Today...

Today, we took part in a promotional video for the hospital this morning.  Of all mornings, Lucy chose today to sleep in.  We had to wake her up just before 9am because the CEO of Nemours and the president of the hospital were waiting outside of Sleeping Beauty's room to come in and film/take some pictures.  It all went well, which I am so very thankful for because when it comes to Miss Lucy you never know how these things are going to turn out.  It didn't hurt that the two big wigs gave her a large pack of Post-it notes when they first introduced themselves to our girl(thanks Maggie for setting them up for success :).

Today, we weaned Lucy off of the PCA pump this morning because she only has a mild case of pancreatitis, wonders never cease!

Today, we took a trip down to interventional radiology (IR) to replace her low profile GJ button with a longer GJ tube.  The doctor at Hopkins replaced Lucy's long GJ tube with a low profile GJ button when he did her procedure thinking he was doing us a favor...no thank you, we wanted our long tube back.  Good thing we did this as her J-tube had migrated out of her jejunum and into her stomach, it was like a deja vu all over again.  Poor Lucy she was not in the mood to have to go through a prolonged tube change.

Today, Drew and I had a difficult but good talk with our palliative care social worker and our surgeon this evening.  We are so blessed to have so many wonderful people in our life who truly have Lucy's and our entire family's best interests at heart.

Today, I had a dozen or so e-mails that I should have addressed, but didn't.  I chose to watch last weeks episode of Scandel instead (thanks Jen for getting me hooked on the show:).  It took me twice as long to get through the episode because there were numerous streaming delays.  Sadly, I didn't mind the delays, I have a lot on my mind at the moment and in an effort to try and process it I need to get away from it.  Can you relate?

Tomorrow is another day...

Not too bad...

That is our girl is not doing too badly.  Her labs are indicating that she has pancreatitis but it's not as bad as it could be.  We have NEVER been on top of her pain, never, but it appears that we are doing a fine job of keeping on top of her pain for the time being.  PCA's are a good thing, when they work.  Wonders never cease when it comes to Miss Lucy, what you expect to happen doesn't and what you least expect will.  We'll see what tomorrow brings, as for today, aside from being a wee bit more irritable than her norm this morning, it's not too bad.

Lucy's PCA above...Baby's PCA below

Whatever Lucy has, Baby needs too!  Thanks to Darci for making the most realistic looking cardboard PCA pump for our Girly's baby doll this morning.  The buttons even feel squishy when you push them thanks to some kind of tacky substance placed underneath them...brilliant.  Um yes, Baby also has an NG tube, in addition to a triple lumen central line,  urine catheter, an IV.... play imitating real life, enough said!  

There are so many things swimming around in my head tonight.  Our expectations with Lucy's stent placement are very different from what is our reality.  Tonight I am not going to go into detail about the situation, I need some time to process everything.  The good doc will be back at DuPont tomorrow after saving the world for the past two weeks.  In case I haven't mentioned this before, he is an amazing doc who just so happens to want to share his gifts and talents globally.  I asked Lucy if she has missed Dr. R, her answer "yes, but not really" :).

Just wanted to let you know...

We arrived back safe and sound to our "home away from home" this afternoon.  We are tired and relieved to be back in familiar surroundings, familiarity breeds comfort, and comfort means more sleep for the mama tonight :).  Meds, TPN, and fluids are all up and running and on schedule.  Pancreatitis has set in just as we expected it would, we are staying on top of her pain with a PCA of dilaudid in addition to her fentanyl patch.  I want to say thank you for all of your thoughts and prayers, love and support it means so much to know that so many care!  

Getting ready to go...

We are hanging out waiting for our transport team to come and take us back to DuPont, they should be here sometime before noon.  Lucy is feeling a little better this morning, pain is not as intense, and no more vomiting. 

We played catch up all night with Lucy's meds.  In other words, the mama didn't get much sleep.

Unfortunately, we had to make many compromises when it came to meds and TPN, there was an error on behalf of the admitting team here which meant that TPN wasn't ordered in time for our girl.  Their pharmacy had to mix up a special bag of dextrose with additives, as well as remake many of Lucy's meds to be the correct concentrations.  Hind sight being 20/20, I think we would have been better off transporting back to DuPont in the middle of the night.

 Sleep has been sparse, and caffeine abundant.

I'm looking forward to nap time this afternoon.

We Aren't in Kansas Anymore...

I suppose if we were we would be covered in snow at the moment....but you get my point.  We are missing our "home away from home".  This adventure has been challenging on every level!!  Thank God I have the skill set that I do, and that Drew is with me to keep me clam!!  Our girl is sick, she is not feeling well.  She is in pain and has been vomiting for the past hour.  We have also been waiting for a diaper for the past hour, we thought of everything today, except for diapers.  I assumed that they would have some, you know the saying never assume anything...

We are going to hang a PCA pump for our girl tonight, I hope that this can happen for her soon.  Please pray for comfort and peace for our girl and that Hopkins can handle the level of care our girl needs and deserves.

Stent is in...

The doctor was able to place a stent in Lucy's bile duct, quickly.  The entire procedure was done in just over an hour.  He came out and told us that Lucy had a significant fibrous stricture with lots of scar tissue.   When I asked him about the debris found in her bile duct, he said very politely in his Nigerian accent, "Oh yes, you are Americans you always want to know how much :)".  The only way to keep her bile duct open was to place a stent.  Lucy is in the pediatric recovery unit, awaiting admission to the peds floor.  She was quite uncomfortable after her procedure, but was given a hefty bolus dose of fentanyl to help make her comfortable.  

Johns Hopkins...

When I woke this morning my prayer was simple, "God please give us ALL the strength to endure everything that we will have to endure today... "

Lucy did well during the transport, the transport team did well too.  They only had to infuse about a half dozen meds :O during the ride.

Luc watched her beloved Doc McStuffins, and had the transport team drawing pictures for her along the way.

We arrived at Hopkins around 11 and....

found our way to the GI suit...

where we proceeded to introduce Lucy to the nurses and doctors here.  Lucy was not really interested in meeting anyone, she preferred to talk with her daddy instead.

Our girl has been a bit anxious today, who can blame her, she wanted to sleep in a crib not a bed...

Once they found her a crib, she wouldn't let anyone in to exam her...remember my prayer this morning,  I sent up a reminder prayer just in case God forgot.

Some mamas rock their babies to sleep, I gave ours Propofol.  I left Lucy in the hands of the team of docs and nurses here at 2:25pm.  They are planning on placing a stent, if her anatomy allows.  

All of our preparations have paid off, we are so so grateful to our team of docs and nurses at DuPont, without them this day would be even more difficult!  Thanks Maggie and Meredith for the tutorial on how to use the Medfusion 3500, and for the PB&J lunch packed in bio-hazard bags :).

Thanks for your thoughts and prayers, love and support!  I will post an update later on today to let you know how things went.

Moving Forward...

Lucy is currently stable, and believe it or not a plan is falling into place.  Lucy girl, now that I have just written these words please don't go and change things on us.   We hoped and prayed that Lucy would "come back" to the point where she is currently.  She is at the point where we feel that we can consider all of our options, and weigh the risks and the benefits of each with the knowledge that Lucy is probably as good as she is going to get.

In case you don't already know, our girl has a God-awful disease!  Having mitochondrial disease is like chasing a moving target, you never know when or where it is going to strike.  It robs areas of the body of the essential energy it needs to run effectively, repeating this process over and over until eventually organ failure develops, and the life of the affected individual is compromised.  We can't stop the disease process in Lucy, nor can we slow it down...there simply is no cure for mito.   Throughout Lucy's life we have medically intervened with everything that we could to support the areas of her body that the disease has affected, all in the effort to give her "the best longest life".  This being said, our options for our girl are running out.

Lucy's entire gastrointestinal system is failing, from her biliary system all the way down to her colon.  None of it functions, none-of-it!  We have supported these failures in Lucy by providing her with IV medicines and nutrition in the from of TPN.  We understood the risks involved with TPN when we made the decision to start it for her several years ago.  We believed that by supporting her body this way we were going to be able to provide Lucy with great quality of life...we have done this for our girl, given her great quality of life! Unfortunately, with this decision came the knowledge that liver disease and life-threatening infections accompanied it.  Admittedly, we never imagined that these life-threatening infections would come in the form of reoccurring ascending cholangitis.  We assumed, as did many of Lucy's doctors, that these infections would come from trans-located bacteria and fungus from her failing intestinal tract, floating around in her blood stream, adhering to her central line, and causing life-threatening infections that way.  Stasis (lack of movement) in Lucy's biliary system, TPN cholangitis, liver disease, a diseased gallbladder, and the dysmotility in her small bowel which holds a "potpourri"(Dr. R's word, not mine) of bacteria are creating the perfect storm for the reoccurring infections in Lucy's liver and bile duct.  We cannot fix the dysmotility problems in Lucy as this is as a result of her mitochondrial disease.  What we are striving for is to be able minimize the number of infections and the pain and discomfort associated with the symptoms of cholangitis. Hoping in turn that by doing this we will be able to continue to give our girl the best quality of life, right up until the end.

We have been blessed with having the best of the best doctors come together and discuss all of our options for our special girl.  After a lot of energy, thought, concern, and prayer we are moving forward with having a repeat ERCP performed, this time with placement of a permanent metal stent.  Placing a permanent stent will hopefully prevent any further strictures or occlusions in her common bile duct.  This is what we are asking the gastroenterologist who specializes in pediatric endoscopic procedures at Johns Hopkins to do for our girl.  We are hoping that by doing this it will clean out Lucy's bile duct and assist in the removal of bacteria from that area of her body.  We also hope that by doing this we will be able to eliminate her existing infection, and reduce the number of recurrent infections she is experiencing.  After reviewing Lucy's e-n-t-i-r-e medical history (I suppose this is why it took him so long to get back to us, that and the fact that he was out of the country most of January into February), the doctor at Hopkins believes that there is merit in repeating an ERCP in our girl.  He shared with our team of docs that Lucy's case is complex(as if we didn't know this:), and although he is in favor of stent placement if things are what they appear to be, he is not sure yet if placing a permanent metal stent is the way to go with Lucy.  He wants to go in and see things for himself, ugh...the uncertainty is agonizing.

Preparations are in place and plans to be transported via ambulance to Johns Hopkins are all arranged, we are leaving here tomorrow morning at 9 am and are set to arrive at Hopkins sometime around 11 am.  Lucy has a procedure time of 2:30 pm which means we are going to be busy catching everyone up to speed on our special little girl once we arrive.  We have done all that we could possibly do from our end to prepare for this adventure.  A days worth of meds are set to go with us so that we can keep her meds on schedule, along with extra bags of fluids and dextrose with additives, hard copies of medical records, medical supplies, anaesthesia records since we have yet to speak with an anaesthesiologist,  special babies, blankets, and treasures are  packed as well as an overnight bag .  Dr. R doesn't know what he is missing...or maybe he does and planned it this way :).  He'll be back just in time to help us get Lucy through the post-ERCP-pancreatitis that is going to ensue after her procedure.  We are planning to stay at Hopkins Friday night, but are are hoping to return back to our "home away from home" sometime on Saturday, depending on how Miss Lucy is doing.

Once we return, our plan is to support Lucy through the post-ERCP-pancreatitis with pain control.  As soon as the pancreatits subsides the plan is to head to the OR here to have her gallbladder removed.  Yeah, Lucy has a mountain to climb before she can get to the other side, but it is our hope and prayer that by doing all of this our girl will have a chance at living the best longest life right up until the end.

Thanks as always for your thoughts and prayers, love and support.  I will do my best to update throughout the day tomorrow.  We could never do this alone and for that we thank you!

From Lucy's Point of View...

This is a picture of the top canopy and bottom rail of Lucy's crib.  Imagine laying on your back and looking up at these happy sights all your waking hours...Lucy is so loved!  

We are gearing up to be transported to Johns Hopkins Hospital in Baltimore on Friday.  Lucy is excited to go for another long ride in an ambulance...not sure she realizes what's in store for her once we arrive.  I am working on a more detailed post about all of the goings on in our life, I will try and post it tomorrow. 

A Gift...

For Valentines day we were given a gift...the gift of HOPE!  We finally heard back from the specialist at Johns Hopkins, he is willing to take Lucy's case.  Now begins the massive task of arranging all of the details necessary to transfer our girl down to Baltimore for a surgery date of next Friday. 

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

Still Here & Still Waiting...

Hurry up and wait...

Yep, the title pretty much sums up our life these past few weeks, we are waiting to hear back from the GI specialist at Johns Hopkins as to whether or not he is willing to take Lucy's case.   I was told that they are discussing Lucy's case today.  My stomach is in knots, as is my brain, my heart, and just about every other organ in my body today.  Drew and I had a wonderful opportunity to meet with the palliative care social worker/counselor on Monday, his role is to talk to us about all of the emotional stuff that surrounds having a chronically sick child that is "end of life"... this is no easy feat! We enjoyed meeting with him, and will continue to do so.  As we were wrapping up our time with him he asked us one last question..."What are you going to do if the doc at Hopkins says no?".  Good question, ahhh, umm, not sure...we really need him to say yes!

To add to our turmoil, our beloved Dr. R is leaving on a medical missions trip to Haiti on Monday for two weeks!  I mentioned in my previous post that we have a million obstacles in our way, him being away accounts for more than half of those obstacles.  I finally mustered enough courage to ask Dr. R what we we're going to do if Hopkins says no.  He looked at me and said honestly, "I don't know, I guess we'll keep her on antibiotics until...".  I didn't need him to finish his sentence, instead he set out to find the GI fellow and asked her to pester(one of his favorite words and activities:) the doc at Hopkins today.

Miss Lucy is holding her own, for that we are so grateful.  Dare I say she is stable, this is Dr. R's least favorite descriptive word, he likes to say that dead bodies are stable.  She sleeps a good majority of the day, but when she is awake her personality shines.  Her body is not as strong as it once was, she spends most of her days in her bed, when you do get her up and about in her wheelchair she tires very quickly.  She is in need of blood, but we are holding off on transfusing her in the hope that we get a date for an ERCP, we would like to tank her up just prior to her undergoing this procedure.

Here's hoping we hear something soon...as for now I am going to continue to hurry up and wait some more.

Determination and a Million Obstacles....

I was giving Sophie a bath last night here at the hospital when I asked her about her day.  Specifically I asked her what they talked about at church.

"Determination", she said, "but I'm not sure what I should be determined about".

The way she said it made me smile.  The kid is the definition of determined, she never gives up, she always tries to find a way.  I told her that determination means to keep on keeping on, to never give up, to keep on trying in the face of adversity, to hold on strong to what you believe...she looked at me and said, "just like you, Mom".  She made me tear up, and I responded, "yeah babe, I guess, just like me".

My tears were not from pride, my tears stemmed from the fact that I have not felt as determined recently. We have what seems like a million obstacles in our way, when I look at them all it is overwhelming.  I can't seem to separate the million from one at the moment.

We learned today that the GI specialist at Jefferson is not willing to preform another ERCP on our girl, he doesn't feel that it is going to fix things.  We are still waiting to hear back from Hopkins.  The GI specialist there has been out of the country for the past three weeks, he is expected to return tomorrow.

Just talking with Sophie reminded me to never give up, to keep on trying in the face of adversity, to hold on strong to what I believe in...even when faced with what seems like a million obstacles.

At the Moment...

I just consumed what seems like my one thousandth grilled cheese sandwich on 12 grain bread in the last three months.  Lucy and I headed out on an adventure to leave a special Valentine on the office door of our special friend Andy.  Sadly we couldn't get to Andy's office so we changed course and headed to the cafeteria instead, but not without first sending our special Valentine through the hospital mail system :).  This adventure is only the second time in three months that our girl has wanted to go for a walk in her wheelchair, it hurts her to sit for too long.  Getting Lucy in her chair is an adventure in and of itself that takes longer than you could ever imagine.  Since we made all of the effort to get out I sucked it up, sacrificed my taste buds, and headed to the cafeteria where I ordered, you guessed it, a grilled cheese sandwich on 12 grain bread with french fries so that the world's greatest french fry licker could lick the one or two, if she is really daring, french fries that she so loves.  While waiting for our order several of Lucy's pumps starting beeping and alarming alerting us that our adventure was coming to an end.  When we returned to the floor we discovered a crack in Lucy's TPN tubing that most likely happened the night before while changing out her TPN.  Great, here's hoping that no bacteria snuck in while we weren't looking and that we have enough TPN left to last us until 8pm tonight when we change TPN bags again.  We replaced the cracked tubing, reset her pumps, put her back in her crib, hooked her back up to everything: wall oxygen, pulse ox, cardiac monitor, apnea monitor, blood pressure cuff, and called it a day.

Lucy is now sleeping and I am relishing the quiet of the afternoon, well as quite as an afternoon can get in the hospital.  We are currently drowning in the sea of uncertainty, but are working towards scheduling another ERCP with placement of a permanent metal stent.  Where and when this will happen, we are not sure of that just yet, but when we know I'll let you know.  In the meantime, the biggest gun antibiotics that we have available are keeping the infections at bay... for now.  We have what seems like a million obstacles in our way, but I have faith that things will work out to be in Lucy's best interest.  Like I have mentioned, we are so blessed to have so many who know and love our girl.  Everyone wants what is best for her, it is overwhelming in a good way!

I am so thankful for dry erase board books (thanks Janice & Jules!), Crayola Color Wonder products, anything with adhesive even if it is 1001 foam alphabet stickers (thanks Deane;), the inventor of "sticky notes"(Drew actually knows of the guy...yeah I'm married to a geek:), chocolate covered almonds from Trader Joe's (thanks Gina:), fresh produce and yummy salads (thanks Heather:), adorable Baby Legs(thanks Kate :), iTunes, green tea, Excedrin Migraine, and Netflix just to name a few.  I just wish the hospital wi-fi worked better during the daytime.  As for now if we want to watch anything without hundreds of pauses for streaming delays, we have found it best to watch things after 10pm.  This just means that I have spent way too many nights staying up way too late.

I can't believe that it's February already...time flies even when you are not having fun:).  When asked how long we have been here, our answer, "almost three moths with a ten day vacation home for the holidays".  I often get asked if I work here, my answer, "no, we just live here".  I have yet to drop a hair dryer in the toilet this admission... seriously, I have done this more times than I care to admit.  Drew joking says to me, "babe whatever you do, don't plug it in!".

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

Spa day...

 I love THIS smile, it makes me smile!

 Our little mermaid....

she takes bathing to a whole new level!!