Deciphering pain...

"My bladder hurts!", Lucy has been consistanly telling us this for almost two weeks.  For her to localize where the pain is coming from is quite amazing, really!  She is has not wavered in telling us where she hurts, except for the two times we went and stuck something in her bottom - then she told us, "My bottom hurts!", smart girl.

What we are trying to figure out is the type of bladder pain Lucy is experiencing now.  Is her pain coming from the inflammation and erosion of her bladder, or is it pain from bladder spasms, or both.  It is obvious that Lucy is in pain, but if it's pain from bladder spasms then we need to treat it with a different medication than if it is pain from erosion of her bladder.  

Lucy hasn't leaked urine since last week, which had us all wondering if she was still experiencing bladder spasms, especially since we injected her bladder with twenty-one injections of Botox on Friday in hopes of stopping the incessant bladder spasms.  Today Lucy underwent a urodynamics study to measure the contractions of the bladder as it fills.  Yep, the study is about as uncomfortable as it sounds.  We learned from the study that Lucy is not experiencing bladder spasms, which means that the Botox worked, or maybe the Oxytrol, or maybe both.  Lucy's bladder pain is most likely pain from the inflammation and erosion of the interior of her bladder.  This type of pain is best controlled by using narcotic medicines, which is what we have noticed appears to be working best to control her pain.

All of the narcotics are making our girl very sleepy and "out of it" looking.  We are all hoping that with the change in catheter drainage systems that Lucy's body will start to heal and she will not have to live with so much pain.  Only time will tell, for now we are doing our best to keep her comfortable.  

Thank you for all of your comments from yesterday's post - your thoughts and prayers, love and support mean so much.     

Clarity…

Warning…this is a long post, with lots of information.  It may cause some confusion, but hopefully it gives more clarification.

Clarity has always been our prayer when it comes to deciphering Lucy's issues.  It sounds like a simple prayer, but oh Lord we recognize that it's anything but simple.

A little background medical history on our girl, humor me for those who have been following our story for a while, it's always good to have a refresher course every now and then.  Lucy suffers from mitochondrial disease, a disease of energy metabolism in which the body is unable to effectively convert food and oxygen into energy.  The mitochondria, if you remember back to your biology days, are responsible for creating energy or more scientifically adenosine triphosphate(ATP).  Mitochondrial disease is a cellular disease, mitochondria are present in every cell of our bodies minus our red blood cells; therefore, mitochondrial disease can affect the whole body. Persons who suffer with the disease experience a myriad of symptoms that range in severity from mild to severe.  Unfortunately for Lucy, her issues tend to gravitate toward the more severe side.  Mitochondrial disease is an incurable disease at the present time.  Sadly, there are little to no proven treatments or drugs that can be used to treat the disease - no FDA approved drugs or proven therapies.  The "mito cocktail" is a custom combination of vitamins and co-factors that are, for all intensive purposes, used to assist the body with metabolism.  It is by no means a proven therapy for patients with mitochondrial disease.  Instead what is done is to treat the symptoms of mitochondrial disease.  For our girl that means treating her failing intestinal system, her dysfunctional nervous system, her seizure disorder, her severe hypotonia which affects most of the muscles in her body, her cognitive and physical delays, her body's inability to produce sufficient blood cells which impacts her immune system, her pancreas, gallbladder, liver, stomach, small bowel, colon, bladder…

With every treatment option presented for Lucy there have been or are risks and benefits, pros and cons, quality of life hopes and concerns.  We have weighed every decision that we have been confronted with by asking, "Will whatever we choose improve Lucy's current quality of life?".  Please recognize that the decisions we have made are just that, decisions that WE, not you, have made.  I mean this in the nicest way, if I have learned one thing in all of things we have been through in Lucy's five years of life, it is that you never know how you will react until you yourself are faced with having to make a decision.

Intervening with one area of the body often has a domino effect when dealing with a systemic disease - you fix one problem only to create more problems.  Problems that are hopefully also treatable or at the very least livable.  Intervening with Lucy's falling intestinal system disturbed the delicate balance of bacteria living in her body, this disturbance has caused issues throughout her body that has lead to our "war on bacteria".  While simultaneously fighting this war, we are fighting with disease progression - one organ after another being affected by the disease.

Which leads us to our most current issue, Lucy's bladder.  Lucy was diagnosed with a neurogenic bladder at around three years of age.  Similar to her failing gastrointestinal system, the nerves and muscles in her bladder do not function correctly, they fail to signal her body to urinate leading to urinary retention that is painful and infection causing.   In an effort to assist her body with this process we began intermittent catheterization years ago.  We cathed our girl this way until last year at around this time when her bladder and urethra became too damaged and diseased for us to drain her urine like this.  We decided to surgically place a hole in Lucy's bladder just above her pubic bone in which we placed a tube that continuously drains urine.  This procedure was know as a suprapubic cystostomy.  We chose to do this as a comfort measure to help alleviate the pain and discomfort associated with straight cathing.  Here's the thing, a foreign object in the body is not natural, our bodies were not designed to accommodate non-human parts.

Upon admission on December 16th we discovered, in addition to severe pancreatitis, that Lucy's urine culture grew positive for yeast.  These two issues are seemingly unrelated; however, when Lucy's body gets stressed from illness it seems to exacerbate other problems in her body - the domino effect I was describing earlier.  Yeast is very sticky and likes to stick to plastic tubing, as such we knew that we needed to replace the tube in Lucy's bladder to try to eliminate it.  Here's the thing with yeast in the bladder, once it resides in there it's not really going to go away - fungus is tricky like that.   In reality, there are several organisms that reside inside of Lucy's bladder, for the most part they live there peacefully - what I mean is that they are not infection causing.  When the bacteria and yeast overpopulate and cause an infection, that's when we treat.  Replacing the tube in her bladder appeared to eliminate the overpopulation of yeast.  Repeated urine cultures after we replaced the tube have all come back negative for yeast.  However, on Monday when Lucy was in the OR, they replaced her tube again and sent the tip of the tube off for culture.  We just learned that it grew positive for yeast.  Like I said, yeast is tricky.

We are in a very difficult spot when it comes to treating infections for many reasons.  Lucy is on continual "big gun" IV anti-fungals and rotating antibiotics all in an effort to keep her infection free for as long as possible.  We recognize that this is not a standard treatment option, but when it comes to Lucy the standards are different, they just are.  Our reasons for pursuing this treatment option were truly quality of life reasons.  Keeping the infections at bay for as long as possible hopefully means less time living in the hospital and more time living at home.  On the flip side, it also means that if an infection occurs it will most likely be difficult for us to treat.  There are not as many anti-fungals as there are antibiotics in the pharmaceutical world, which means our med options are limited.  Even more limiting is the fact that Lucy has had multiple fungal infections in her life that we have treated.  The fungus that she is growing in her bladder is resistant to many of the anti-fungals that we have used before.  The only other anti-fungal option left for us to treat the fungus in Lucy's bladder is Amphotericin - nickname "ampho-terrible".  The yeast in her bladder is there, but not infection causing, therefore we are holding off on treating her for this.  In all honestly, we are afraid of two things.  One, her becoming resistant to ampho if we were to use it to treat the yeast in her bladder, it's the only remaining anti-fungal option we have if she were to get a systemic fungal infection.  We don't want to have to use it unless we really need it!  And two, our team is terrified that Lucy would not survive an ampho infusion, she is too medically fragile and the medicine is really that awful!  

It's evident to everyone that knows and loves our girl that Lucy is in a lot of pain!  Since a fungal bladder infection was not the source of Lucy's excruciating bladder pain, it begs the question then, what is?   Our best guess is that the pancreatitis set off a series of events inside of Lucy's body, stressing her bladder which has lead to an increase in bladder spasms and even more inflammation in her bladder, an already very diseased organ in her body.  It was the straw that broke the camel's back, so to speak.

As her belly pain from the pancreatitis was beginning to subside by the end of last week, her bladder pain was dramatically increasing.  Incessant bladder spasms were causing Lucy to actually soak through diapers, it's the propulsion from the bladder spasms that is causing this because Lucy cannot urinate on her own.  Lucy's complaints shifted from telling us her belly hurt, to crying that her bladder hurt her "a lot".  We attempted to instill a numbing medication in the bladder in hopes of alleviating her pain, instilling anything into the bladder caused her more pain!  We discontinued all meds into the bladder and switched Lucy to a topical oxybutynin patch in hopes of helping control her bladder spasms.  Sadly the patch at the dose Lucy requires causes vision loss in our girl.  For now vision loss is something that we are willing to accept if comfort can be achieved.   The correlation between Lucy's vision loss and the patch was a discovery our urologist put together last year when we were using the patch regularly.  We discontinued the use of the patch post suprapubic surgery because for a while there Lucy was not experiencing bladder spasms.  Unfortunately, bladder spams started plaguing Lucy again in September when she became sick with sepsis from cholangititis.

Pain management has been our number one priority, but comfort at all costs is something we are not willing to say - at least not yet.  Comfort at all costs means just that, we will achieve comfort no matter what.   We increased Lucy's pain medications dramatically over the course of the past 11 days, but Lucy tends to walk a fine line between pain control and breathing.  By Sunday her pain had increased dramatically and during rounds Lucy clearly showed the team just how much pain she is experiencing.  Our main doc was compassionate and quick to respond as was the Chief of Urology who was on call and meeting Lucy for the first time in person.  Sure he knew of Lucy, she kinda has a reputation around the hospital for being an exceptional gal, but to meet her in person for the first time under these circumstances made an impression.   Both docs agreed that shortening the interval between her Valium dose from every three to every two hours could help, Valium is a very effective med for bladder spasms.  In reality it didn't help, instead it suppressed her respiratory drive enough that she was in need of quite a lot of breathing assistance from Vapotherm.

Very early Monday morning while switching Lucy over to Vapotherm we simultaneously made the switch to a PCA pump of dilaudid.  This allows Lucy to receive a continuous infusion of pain medicine as opposed to receiving scheduled doses of pain medicine every few hours in which the medicine was wearing off before her next scheduled dose.  Our girl was riding the awful roller coaster ride of scheduled dose narcotics in which you experience the peaks and valleys of the medicine.  The PCA allows a continuous infusion of pain medicine that eliminates the peaks and valleys.  It has made a difference, thankfully, but as her pain persists her tolerance to the medications are building.

Over the course of the past several months Lucy's picky picky bladder has been a source of discomfort and frustration.  Cultures have for the most part been negative for bacteria, with the exception of the positive yeast culture, but her urinalysis has shown persistent white cells over the past month.  All of her bladder issues, combined with the dramatic increase in pain since admission, prompted us to take Lucy to the OR on Monday to examine her bladder and assess the situation.  While in the OR we also planned on instilling Lucy's bladder with a "bladder cocktail" containing a steroid for inflammation, heparin to dissolve blood clots, gentamicin for antibiotic coverage, and marcain for pain control  While preforming the cystoscopy they discovered severe inflammation in Lucy's bladder, most likely due to disease progression, and also from having and indwelling catheter in her bladder for so long.  In fact they discovered the indwelling Foley catheter that we have been using to drain Lucy's bladder has been causing constant irritation to the bottom of her bladder, the trigone, the most sensitive part of the bladder.  This constant irritation has lead to erosion to this area.  The debris and sediment that has been clogging Lucy's bladder is caused by tissue and cells from the erosion of her bladder.  Poor girl, the Chief of Urology who preformed the procedure came out of the OR several times to discuss his findings with Drew and I.  He attempted to described to us the type of pain that Lucy is experiencing, I will spare you the description and just tell you that it is an excruciating pain!  In an effort to stop the constant assault to the bottom of her bladder, we had to change the type of drainage tube we were using from a Foley catheter to a pigtail drain.  A pigtail drain, although a bit stiffer than a Foley, does not have a poky end, but rather a curly end that forms a loop when installed, hence the name.  Our hope with this type of drainage system is to prevent the tube from poking into the bottom of Lucy's bladder.

In spite of changing the type of tube and instilling the "bladder cocktail", Lucy's bladder pain and bladder spams continue to persist.  We took Lucy back to the OR yesterday to give Botox injections into her bladder in an attempt to stop the incessant bladder spasms that are so painful.  Lucy is being described as having interstitial cystitis/painful bladder syndrome, but she does not really fit the profile for the disease.  In reality, Lucy's bladder issues are related to her underlying mitochondrial disease.  We have intervened with so many areas of Lucy's body, prolonging her life, more and more organ systems have become affected by the disease.  We are not sure if the Botox will work, but at this point we felt that it was worth trying.  So far we have not noticed any changes, if anything she is more uncomfortable from the procedure and bladder spasms are still persistent.

While discussing the enormity of our situation with the many loving and caring doctors in our life, I keep saying to them that I can't believe it's her bladder that is going to take her down, her bladder.  Their overwhelming response is it's not really her bladder that is going to take her down, it's the disease progression, her bladder just happens to be the biggest source of pain and discomfort at the moment.  

We are in a very difficult position, at another cross roads.  We are running out of medical options that could improve Lucy's comfort and quality of life when it comes to her bladder.  At times it seems almost unbelievable that we are here, mainly because we have always had the most "out of the box" thinking and extreme options presented to us all throughout Lucy's life that have, for the most part, provided her with the "best longest life".  It feels surreal to be at the point where we have exhausted all of those options and now need to consider "comfort at all costs".   We are not giving up hope, we will never lose hope.

Back to the OR...

I am working on a post in which I detail more of the issues surrounding our current issues.  I promise to post it soon, I just need more than a few minutes here and a few minutes there to get my thoughts out clearly.  Soothing a very cranky and uncomfortable girly has been a full time job as of late.  I am a mom, comfort is what I do best.

In the meantime I wanted to quickly share with you that we are heading back to the OR today, sometime around noon.  We are going to make some changes to the new drainage system we placed in Lucy's bladder on Monday in hopes of having it drain better.  We are also going to attempt to inject Lucy's bladder with Botox to help prevent the incessant and extremely painful bladder spasms.  We really don't know if this will work, but at this point it's worth trying.  Our fabulous urology team(really and truly they are THE BEST) is also going to look inside of Lucy's bladder and asses whether or not the "bladder cocktail" they instilled in on Monday helped with the severe inflammation that is also a source of so much pain.

Your thoughts and prayers, love and support are once again very much appreciated.  Here's hoping and praying that what we do today makes a difference for Lucy, she is in so much pain.

'Twas the night before Christmas...

Many thanks to all of Santa's elves for making ALL of this possible!

Silent Night...

Ah, not really!

We are making the best of a not so ideal situation, we're spending Christmas together in the hospital.  

There's a slumber party going on in Lucy's room tonight!  Tomorrow morning I will receive the best Christmas present ever, waking up with all four of my kiddos and my husband on Christmas morning in room 3708 "our home away from home".

Merry Christmas to all and to all a good night, hopefully ;).

Back in our room...

Just wanted to let you know that we settled back in our room.  Lucy's procedure proved to be informative.  Again I will include more detail in a later post.  Thank you for your thoughts and prayers, love and support.

In the OR

I apologize for the lack of detailed updates.  When I have more time I will detail with you some of the events that have lead us to where we are currently with Lucy's most recent issues.  Lucy is currently in the OR having her bladder scoped in an effort for us to try and figure out why she is in so much pain.  Prayers for comfort and clarity are once again welcome.

Painful...

Lucy, Lucy, Lucy…oh Lucy!

My mama heart aches for you and what you have had to endure and what you are having to endure at the moment.   It's painful for me to witness you in pain! 

I will write in more detail about Lucy's most recent issues in a later post.  For now I will simply say that Lucy's bladder pain is beyond what any of us who know and love our girl ever thought possible.  Like many of the organs in Lucy's body, her bladder is affected by her disease and as such is another deteriorating organ system. 

Pain control is proving to be extremely difficult.  We have been increasing her pain meds dramatically over the weekend all in an effort to make Lucy's pain tolerable.  Keeping Lucy breathing and her pain tolerable, it's difficult. 

I am currently watching and listening to our girl struggle to breath, she is retracting so badly.  We just made the switch from her regular oxygen to Vapotherm,  a high flow oxygen delivery device.  She is proving to everyone that she is in need of a lot of breathing assistance at the moment. 

Prayers for Lucy's peace and comfort continue to be needed.   

From where I sit...

 Lucy could use some prayers for peace and comfort,

 as well as some prayers for clarity on her issues. 

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

Tomorrow's another day...

It's late and I am just heading to bed, but I wanted to just give a quick summary of our day….

It was much the same as the day before, not much change.  Lucy's bladder is really bothering her.  We are waiting on culture results to find out specifics on the yeast.  Some of our docs are inclined to treat it right away while others are waiting for more information.  I see the logic and reasoning behind both points of view, either way we need to find a way to keep our girl comfortable.

I am hoping tomorrow we will gain more clarity on the issues and discuss a plan for moving forward.

I sent an email to a friend earlier today in which I told him that I was relinquishing all control over the calendar - in other words I was going to stop obsessing over whether or not we were going to be home for the holidays.  Then I asked him if I sounded convincing, to which he simply replied "no".  I'm trying, really I'm trying.

Thanks to Lucy's school friends for making her the most creative paper ornaments for her Christmas tree, they are beautiful!  Our CCF family gathered tonight to celebrate the holidays together by making ornaments.  We hung them on a small tree that afterwards was brought to Lucy's room for her to enjoy. It's beginning to look a lot like Christmas:).

Thanks for all of your thoughts and prayers, love and support.

Mama, I don't feel good...

Our secondary bladder issue, has become a more of a major issue.  Bladder pain was a huge problem for our girl today.  We attempted instilling her picky picky bladder with Lidocaine all in an effort to help Lucy cope with the discomfort.  She continues to require around the clock pain medicine despite her lipase trending down. 

"Mama I don't feel good" she said to me in her very quiet voice just before falling asleep.

When Lucy has been awake she is only comfortable for brief periods of time.  For the most part the above picture is what our girl has been doing for the past three weeks. Sleepy, sleepy Lucy her body fights so hard just to keep on living, she doesn't know how to live any other way. 

Send us some holiday cheer...

Back by popular demand, e-cards.  Help us overload the system here at the hospital, and send Lucy some holiday cheer (our friends in patient relations gave us permission to do so :).

Just click this link and follow the directions on the hospital web site.

Lucy's room number is 3708.

On the fence...

Not much change in our girl clinically today.  Her lipase did come down quite bit from the +15,000 it was yesterday, but it's still in the thousands which is very high.  Our pain management plan seems to be working; however, Lucy was much more irritable today.  Tonight was rough as well, Drew and the kids came to see us but Lucy was grumpy and very tired.

After some discussion during rounds this morning, there is a concern that Lucy has an infection that could be causing this.  Our main doc and I both suspect that Lucy was septic yesterday, which is why he choose to make an abrupt change to her antibiotic coverage.  It's funny but neither one of us wanted to discuss this thought with each other yesterday.   Her white cell count came down a bit, but is still very high for Lucy.

In an effort to assist Lucy's body with all of the systemic issues she has going on at the moment, we tripled her normal fluid rate.  Needless to say she is looking a little puffy today.  Dr. R is very concerned about Lucy having a systemic inflammatory response to all that she has going on, so he is monitoring her very closely for any changes that could indicate that she is heading in the wrong direction.

Lucy's urine is a cause for concern, she is growing yeast.  This is a significant finding, but we believe that it's secondary to everything else that she has going on.  Despite it being a secondary issue, of primary concern this afternoon was changing Lucy's supra-pubic catheter.  Poor baby, I can think of nothing worse than waking from a deep sleep to somebody pulling something out of a hole in your body…geez, it's no wonder she's so irritable.  Infectious disease has been consulted on this issue as Lucy has been on an IV anti fugal for the better part of the past year.

In conclusion, Lucy is on the fence at the moment.  She could go either way, it's up to Lucy's body to let us know what it's going to do.  Do you think it cares that Christmas is a week away?

Our home away from home...

We have been admitted to "our home away from home" yet again, and so close to the holidays.

What is it with Lucy and being in the hospital for the holidays?

In case you've lost count, there are only 8 days remaining before Christmas, 8 days!

Here's hoping for a miraculous recovery for Lucy, and also that UPS pulls through for us.

Lucy has a pretty severe case of pancreatits as well as an infection somewhere.  Where, we don't know that just yet.  There is some speculation that it could be in her urine, her urinalysis looks suspicious.   There is also a fear that she has bacterial pancreatitis, meaning that it's an infection in her pancreas that is reeking havoc on our girl.  It could be both... or something else...you never know with Lucy which is why we are here.

Lucy's white blood cell count is very elevated for her, this has us all scratching our heads because she has not elicited a fever from all of this.  This is not the first time we have seen her do this, she had sepsis in September without a fever.  We made another change to her antibiotic coverage, ramping up our gram negative coverage.

(Poor baby would not let go of the bucket, she's even sleeping with it tonight)

Pain control was priority number one when we arrived to the hospital at around 4:45am!  Lucy was in so much pain it literally made her sick.  Vomiting is not something Lucy does often, nor is it something we want her to be doing.  She doesn't have the muscle strength to bring it all up or move her own body to get it out (sorry to those of you who are weak in the stomach), we worry that the infected bile that she is vomiting is flooding her lungs which can potentially cause an aspiration pneumonia.  Poor baby struggled until we got on top of her pain.

(Yikes, a heart rate of 181 bpm, and an elevated blood pressure to boot)

The cocktail of pain meds that we are giving her appears to be helping.  Pain team is so familiar with our girl, dare I say it was easy coming up with a multi-phase plan for pain management should Lucy's needs escalate over night.  There was even a time this afternoon while infusing her plethora of her pain that we all were able to enjoy a silly, very chatty Lucy.  Hey, we'll take the good times whenever we can get them.    

We are so grateful for the amazing team of doctors and nurses who know and love our girl! They help to make our life livable, and that's saying a lot!

I had all of twenty minutes of sleep last night…at least it was something, right!?!

Wrong!

Lucy's meds are all ordered and timed correctly and we have a fabulous nurse working with us tonight.

Translation, I am planning on sleeping ALL night.

Thanks for your thoughts and prayers, love and support.

ED...

Lucy woke in the middle of the night with increased abdominal pain that was way above and beyond her normal belly pain.  She's shaking, retching, vomiting…and just plain miserable.

Not sure what's going on but everyone is working hard at trying to figure things out.

Packing Lucy up quickly…it should be an Olympic event.  I think I have what we need to keep her on med schedule until orders can be entered into the system and pharmacy can begin compounding everything.

This life, our life, it's not for the weary or faint of heart.  If you could please say a prayer for comfort and clarity for our little one we would very much appreciate it.

Secured...

We are home, safe and sound.  By the time we left the hospital this evening the snow had stopped and the roads were better.  The big kids are nestled all snug in their beds, while Lucy and I are snuggled together in my bed - infusing meds, what else is new.

Today's procedure was not quite as simple as we were hoping it would be, but then again is it ever really simple!?!

Our surgeon and IR doc came out to tell me the good, the bad, and the ugly.  Basically, the stoma tract has stretched out considerably, and as such it's becoming increasingly difficult place and keep a tube in it.  Our goal is to keep the tract open, but not necessarily draining because as long as Luc has a stent in her bile duct there is an outlet for bile to drain.  The benefit to keeping this tract is so that we have a back up for if and when there is ever a problem with her stent.  This tube serves as a window or a porthole into the area of her body that is a huge cause of concern.  It's very simple for us to inject a little contrast through her tube and see just how things are flowing without having to subject Lucy to repeated MRIs.  

While  attempting to suture the tube securely to Lucy the tip of the suture needle broke off causing our surgeon to have to make a small incision in our girl's abdomen to retrieve the broken piece of the needle…seriously.  To add insult to injury our surgeon accidentally dislodged the tube while all of this was happening and another tube had to be placed.  In his defense the tube does not stay in place without it being sutured.  Does anyone else see the irony in this?  As unbelievable as all of this sounds, it was humorous to hear them both tell me what had happened.

Unfortunately, Miss Lucy has had to deal with the brunt of all of this.  She was experiencing quite a bit more pain tonight as a result of all that she had to endure today.  Pain meds are a good thing.

When all was said and done another Malecot tube was finally sutured securely in place.  Here's hoping that it stays in place well into the new year!

I am declaring tomorrow a snow day for Lucy and I!   We are going to cozy up together and maybe make some gingerbread men... her favorite kind of men.

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

Rinse, Lather, Repeat…Ugh!

It's snowing, and as such our kids have their first official snow day of the school year.  They had a two hour delay yesterday, but that doesn't count according to Jack.

Snow is not wheelchair accessible…just saying.

When I heard the weather report last night I didn't want to believe it.  They predicted Sunday's storm incorrectly…less than an inch turned out to be almost a foot of the white stuff.

This morning I had a bit of a panic attack when I looked out the window after receiving a call from the school district at 5:30am alerting us that school was canceled.   You see, Lucy is scheduled today to have surgery to replace her cholecystostomy tube…again.  Yes, you read that correctly!  Rinse, lather, repeat…ugh!  Less than twenty-four hours from the placement of her tube on Friday night this tube came out because it was not sutured in place.

Not only did I have to pack and prep Lucy to get to the hospital by 11am, I had to find a place for the kids to hang for the day.  Never-a-dull-moment in our life e-v-e-r!

So very very thankful for amazing friends.  The kids packed up all of their snow clothes, sleds, and a few snacks and were on their way to frolic and play, while I prepped and and primed IV bags and meds  to take our girl to the hospital for the day.

After discussing the ins and outs (pun totally intended) of how we are going to keep this tube inside of Lucy, we kissed our girl good night at 12:45pm and left her in the the very capable hands of our IR team and surgeon.  The plan for Lucy is to suture the heck out of this tube…really!

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

Update...

Thank you for your prayers, they were able to get a tube back in…whew!!!  Our urologist was also able to take advantage of Lucy being under general anesthesia and did some work on her bladder, Luc has been having some issues with sediment and blood clots clogging her catheter.  We also took the opportunity to change her GJ tube while she was under, we considered it an early Christmas present.

Say a little pray please...

This time last week we were here…

Today we are sitting in the waiting room, waiting for our little one to come out of surgery…again.  Lucy's gallbladder tube came out sometime during the course of last night, not sure exactly when.  By the time I realized this her stoma, or the hole that the tube comes out of, was closed shut.  I could not insert anything into the hole to try and open it up.  This discovery set several balls in motion today which ultimately ended with a team of doctors and nurses working together to try and reinsert a new tube into Lucy's gallbladder site. There is a very real fear that we may not be able to get a tube back in…if this is the case we will need to weigh the risks vs the benefits of putting Lucy through another gallbladder tube surgery.  

If you could say a little prayer please that our wonderful team of docs will be able to reinsert a wire and dilate Lucy's stoma enough to get a tube back in, we would be ever so grateful.

So very thankful for another year….

Last year at this time…

our girl was in the fight of her life.

She was so very very sick!

Our life has never been the same since she was born, even more since this time last year. 

Despite it ALL, we are trying our best to live life.

This time this year…

we are in Disney World, celebrating life...

and making memories!!!

 Hola Handy Manny, meet Lucy.  Bet you didn't know this, but you just so happen to be the LOVE OF HER LIFE (at the moment).  We thought it might be nice for her to meet you in person :).

We are all about seizing the moment, and boy have we had some moments this week.  I will write later about the story behind this story, how we decided to forgo any sleep and stress over how on earth we were going to do ALL of this in "the happiest place on earth".  But the ending goes something like this… life, it's all about the memories.

(To all of those who know and love us and had no clue that we were planning this, we didn't really know either until last week :).

Ever feel like this...

Happy Halloween from a yo-yo, an angel, Little Red Riding Hood, and a little mermaid not to be confused with The Little Mermaid (pictures soon to follow, just as soon as I actually take some :)

Pumpkin courtesy of Drew, the master carver and creator of all things jack-o-lantern in our house.

Still here...

The past few weeks have been a blur...

Today we're spending the day at the hospital filling Lucy's tank with some blood product.  Blood transfusions take a l-o-n-g time! Once the blood actually hangs I know we have about four hours to go.  Before that it can take hours to get her type and screen back and have the blood bank process the blood.  I'm not complaining, just sharing a bit about the process.  I'll gladly sit through hours and hours of transfusions if that's what it takes to keep our girl going.  Thank you to all who donate blood, we appreciate you!

I have so many things I want to document here.  I am just going to have to sit down sometime in the very near future and write a random "here's where we are in our life at the current time post",  but today while I watch blood drip every so slowly into my little one's body I am working on a post because I want to share something with you about something special coming up in our life.

Stay tuned...

In the mean time thanks for checking in on us and for your thoughts, prayers, love and support!

It's In...

Our surgeon was running behind today, 

Lucy waited patiently for him,

and then fell asleep.

Lucy is the proud owner (whether she likes it our not) of a brand spanking new 7fr double lumen central venous catheter placed in her left subclavain vein.  In other words, she has a brand new line coming out of the left upper quadrant of her chest with two points of access.  Our surgeon, whom by the way we love, love, love, reluctantly took out, in his opinion, the perfectly placed PICC line.  Perfectly placed if you don't ever want to wear pants, that's what I told him.  He was a good sport about the whole thing.  He can be so stubborn, but then again so can I.   Seriously, the jokes and sarcasm have been flying around here all day, it definitely helped to lighten the mood which is always welcome when your child is in the operating room.

Luc is a bit cranky and tired, she tends to wake up from anesthesia on the wrong side of the bed, and she did just that.  We are going to let her sleep it off for a while.   I am however packing us up, we are going home tonight!  The doc at Hopkins who placed Lucy's stent is out of the office for the next few weeks. When he returns we will have to make arrangements to go back down to Hopkins for another ERCP to clean out the debris in her biliary stent.   As for now we are going to go home and resume living life. 

Thanks for your thoughts and prayers, love and support!

Location, Location, Location...

Everyone knows the most important rule in real estate....

location, 

location,

location! 

Our main doc and I agree, we don't like the current location of Lucy's temporary line.  Our surgeon is not necessarily in agreement but is going to move the line back to her chest, aka prime real estate.  Cultures have remained negative in both her blood and her urine which means we can head to the OR. 

Lucy has an OR time slot for 11:30 tomorrow morning.

A pretty boring day..

When I woke this morning I saw an email from the good doc that read "Stopped by early everyone asleep.  Will touch base later today.  Otherwise pretty boring day."  I thanked him for letting us sleep in (sad but true, I get more sleep in the hospital than at home) and told him that we were looking forward to a pretty boring day.

Our room was dark, quiet, and dare I say peaceful despite being the loudest room on the unit.  Lucy slept pretty much all day, she has that look about her that says I'm not really here even though I'm here.  Yesterday's procedures took a lot out of her.  I was sad to see her like this today because, like I mentioned, we have had some pretty amazing days with her recently.  We savor the good days when they come and look forward to them returning when they are gone.

Blood cultures are still negative for growth as of 24 hours, which is a good thing.  Urine analysis looked pristine, also a good thing.  Lucy's labs are indicating that she is trying to fight off something, her white blood cells are elevated for her.  If I had to guess where the possible source of infection is it would be her bile duct.  Her liver function labs are not great, but that's nothing new.  We are waiting to hear back from Dr. O at Hopkins as to what he wants to do about the occlusion in Lucy's biliary stent.

Drew and the kids came for dinner tonight, it was good to see them.  Lucy woke for a little bit while they were here but then went back to sleep.  The kids showered and put on their pajamas and then went home to go to bed.   Tomorrow's another day, kind of glad I'm not the one who is going to have to wake the kids in the morning.  They are going to be tired cause they left here kind of late.  Good luck with that honey:).

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

Settling in...

Seconds feel like minutes, and minutes feel like hours just ask any parent who is waiting for their child to come out of surgery.  Whenever Lucy is under anaesthesia for any procedure I look at my watch at least a million times.   When all was said and done two hours had gone by and lots of new tubes had been placed inside our little one's body.  Her chest is line free at the moment and I couldn't resist rubbing her bare chest when I saw it.  They placed her new temporary PICC line in her right femoral artery, aka her groin area.  Not the best location when you wear a diaper or if you want to wear pants.

We had the dreaded conversation about line access with our surgeon today.  There are only so many places in the body that you can place a central line, preserving vein access is huge in our world.  Lucy will have a scan of her upper extremities sometime tomorrow or the next day to look at all of her veins so that we can strategically locate a new spot for her new central line that we are hoping to have placed on Friday.  Our surgeon was urging us, however, to consider keeping the PICC for as long as possible, but in the same sentence did say that he would keep our OR time slot on Friday.  We understand the need to preserve line access, but we also need to consider Lucy's quality of life when it comes to having to live with all of these lines and tubes in her body.  Where the PICC is placed is a terrible location to have a bunch of tubes coming out of your body.  Lucy woke briefly tonight and started pulling at her new line.  She's not happy with where it is and wanted it out.  She's still pretty out of it tonight, hopefully when she is a little more alert in the morning we can talk her into not pulling at it.  

As for her gallbladder tube, after cauterizing the granulation tissue that was growing around the stoma they were able to successfully place a new tube with relative ease.  One of the reasons we choose to keep Lucy's gallbladder tube is so that we can use it as a potential pop off drain if the stent were to become occluded.  Last week Lucy put out more drainage from that tube than ever.  When I told our surgeon this it prompted him to look further.  When injecting dye into her stoma site they discovered that her stent is partially occluded with sludge and debris.  The stent is draining, albeit not as well as it could.  Our surgeon is going to call Dr. O at Hopkins and find out if he can clear the occlusion via ERCP.  Our IR doc said that she could go in and try and clear the occlusion, but that would require having to make an incision in Lucy's liver.  We all agreed that undergoing another ERCP, as big of a hassle that it is, is the better way to go about clearing the occlusion.  Seriously, if it's not one thing it's her bile duct!

We are settling into our home away from home.  Labs and cultures were done when we arrived on the floor tonight, please pray for negative cultures.  I am choosing to look on the bright side of things, at least I will be able to get a full nights sleep for the next few nights.   

We just can't seem to stay away...

We are packing up and heading to DuPont today.  On Sunday just as we were getting ready to leave for an event at our favorite place, Longwood Gardens, we discovered that Lucy's gallbladder tube had come out and was literally hanging on by a thread.  This prompted a quick email to the good doc in which I simply said, "Now what?".  There has been some debate as to whether or not we should keep this tube for Lucy now that she has a permanent stent in her bile duct.  However everyone agrees, including us, that there is merit to this tube and as such we are going to try and keep it.

Last week when drawing labs from Lucy's blue lumen on her central line, aka her life line, we discovered that the line was ballooning whenever we flushed it.  This is no good, it is on the verge of breaking, and as you can imagine a broken line would be a disaster of epic proportions in our life.  This particular central line has been in Lucy for 14 months, that's one year and two months people!  That's a record for us! This line has also had the heck TPA'd (yes, that's a verb in our world) out of it too.  Due to the duration and wear and tear on this line Lucy's going to have this line pulled and a brand spanking new line placed, but not all at once.  Nope, that would be way too convenient.

To complicate matters, Little Miss has been dealing with intermittent fevers for the past week or so which has us all wondering if the almost broken line is harboring bacteria.  Our surgeon believes because the line has been in her for so long that it likely has bacteria on the outside of it that has colonized and taken up residence there.  When we go to pull it there is a fear that Lucy will be showered with bacteria.  Not good, so we are taking measures to ensure that Lucy stays infection free.  Her old line will be pulled today and a temporary PICC line will be placed.  On Friday we are planning on heading to the OR, if blood cultures are and stay negative, to have a new double lumen Broviac placed in Lucy's chest.

Since the placement of her biliary stent, Lucy has been feeling better, praise God!  She has her ups and downs all-of-the-time, but the past few days have been pretty great days for our girl.  Here's hoping that we keep her well and that things go according to plan this week.

Lucy is currently under anaesthesia having ALL of her plumbing replaced: central line, gallbladder tube, gj-tube, and suprapubic catheter - that is if our urologist has time, if not that will be replace on Friday.

As always, thanks for your thoughts and prayers, love and support.

Energy for Life Walkathon 2013

A huge thank you to all of you who came and walked with us today.  We could never do this alone, and we thank God that we don't have to.
We walked not only for Lucy, we walked for our friend Wyatt (July 23, 2002-September 27, 2013), and for all of the families effected by this devastating disease.

Make a video - it's fun, easy and free!
www.onetruemedia.com

Oh me, oh my...tomorrow is the day!

Please join us, tomorrow,

 Saturday September 28th

For the 4th Annual UMDF Energy for Life Walkathon 

to be held at Campbell's Field in Camden, NJ.  

Registration begins at 7:30am with the walk to start at 9:00am.  

(Believe me we know that's early, but we are going to move mountains to be there and you're gonna to want to see that:)

  Help us reach our goal of raising funds and awareness for mitochondrial disease today and join our team 

"For the Love of Lucy"

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

Jack-Black-Widescreen from UMDF on Vimeo.

A very special thank you to all of you who faithfully support our family and our efforts to raise awareness and funds for mitochondrial disease, a disease that affects our family and the families of so many, we could never do this alone!

Getting better...

Yesterday was the first day in weeks that Lucy was awake and alert for a good period of time.  She was more "with it" than "out of it".  We are all so very thankful for that!  It looks like we have turned that infamous corner once again!

This toothless smile picture was taken this morning.  And yes, that's an IV in her doggie Petey.

Infectious disease has some of the specifics back on some of the bugs taking up residence in Lucy's biliary system.  We all have bacteria in our digestive systems, it's when the balance of good and bad bacteria get out of control that we run into problems, for Luc there is not such thing as balance.  We have seen all these bugs grow before in various parts of Lucy's body - pseudomonoas, enterococcus faecalis, another enterococcus species yet to be determined, and one more bad bug stenotrophomonas maltophilia.   Oh yeah and yeast, lots of it.  The specifics on the types of yeast will take longer to get back.  These bad bugs appear to be the trouble makers living inside of Lucy, all of these bugs have been infection causing at one time or another.  Psudonomas is the bug we believe that caused her to have colangitits this time, it's resistant to the antibiotic that she was on when she got sick.

Over the weekend discussions were had with the infectious disease team about their recommendations.  Now that it's Monday morning our main doc and I need to discuss just how exactly we want to go about implementing those recommendations.  We both agree that we need to give Lucy time to get better before we go and make any changes.

All that being said, we can monitor Lucy's labs, manage her pain, and take care of all of her medical needs and then some from the comforts of our own home.  Our main doc agrees and is giving us the boot.  We are going to take this show on the road once again, because "there is no place like home"!

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

Can I give you a little advice...

We have just a little bit of experience with hospital life :), in case you didn't already know.

We are living the hospital life again,  and I thought now might be as good a time as any to let you in on some of the details of just how exactly we manage to survive while here.

Caffeine, lots and lots of it, coupled with some Excedrin Migraine.  I'm only sort of kidding about the lots and lots part, but the Excedrin Migraine is no joke.

Some of you might find this to be informative, for others maybe not.  For me, I know that one day I am going to look back at this time in our life and wonder just how exactly we did it.  I blog mostly for me (just so you know), but if by chance I manage to educate, amuse, or inspire you with what I write...well, then, that's even better.

To survive in any environment, especially the hospital, for me a healthy mindset is a must!  Whenever we walk through these doors I know that we are in need of the experience and expertise of those who frequent the halls here.  I have a tremendous respect for the care, concern, love and support that we receive from our medical team. Without them we would not be able to live the life we do live!  I also know that when we walk through these doors we are at the mercy of their schedules, procedures, and protocols... this can be the most frustrating thing of all.  This means that I have to surrender control, perhaps the most difficult of all human traits to relinquish; control of our time, control of how and when I prefer things to be done, and some but not all control over Lucy's medical care.  I could never give up all control of her care, nor do I think the staff here would every want me to give up all control of Lucy's medical care.  Have you met our girl...she's complex!  I have to keep in mind why we are here and what our needs are, it helps to channel the chaos that surrounds.   You'll wear yourself out if you don't have a healthy perspective and a grateful attitude, especially when in the hospital.

Plan for the worst and hope for the best. I realize that may sound awful, but that healthy mindset I just described, this motto helps me to keep that in check.  If I'm being completely honest here, I have set myself up for disappointment and heartache so many times by ignoring the worst and only planning on the best.  When you have a child with a progressive degenerative disease, lets face it, difficult situations are inevitable, be prepared but NEVER loose hope.

We've had our fair share of bad news and unexpected complications.  I've learned along the way that if you react to every-single-little-thing it will suck the energy and emotion right out of you. Keep things in perspective!  This life, our life, it's a medical marathon, you need to pace yourself!

The love and support of those around you...invaluable!  It takes a village and we are so very blessed for all of the people in ours!  Trust in others, be grateful for their love, support, and efforts. Be gracious and thank God that you never have to do "this" alone.

Speaking of alone, having a special needs child, one who is also medically fragile, it's extremely isolating, lonely.  It's difficult to find others who can relate to what you are experiencing, but they are out there, especially in the hospital.  We have met some of the most amazing families while in the hospital.  As humans we are born to be relational.  My advice, seek others and develop friendships with those who can relate to what you are living.  But beware, don't compare, no two situations are identical.  Respect the differences, and the challenges.  There is a bond, a kinship among special needs families like no other.   They just get it.

Educate yourself, it's a great big world this medical world.  Knowledge is power!  I believe that the worst part of this medical journey, of any journey really, is fear of the unknown.  Once you know you can begin to gain an understanding, then formulate a plan to move on from there.   The more you know about your child's medical issues the better you will be able to take care of and advocate for your child.  Medical jargon can sound scary, keep that in mind.  Reading a medical report reminds me of reading one of Sophie's Fancy Nancy books - medical jargon is just a fancy way of describing medical issues.  Doctors understand medical jargon, it helps to speak-a-their language.   Our main doc is like a walking Encyclopedia Britannica when it comes to describing medical anything.   He likes to challenge my medical knowledge often, I think he's afraid that I might know more than him one day:).

One overnight stay in the hospital might cost more than a night at the Ritz Carlton, but don't count on the amenities being better.  I always have a bag packed with the essentials to survive a night or two.  For me that means the basic bathroom supplies, a change of clothes or two, pajamas and a sports bra for sleeping.  Ladies, do us all a favor and pack a sports bra, please!  I like to pack an insulated cup for hot beverages which happen to be free for families staying at our hospital, the larger the better because it's difficult to leave your sick child for refills.  I also pack a bath towel from home, if I remember.  One of our towels is the equivalent of four sheets of hospital sand paper hospital towels.  Most importantly for me to pack, aside from Lucy's meds and supplies, is a means of connecting to the outside world - phone, computer, iPad, and all of the corresponding chargers.  Food is always good thing.  I usually have a protein snack of some variety in my diaper bag (power bars, almonds, chocolate-that has some protein, right?) except when I eat it and forget to restock my bag.  For longer stays, I bring a reading lamp because there are only two options for lighting in our hospital, bright and really bright.  I also bring a pillow from home, it helps to cut back on the amount of Excedrin I use.  We're in the hospital so often that I have a special "hospital only" pillow that gets disinfected every time we come home.  At our hospital there is access to free laundry which means I don't need to pack so many clothes, but the detergent is um...very industrial, so I bring my own.  If we are going to be here for a while I turn my bathroom into a walk in closet of sorts and hang my clothes from hangers on the shower rod.  As a luxury I  bring our own hand soap, hospital soap is so drying and doesn't smell good enough for Lucy.   A little aroma therapy in the form of hand soap, it's healing.  Just because it's the hospital doesn't mean they have the same medical supplies that we use at home.  I bring with us the medical items that we consider difficult to live without.

Sleep is a precious commodity in our world!  My best advice, take it when you can get it, but truthfully I don't always follow my own advice.  I am a terrible napper, I just can't seem to shut my brain off in the middle of the day.  Very often I'll stay up late and watch a movie on Netflix, or talk to my husband on the phone until the wee hours of the morn.  Sometimes we do both together :).  The Internet is not as busy at night, and as such there are fewer streaming delays.  Oh and Netflix, how did we ever live without? Drew and I  don't see each other very much when we are in the hospital, when we do we're never alone.  We miss each other, so this is our time to connect, a kind of long distance "pillow talk".  I crave peace and quite in my life, who doesn't, but even more so when we're in the hospital.  There is rarely a time when you're living here that you are ever alone.  If you shower before 6am you have a pretty good chance of not being interrupted, but even then I have had to answer questions while washing my hair....that's just our life.  My mind needs time to escape and to process, for me I find the best time for this to happen is when I should be sleeping.

While we're on the subject of sleep, getting a child to sleep and stay asleep is tricky business.  Now try it in the hospital,  go ahead, I dare you :).  Something we started doing for Lucy when she was a wee little baby was to play music when she sleeps (actually, we did this for all of our kiddos).  We play the same soothing lullaby music for Lucy all-day-long no matter where we are, it provides her a sense of familiarity, comfort, and relaxation.  As an added bonus it provides anyone who enters Lucy's room a feeling of comfort and relaxation and that's saying a lot in a hospital environment.

Believe it or not, our weekdays fly by while we are here.  Weekends, not so much. Hospital time is different - slower yet faster, longer but shorter.   I appreciate that someone writes the date on the whiteboard in Lucy's room everyday, you can seriously loose track of time while living inside these walls.  There is constant activity and visits from doctors, nurses, therapists, and friends (if we are so lucky) on any given day which means that we don't have a lot of quiet time.  My thoughts and attention are all over the place when we are here, my mind is constantly divided with worry and concern for Lucy and the rest of our little family.  When we are here we really do need to be here and am so grateful for here!  Because of that, you will rarely hear me complain about the sacrifices.  But it is hard, long distance parenting is hard, a long distance marriage even harder.  We strive to keep our kids daily life as routine as possible, because in the midst of all of this they are living their childhood and are growing up.   Having a routine and after-school-activities helps them, which makes all of the crazy scheduling that we do worth it!!!  I manage our family life from the palm of my hand, sounds impressive doesn't it, but...ahh...with my phone;).  It's ridiculous how much I rely on that thing!  We live about forty minutes from the hospital.  Despite the distance, we make an effort to have dinner as a family as often as possible when we we are here, and on Fridays it's pizza and movie night.  The kids bring their jammies and get ready for bed here so that when they get home they can just crawl under the covers and go to sleep.  This routine helps us stay connected as a family, it works for us.  We try and make every effort to include the kids in Lucy's hospital life, it's important to us and them.  Our hospital supports us in this, they are truly a family centered care facility.

So there you have it, a little insight, OK maybe more than a little, into our crazy extraordinary life.

Thanks as always for your love and support, thoughts and prayers.  We could never do this alone, and are so grateful that we don't have to!

Shhhh...

Let her sleep,

for when she wakes...

she will move mountains!

Slowly, steadily, she is getting there.

Labs look about the same today, bile cultures are growing everything and then some. We're waiting on the specifics from the lab so we can determine our course of action.  

Sleep and feel better soon my love, we're looking forward to seeing your spunky personality return. 

Sleeping Beauty...

She puts Sleeping Beauty to shame,
sleeping more than 21 hours a day at the moment.

We arrived back at DuPont last night just in time to change TPN.  Lucy thought she would make things a bit more exciting for the transport team when they arrived.  She greeted them with the sounds of her cardiac monitor alerting us all that she was throwing out PVC's and dealing with some arrhythmia's. 

Our girl, she knows how to make things exciting.    

Thankfully that was the only cardiac event she had. 

Lucy's labs look pretty much like they looked yesterday, she had pancreatitis going into this procedure and she still has pancreatitis.  We suspect that the infection that was making her sick last week is what probably caused the pancreatitis. 

Infectious disease has been consulted and we are looking into trying to figure out how to keep  Lucy infection free for as long as possible.  Cultures on just about everything have been ordered.

  

Overall, Lucy is not feeling well.  We are hopeful that with a stent back in her bile duct she will once again be able to drain bile, in turn ridding the infectious bile from that area of her body that we believe is making her sick.

Third time's a charm...

Lucy feels about as good as her hair looks today

Third time's a charm.  Dr. O was quite familiar with Lucy's anatomy and was able to place a stent successfully in Lucy's bile duct.

As an added bonus he was able to navigate his scope around her gj-tube, which means he spared Lucy from having to go through another procedure tonight to have a new tubie put back in.  I'm sure if she was awake she would thank him for that.

We are planning on transporting back to our "home away from home" just as soon as our transport team can get here.  It's rush hour, so I think we'll just sit back and relax for a while.

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