She slept a lot today, but when she was awake she was pleasant.  Lucy has an On-Q Post-Op Pain Relief System strategically located near her largest incision site which is delivering a local anesthetic, I love that they did this for her.  She also has a PCA of dilaudid running which is helping to keep her comfortable as well.  Once we got on top of her pain last night, it has been much easier to stay on top of it.

Lucy woke this morning with no fever.  She had two spikes in temp last night that we believe was as a result of a shower of bacteria that spread throughout her body.  There was some fear of what antibiotic we would put her on if her temps did not come down, ugh I hate that this is our reality.  Thankfully after two doses of Zosyn,  no more fevers.

Although the procedure did not go as we had hoped, her post-op course is going better than we expected.  We moved out of the PICU and back up to "our unit" late this afternoon.  Once we arrived back to familiar territory an albumin infusion was ordered followed by a whopping big dose of lasix to begin the process of deflating Lucy.  She came out of the OR looking like a balloon.  I'm always amazed at how quickly the albumin-lasix combo works, Lucy looks more like Lucy again tonight.

The FFP infusion worked as well,  Lucy's clotting studies looked better this morning, her lactate was also much lower.  Lucy is in need of a blood transfusion, but we held off on doing that today for fear of fluid overloading her, we'll do it tomorrow.

Dr. R came in to see us early this morning even though he was not the one on call this weekend.  Dr. K called him last night to give him all of the gorey details.  After a discussion about how things went we all agreed to just take things one day at a time for the moment.  After agreeing on this he told us that we have a tentative date for stent removal on May 31st.  One day at a time... right!

Sleeping Soundly...

Some details about today...

Lucy is doing what Lucy does best, she is keeping everyone on their toes.  She is in pain, has a fever, and is down right irritable!  She is where she needs to be at the moment, her care is being managed by the PICU nurses and docs. For me to say this is means that we really DO need to be here, we try and avoid the PICU at all costs.  It's nothing personal with the unit, it's just that we have a place in this hospital where we call home and the people there take wonderful care of our girl. We are monitoring Lucy very closely for any signs of infection, blood and urine cultures were done the moment she arrived to the PICU.   It has been a non-stop med marathon in here since leaving the OR and arriving to the unit a little before 7 pm.  Lucy's body is stressed, she is telling us so, as is her lab work.  She had quite the elevated lactate level when she arrived in the PICU so extra fluids and bi-carb were ordered to see if we could neutralize her levels.  Lucy's blood clotting labs were off  putting her at risk of bleeding,  we are going to run an infusion of FFP (fresh frozen platelets) to help minimize this risk.  Thanks to those of you who donate blood, it truly saves lives.

Dr. K was able to access the gallbladder laparascopiclly, but then had to switch to an open procedure to finish.  Lucy's gallbladder is very diseased, it is small and necrotized we suspect from lack of use, especially since placing her stent.  You know the old adage, if you don't use it you'll loose it.   Lucy has about a 2 inch incision where her new tube is stitched in place.  Dr K gave her a local pain medication that is being administered directly to her incision site, we are being told that this will help her a lot.  I'm hopeful that it is helping, but her breakthrough pain has been tough.  In addition to the local anaesthetic she has a PCA pump of dilaudid running continuously as well as PRN doses every two hours as needed, and she has needed them.   At one point tonight as she was crying she told us that it hurts to cry and even breath...talk about breaking a mama's heart.  On the other hand I was so proud of her for telling us this because more often than not Lucy will just withdraw from you when she is in pain.

One of our fears in doing this surgery was the fear of the gallbladder tearing or shredding and having bile leak into her interstitial tissue.  This happened, her gallbladder tore and shredded as Dr. K was suturing it in and as such we are fearful to allow anything to drain through her gallbladder at the moment.  Dr. K caped off the drain and wants to wait a week to let things heal, repeat a cholangiogram, and then make a decision as to whether or not he wants to open up the drain to allow bile to flow through it. As long as the stent is in place, bile is flowing down and out, when we remove the stent that is truly when the drain will be put to the test.

As for her bladder, things went smoothly in that area.  An 18fr catheter was placed so that we can allow for better drainage.  So far so good.

When discussing the procedure after the fact with Dr. K we all agreed that we need to wait and see how things go before making any hasty decisions.  Although we planned on complications, it doesn't make them any easier to experience.  The phrase, "it's Lucy" was uttered more today than usual.

Drew and I are standing vigil tonight at Lucy's bedside while our other three kiddos are sleeping at our friends house.  The attending who is on in the PICU just so happens to be one of our palliative care docs who knows Lucy and understands the phrase "it's Lucy!".

Quickly...

I want to quickly update all of you.   They are just wrapping up at 6:00 pm.  Things did not go as smoothly as we would have liked, but a drain tube was placed in her gallbladder.  We are not sure if this is going to work, but for now the stent is still in place so things will continue to drain as long as it's there.  Things were more difficult than expected and as such we are heading to the PICU, they will be better able to manage her pain tonight.

For now, I need to keep reminding myself to breath.

Thanks as always for your thoughts and prayers, love and support, we appreciate it very much.

A few things...

Lucy's gallbladder is shredding as they are trying to suture it to the skins surface, this was something that we were afraid of.  They are going to do a dye study to see where things are flowing. If they can't get it to adhere to the skin's surface then we may have to close her up and reassess.  Drew and I are sitting in the shade outside the PACU by the fountain, praying, worrying, reminding each other to breath.  

4:17 PM

Dr. K was successful in laparoscopically locating the gallbladder and is in the process of placing a 28fr Malecot catheter in her gallbladder.   We were told that Lucy is behaving, Dr. R will be so proud of her, she is actually following his orders :).

Here we go...

At 2:46 pm we kissed Lucy good night and said we would meet her at the ice cream truck in her dreams, she has dreams of eating 100 scoops of " only anila"(only vanilla!) ice cream.

I will update when we know more...

Running Late...

The OR is running a little behind, it's 1:45 pm and we have yet to go downstairs.

Lucy had an EKG and echo this morning to rule out any cardiac issues, she has been having episodes of super ventricular tachycardia that appear to have resolved since monitoring her more closely :).  All was good, which makes us all happy especially anesthesia.

I will update when we know more...

Thanks for your thoughts and prayers, love and support!

Anatomy of My Sister...

By : Sophie Marlett

When Sophie was finished with her drawing, she came to me and asked, 

" Now Mom, where's her new tube going to go?".

Decisions, Decisions...

Last week we met with our surgeon to discuss our options for Lucy.  To say we were more than a little nervous about having this discussion, a discussion in which we were faced with having to make a decision on which way we were going to take Lucy's life, would be stating the obvious.  As I outlined in a previous post, we are faced with some really tough choices, having to choose between one or the other, well that's like trying to choose which of my children I love the most - it's impossible!

Thankfully (not sure if this is the appropriate word, but it's the best word I can think of at the moment), we were given another option, an option that Drew and I are affectionately calling option 1.5.  It's falls in between our two options; our first option of doing nothing to try and fix Lucy's bile duct issues, and option two of doing everything we can to try and fix Lucy's bile duct issues.  Option 1.5 is a less invasive procedure, but one that still involves surgery.  However, it is a surgery that has been done before, but not exactly for the use we are intending.  Option 1.5 was presented to us  as an option that could help alleviated some of Lucy's pain by helping to reduce the amount of pressure in Lucy's common bile duct.  This option involves surgically placing a drain in her gallbladder, also known as a cholecystostomy.  A quick plumbing lesson on bile drainage.  Bile is formed in your liver and flows into the gallbladder.  It then exits into the common bile duct, where it continues it's journey down past the pancreatic duct opening and into the duodenum (the top portion of the small intestine).  By placing a drain in Lucy's gallbladder we are hoping to intercept the flow of bile from entering her common bile duct (CBD) by having it drain out of her gallbladder into a drainage bag.  It is our hope that by reducing the amount of bile entering her bile duct we will reduce the pressure inside of her biliary system.  It is the pressure from the accumulation and back up of bile that is thought to be causing Lucy so much pain.  If we reduce the pressure we are in turn hoping to reduce her pain.

There are a tremendous amount of unknowns with this procedure.  We know Lucy's gallbladder does not function, we confirmed this when we did a HIDA scan back in January.  It is full of sludge and stones, and in all honestly needs to be removed.  But if we keep it, create a stoma, and drain it we believe that this diseased organ can be used to serve a purpose.  That is if the organ isn't too diseased.  We really will not know how things look until we go in and see it for ourselves.  Our surgeon, Dr. K, is giving us 50/50 odds at being able to do this procedure laparoscopically.  However any procedure, be it laparoscopic or open, in which we have to work around or near Lucy's diseased intestines poses a huge risk of infection.  The risk of infection or sepsis is very real with this procedure!

Dr K is not sure what device he is going to use to create the drain, but we did rule out creating any sort of ostomy, bile is just too acidic for an ostomy.  Again he is going to have to see what things look like and then make a call as to what hardware he is going to use for a drain.  Where exactly Lucy's gallbladder is internally also plays a factor, this will only work if we can create a stoma that will allow bile to drain via gravity.  It's prime real estate on Lucy's torso, her central line is placed on the same side of her body as her gallbladder, albeit higher up, but nonetheless it takes up one fourth of her torso. Her GJ is somewhere on her middle left, and her supra-pubic catheter is smack dab in the middle of her body about half way below her belly button.  Her supra-pubic should hopefully not interfere with the placement of this new stoma :).

This surgery does nothing to eliminate Lucy's recurring cholangitis infection risk as her bile duct will still be connected to her diseased small intestine.  Our plan to keep the infections at bay is to keep Lucy on the "big gun" antibiotics and anti-fungals indefinitely.  As for the stent, the best way to remove it is via ERCP, which means another trip/procedure back to Johns Hopkins in Baltimore sometime in the very near future.  It has to go, it poses an even bigger risk of infection if left in place.  We understand that Lucy will most likely develop a stricture either upon stent or soon thereafter which is why draining her and alleviating the pressure is so import.

We are back at DuPont tonight, we arrived earlier in the day today to prep for surgery tomorrow.  We are moving forward with this procedure because it has the potential of improving Lucy's quality of life, if it works.  If it doesn't work, it is somewhat reversible in that we can pull the drain and close the stoma, and possibly remove the gallbladder if need be and her anatomy will remain in tact for the most part.  We really won't know if it works until we try it.  We are going to be holding our breath a lot in the next few weeks, hoping and praying for a positive outcome.  If things don't go as planned we'll have to go back to the drawing board so to speak, but the fear of putting Lucy through anything more invasive has us all worried!  We are planning on going back to Hopkins just as soon as she recovers from this surgery.  We have surpassed the eight week mark with this stent and as such we are left wondering if it will come out, God I hope so, but again it is another unknown.

Lucy is scheduled for surgery at around 2:00 Friday afternoon, I know many of you won't read this before that so I just wanted to be clear on her her time for all of you prayer warriors out there.  At the same time that Dr K is doing his thing our urologist is going to be doing some work on Lucy's bladder.  We are hoping to place a larger catheter in Lucy's bladder stoma to allow for better drainage.  To say we are nervous is again stating the obvious, but our reasons for moving forward with this fall in line with what we have wanted for Lucy all of her life.

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!