Bear Mail...

Thank you so much for ALL of the wonderful cards, they have been amazing to read!  Our friends in patient relations told us that Lucy backed up the system yesterday :).   

Your thoughts, prayers, love, support, and beautiful words of encouragement mean so much!!!! It has been an emotional past few weeks and I have not had the time to let it all out so to speak...until now.  We are so blessed to have so many who care.     

  Thank you!

We along with so many are praying for our little girl!

Thank you so much for supporting us on this journey!

Clarity...

Monday afternoon Dr. R came to our room to discuss a finding that one of the GI fellows brought to his attention.  The fellow spent some time looking over Lucy's entire radiology history, and as you can probably imagine it's extensive.  She discovered that Lucy's common bile duct was significantly enlarged, which is a rare but serious concern.  Not only was it grossly enlarged, but it has grown rapidly in the past eighteen months.  Her last CT scan in April of 2011 showed that her common bile duct was enlarged to 1.5mm, where typically it should be around .25mm in diameter.  I recall having a talk with our surgeon about this finding when Lucy was in the PICU back in 2011, he thought that at some time in the future it would need to be investigated,  I suppose now is the future.

We are so grateful for the vigilance of Lucy's medical team and in particular the GI fellow who thought to pursue this avenue.  Dr. R admitted that he never thought to look at this because it is so rare.  When talking about Lucy, rare is common in our world!  It was Lucy's consistency in telling us where her belly hurt and her increased pain that lead this fellow to investigate further.

Yesterday, our day started off bright and early, ultrasound came to Lucy's room at 5:30am to do a scan of her liver, gallbladder, pancreas, and small intestine, talk about a sneak attack.  Lucy hates having an ultrasound done, the combination of the element of surprise and the fentanyl patch made having the scan tolerable.  That and the fact that she is one sick little girl who is unable to move on her own meant that she stayed relatively still.  She couldn't roll away, which is her usual M.O. for trying to get out of an ultrasound.  The ultrasound revealed that there were many areas of concern in Lucy's liver, bile ducts, and gallbladder.  More specifically, Lucy's common bile duct was grossly enlarged, her gallbladder wall thickened with sediment and sludge, and her bile ducts in her liver were found to have blockages as well.  There were two conflicting radiology reports that both concluded, however, that an MRCP was warranted to have a better look at things.

The MRCP showed that Lucy's common bile duct was indeed grossly enlarged and that she has many areas of concern in her liver and gallbladder.  We learned that her common bile duct has grown to 12.5mm in diameter!    Not only did they discover this, but they also discovered that Lucy has a stricture in her common bile duct, an area where it narrows and is nearly obstructed.  This is extremely painful!

Last night Dr. R came and showed us the pictures from Lucy's MRI, her common bile duct looks like a funnel, wide at the top narrowing just below where the the gallbladder drains into the common bile duct. There is no visible obstruction, but something is causing the narrowing. This is a very unique and interesting finding in a kiddo Lucy's age, this just isn't something that is seen very often in pediatrics. What has caused this, we are not sure.  In order to investigate the problem further Lucy is going to need to undergo a ERCP and have a stint placed in her common bile duct to help the bile duct drain into the small intestine, a procedure that is rarely performed in pediatrics, but rather more commonly in adults.  Because of this Lucy is going to need to be transferred to Jefferson Hospital in Philadelphia where an adult gastroenterologist has agreed to take Lucy's case.  He is "fascinated" with her case because it is not something that is seen everyday.  He mentioned that there are a handful of cases in which patients with severe dysmotility of the intestinal track can also develop dysmotility of the biliary system.  There is also a concern that this is a congenital abnormality, something that Lucy was born with, and overtime has developed into what it is now.  We really won't know until after the ERCP.

After the ERCP, Lucy will be transferred back to DuPont for recovery.   Our surgeon here at DuPont is going to have to decide if, when, and how he is going to perform a second surgery to remove her gallbladder as it is not healthy and full of slug and sediment that can drain into the common bile duct, thus causing the stint to become obstructed.

At the moment Lucy is one very sick little girl who is in a lot of pain!  She most likely has colangitis, an infection of the common bile duct which explains her fevers. We started treating her with antibiotics again this morning. The fentanyl patch is working, but she is also requiring PRN (per requested need) doses of dilaudid in addition to the fentanyl, more today than yesterday.  Her respiratory rate is low, so we need to be careful.  Lucy's metabolic panel is a mess.  Today she had yet another differential diagnosis added to her already long list of diagnoses, hypercalcemea.  Despite taking the calcium out of her TPN for the past two days and increasing her fluid rate to try and flush the calcium out of her body, we have been unable to lower her calcium levels in her blood. An EKG was order and endocrinology was consulted today as we have begun the workup for this newest issue.

Now comes the very complicated task of arranging all of the details that need to be considered when doing a procedure like this for a girl as sick as Lucy, with a complex medical history.  Jefferson is not a pediatric hospital and as such there are discussions being had between the doctors here at DuPont and the doctors up at Jeff, one of our biggest concerns surrounds anesthesia.  We don't know when the procedure will take place, but we are hoping for sometime next week.

At the present time we are very concerned about our girl, she is not doing well.  We are both relieved and worried to have an answer to her issues.  Relieved that there is a treatment for what she has, and worried that she has to undergo this treatment.

This is a lot to process.  We want to thank all of you for your thoughts and prayers, love and support.  We could never do this alone!

E-cards...

During our last admission Lucy received an e-card from friends we met here at DuPont who are in our Chronically Cool Families support group, a support group that was started by another amazing family that we met here at the hospital when Lucy was a teeny tiny baby.  Lucy loved getting "mail", she held on to that card all day.

http://ecard.nemours.org/welcome.act;jsessionid=dHj2sk77FBkCCpT1kZ-S7Q**

The hospital website has a link to send a patient in the hospital an e-card.  They will then print the card and deliver it to the patients room via "bear mail".  Just click here  or on the picture above and follow the directions if you would like to help brighten Lucy's day.  Lucy's room number is 3704.

Just another reason we love this hospital, they care so much about their patients and their families well being.

An Answer to our prayers...

Be careful what you pray for because you just might get an answer, and that answer may not always be what you want to hear.   Lucy is scheduled to have an MRCP, an MRI of her liver, pancreas, and gallbladder at 2:30 this afternoon.  Please continue to pray for clarity, Lucy's comfort, and that Lucy tolerates the procedure well.  Whenever Lucy and anaesthesia come together we get nervous.

Not much change...

Things with Miss Lucy have not changed much, including her pajamas.  She has barely been awake all day, really all week. When she was, she clearly needed to go back to sleep.  Sadly, she was not sporting the post transfusion glow this morning that we look forward to after she receives a blood transfusion.  She is the color of paste, with freckles and really messy hair.  The only thing red about her are her eyes.  So not the picture of health, but yet still so cute!

Dr. R came in this morning to discuss things.  We spent the first few minutes of our meeting discussing all the things that we probably shouldn't have done in the last few weeks, each of us taking turns going back and forth.  We concluded that we could not change the past and needed to discuss the here and now.  The here and now meant that we needed to transition Lucy from a continuous infusion of dilaudid to something else.  It took most of the day to come up with a plan, when all was said and done we agreed that for right now Lucy needs to be on a fentanyl patch with PRN doses of dilaudid thrown in for added pain control.  The patch will provide her with a continuous dose of fentanyl, which is safer than keeping her on a continuous infusion of dilaudid because the risk of hyperalgesia is not there with fentanyl.  She was experiencing an increase in pain today,  we transitioned her over to the patch this evening and are hoping and praying that this will be the right med for our girl.

We are continuing to search for answers as to why our girl is so sick.  Lucy had some critical lab values in her metabolic panel that needed some attention, in particular an elevated calcium.  We added a third dose of Lasix to see if could help her body flush out the excess.  There are a few theories being discussed, one requires us to have a second look at her belly via ultra sound tomorrow.  If we can not get a clear picture then we may need to consider doing an MRI.  This would require anaesthesia and with Lucy's respiratory rate being as low as it is we are very concerned about doing this.

Continued prayers for comfort and clarity are grately appreciated.

Sleeping beauty...

Sweet Lucy had a bit of a spa day today, complete with rinse-free shampoo, heated bath wipes, lotion, a blood transfusion, and an extra bolus dose of dilaudid. 

We along with so many are praying for a turn around in our girl.

Some things...

I felt more rested this morning than I have in days, which is odd since I never sleep well when we are here.  Knowing that I am not the only one in charge of Lucy's care has helped to lower my stress level a bit.  I wake every few hours throughout the night and then force myself to go back to sleep for a few more,  keenly aware that I need to wake up early.  I startle easily, because of this I like to wake before all of the hustle and bustle of the hospital begins, you know before the resident is in your face asking you before you can even see straight how the night went.  FYI I am not a morning person, I require twenty minutes, twenty full minutes, to gather my whereabouts and focus my eyes before I am fully functional in the morning.  That is unless there is an urgent situation, then adrenaline kicks me in the a** and I fly out of bed faster than I ever thought possible.

The night before last I was awaken in the middle of the night by the resident, for a split second I had no idea where I was or who she was.   Lucy's oxygen saturations were falling into the 80's and they were asking me if this was normal for her.  Normal for Lucy, there is no such thing.  Luc was in need of a little more support so we cranked up her O2 and watched as the monitor brought her numbers back up to 100 percent, but not before first repositioning her nasal cannula. It's always good to check to make sure that it is on.  It would have saved the mama, the nurse, and the resident from having a mini heart attract as we continued to crank her up O2 up to five liters with no rebound in her numbers.  Once her cannula was positioned correctly we set her O2 at 2.5 liters, watched her numbers climb back up into the nineties, and all took a deep breath ourselves.  Her breathing is more shallow than usual, increasing the O2 has helped to increase her respirations as well.

Our palliative care team stopped by to see us yesterday.  We are taking things one day at a time, but we talked about switching Lucy over to a fentanyl patch sometime in the very near future .  There is a very real concern that Lucy could develop hyperalgesia, a condition in which her body would respond with increased pain as a result of a paradoxical reaction to opiate pain meds.  Apparently this is what happen to Lucy when we tried to sedate her with Ketamine in the spring of 2011, as we increased the dose of ketamine her pain continued to worsen.  In Dr. R's words, "this is a very real reality and would be horrific if it happened".  I am processing all of this, it is not easy, but comfort is our ultimate goal for our girl and we will do what we need to do to keep her comfortable.  I was told that hyperalgesia is not a concern with fentanyl.

The plan for the weekend is to keep her stable until Monday when our main doc is back, I can't wait to pick his brain about a few things.  Cultures are still negative, and her labs are not very telling.  Clinically she looks awful, but if you asked Lucy she would say she is good, she is always good and never tired for those who ever ask her :).

 Our little fighter, she tried to smile, and then immediately feel back asleep.

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

Our week in pictures...

This is pretty much what the past week has been like for our girl....

Monday...

Tuesday...

Wednesday...

Thursday....

Today...

 I realize she is wearing the same pair of pajamas in every photo.  Pick your battles, right!   We made her good and mad tonight and changed her into clean pajamas, socks, and even changed her diaper.  Yep you read correctly, we changed her diaper for the first time in I don't know how long not because it was dirty but just because, there's the benefit of having a Foley catheter and no bowel motility.

Lucy is one irritable girl, some might say that is the understatement of the year.  Fevers are down, but her pain is not.  Cultures are still negative, her viral panel was negative too.  Lucy girl, nothing about your life has been easy, but we love you none the less.

We continue to pray for answers, and comfort for our little one.

Long Week...

I'm tired, it has been a long week of very little sleep.  Not much has changed with our girl.  Fevers are still there and she is continuing to experience pain although I believe that it would be much worse without the continuous narcotics. Labs and cultures were drawn this morning, and a nasal wash was done tonight to check for common viruses.  Lucy just plain does not feel well.

Drew and the kids spent the day cooking Thanksgiving dinner, I was so impressed!  They came to the hospital this evening and brought me dinner, it was delicious.  I officially nominated them to cook Thanksgiving dinner from here on out, with the exception of dessert, I think I had better keep that responsibility.  

We are where we need to be at the moment, I feel a sense of relief knowing that we are here.  Thank you so much for all of your thoughts and prayers, love and support.  Although this is not how I wanted to spend our Thanksgiving, we have so much to be thankful for!

ER...

Our girl is a mess!  We tried with all our might to treat this at home but Lucy's body has other ideas.  Lucy woke early this morning with a fever, in addition to low O2 sats, and pain.  We are currently in the ER.  Please please if you would pray for comfort for our little one, and for peace and understanding for our other three little ones.  I will update later.

Tough times...

I really wish I could be posting about something fun and frankly mundane, but that is just not where we are in our life at the moment.  Lucy is not doing well.  She is experiencing intense pain that we just can't, as hard as we have been trying, get under control.  Whatever illness that struck our little one, be it an infection or something viral,  has set off a firestorm inside her little body thus causing her MAS to flare and her organs to swell.  We are currently dosing pain meds every two hours around the clock, in addition to all of the other two dozen or so IV meds that we infuse on a a daily basis.  After multiple conversations with the good doc we are hoping today to be able to switch Lucy to a PCA pump of narcotics, this will allow her to receive a continuous infusion of pain meds 24 hours a day.

I have such a difficult time seeing her in pain, and at the same time it is equally as difficult for me to keep pushing so many pain meds into our four year old's body.  I have to keep telling myself that this is what she needs at the moment.  I can't help but think about the long term when it comes to pain meds. Our wonderful infusion nurse told me that I need to just focus on the here and now; and right here, right now she is in need of this.  

At the present time we are still home with her and we are trying our best to remain at home as we really really want to celebrate Thanksgiving together in our house around our table.  I realize that if they tell us that she needs to be admitted I am fully prepared to do this, it is what she needs, what she deserves.  We have an amazing care team that fully trust in our care of our girl and know whole heatedly that tomorrow is a holiday and that we very much want to be at home.  I have faith that no matter what Lucy is going to get the care that she needs, we will do our best to make her as comfortable as possible.  She is in pain most of the time, and complaining of belly pain all of the time.  With pain meds she perks up a bit, but without them she is withdrawn and silent.

We have so much to be thankful for this Thanksgiving, including the love and support, thoughts and prayers of so many.  We have been blessed to live this life, and are reminded of that on a daily basis.

Home...

We are home!  Lucy is not great, but there is really nothing that they could do for her there that we can not do for her at home, so we came home around bedtime last night.  We left with no real answers to why she is sick.  Cultures are still negative, which like I mentioned is not necessarily a good thing.  We took her off of antibiotics because we don't have any proof of an infection, yet.  If her fevers return so will we.

Lucy had a CT scan of her belly yesterday, we were looking for answers as to why she is experiencing increased abdominal pain.  Let me just brag about our girl for a moment, she did great in the scanner or giant donut as Dr. R told her it was.  He told her not to eat it, she thought that was funny.  They were worried that she would need to be sedated, but I knew she could do it without sedation and she did.  Ultrasound, now there's a test that the girl should be sedated for, ugh...not sure why but she hates it.

The results from the scan showed us that her bowels are in bad shape.  She has areas that are are narrow and areas that are dilated and fluid filled, indicating organ failure and no motility.  Lucy is in intestinal failure, that we know, the scan showed us just how severe it is.  Dr. R came in and discussed this with Drew and I, I knew it was not going to be good news when I saw the look on his face.  We discussed not being as conservative with the pain meds, she is in pain, and as we could clearly see on her scan needs meds to be comfortable.

Everyone who took care of our girl this week agreed that she is just not the Lucy they are used to seeing, and that she is just a wee bit more irritable than her norm (insert sarcasm here).  We are going to do our best to try and make her as comfortable as possible.

Oh turkey...

These pictures are of the "messy craft" that Lucy did with Jen yesterday, they put the finishing touches on the turkeys today... too, too cute.

I really have nothing new to report tonight.  We are still waiting to see if anything grows on her cultures.  It is not necessarily a good thing that nothing has grown.  We know there is something wrong, and it reeks of an infection, but we don't have any proof...yet.  Increased belly pain continues to have us searching for a cause.  Dr. R suggested "throwing her" in the CT scanner to see if can get a better picture of things,  I agreed with that, but suggested setting her down gently in it instead of throwing her :).

The kids and Drew came to the hospital to have dinner with Lucy and I and to attend our monthly family meeting tonight.  It was so good to see them, but I hated saying good-bye to them tonight especially since we don't have a plan yet.  I shouldn't have to point out that Thanksgiving is right around the corner and that the turkey is not going to cook itself, so we are hoping and praying for answers so that we get home soon.

Thank you for all of your thoughts and prayers, love and support we appreciate them so much!

Our Day...

This picture speaks for itself...I think that she feels about as good as her hair looks.

Things with our girl have stabilized, praise God.  Her temps are down as are her heart rates, but she is still just not feeling well.  I believe that the antibiotics are doing their job, her temps have come down to below normal literally overnight.  Cultures are negative, but it is still early, Lucy has been known to grow positive after 72 hours, so we wait.  I have said this before, the not knowing, the waiting, it is the most difficult part of this journey.  Based on how she presented, her lab work, and how her fevers have responded to the antibiotics, we are suspicious that this is could be a line infection.

We took a trip down to ultrasound this morning to see if we could get a better picture of what's going on inside Lucy's belly.  Dr. R wanted to be sure that we were not overlooking a possible bowel obstruction.  Thankful, again, the report came back normal, not sure what we could really do for her if she has an actual bowel obstruction.

Lucy spent the majority of her day sleeping, she did wake up for a short period of time to do a messy craft with our friend Jen from child life, it was the highlight of Miss Lucy's day by far. She wore herself out painting, and has since gone back to sleep.  Drew and I spent the majority of our day whispering back and forth to one another all the while trying to do work from our computers.  It was nice to have his company today, it is not often that he spends the entire day with us while we are in patient.  Divide and conquer is how we get through most admissions, we have had to learn to live life this way.  Tomorrow the kids and Drew will come over after school to have dinner with us and then attend our monthly Chronically Cool Families Meeting.

We continue to pray for answers, hopefully we get some more clarity tomorrow.

We have been admitted...

This pic was taken while at an appointment in cardiology, she picked these glasses from their prize box and then wanted me to take her picture so that she could see herself with them on...silly girl. 

I have been meaning to post an update this week on how things have been going, I guess Miss Lucy decided to take matters into her own hands as she has been admitted to DuPont tonight with fevers.  Times have been tough when it comes to our littlest, she has just not been herself lately.  Her bad days seem to outnumber her good ones.  We have been praying for answers, and now more than ever I find myself praying that if we find the source of her fevers, perhaps we will be able to put to rest some of our fears and concerns for some of her other issues.

Lucy and I spent most of the day at DuPont believe it our not, we had an appointment with neurology in which we discussed adding Vimpat to our list of anti-seizure meds.  It was a frustrating appointment on many levels, but we have a plan and it looks like we are going to be moving forward with it.  Talks of just how and when we were going to start the med were had, and now it appears that she will be able to get her first dose in the hospital after all.

This afternoon when we arrived home from the hospital with one minute to spare before the school bus arrived in front of our house, Lucy was crying to me that her insides hurt and that she wanted to lay down.  When I laid her in her bed and connected her to her to her pulse ox it kept alarming for high heart rates.  I sent an e-mail to the good doc that simply stated, "Can you please call me..."  When he called back he said that he was worried because I never just ask him to call me.  We discussed her current issues, and came up with a plan, sort of.  Lucy went and changed the plan when her temps started to soar.  We are always worried about sepsis when it comes to fevers and Lucy, so I quickly gathered as many things as I could think we would need for the night all the while trying to coordinate who was going to get the kids and where they were going to go.  Megan was at dance, Sophie at music, and Jack had just arrived home from science fair when all of this was going down.  It took what seemed like an act of congress to make arrangements to get everyone picked up and in one location.  Thanks to all of you who were a part of this tonight, we could never do this alone!

Our nurse Helen was just coming on shift when Lucy's temps started to soar, she and I packed Lucy up and brought her to the ED.  Drew met us here just a few minutes after we arrived.  We have been admitted to our regular home away form home where Lucy was greeted by the wonderful nursing staff,  we have started Lucy on our typical protocol for fevers.  Cultures are cooking and labs were drawn, Lucy's white cell count is up considerably for her which has me even more concerned that what we are dealing with is really stirring things up in her.  She is not feeling well, and is in pain, but has finally found rest.  We are waiting for radiology to come up to take an x-ray of her belly as she has been complaining of increased belly pain.  Afterwards I am planning on getting some sleep as I believe that we have her very complicated med schedule under control :).

I will update more tomorrow.  Thanks as always for your thoughts and prayers, love and support.  Like I mentioned we could never do this alone, nor would we want to!