Saturday, September 28, 2013

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.


Friday, September 27, 2013

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



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!

Monday, September 23, 2013

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.

Saturday, September 21, 2013

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. 

Thursday, September 19, 2013

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.

Wednesday, September 18, 2013

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.

Second verse same as the first, a little bit louder and a little bit worse...

This morning Dr. R and I were discussing how today could not have come at a better time.  Lucy is not feeling well and is need of having this procedure done today!

Lucy has pancreatitis already, yesterdays lab work is telling.  We are going to have to monitor her labs very closely after this procedure.

We arrived at Hopkins around noon and I kissed Lucy good night around 3:00pm and left her in the wonderful capable hands of GI team at Hopkins.  With her eyes shut and an oxygen mask around her mouth, and my ear close to her she asked me to sleep in the chair next to her :).  FYI she asks me to sleep in the chair in her room e-v-e-r-y-d-a-y! 

Dr. O did not hesitate to say to us very eloquently that this is the right thing to do. 

Tuesday, September 17, 2013

Stenting her is the right thing to do...


I just ate my breakfast at 3:45pm, or maybe that's considered lunch, or maybe even and early dinner.  Needless to say my head is pounding from ignoring my hunger all day.

It has been one of those weeks already, and it's only Tuesday.  If I'm being completely honest, it's been one of those years...

It's possible that I have too much to do in a day...just saying.

After getting everybody off to school this morning I had the monumental task of packing up the things Lucy and I are going to need to survive for the next few days while we are in the hospital.  Notice how I wrote a few days, I am being optimistic here :). I think the only thing I forgot to bring was food for me.  Yep, I left the bag with my lunch and then some sitting right on the counter in our kitchen, hence the reason for my not eating until 3:45pm.  It's alright, I promised Lucy a dinner date in the cafeteria tonight, that's how much I love her :).  One grilled cheese on 12 grain bread for me and one french fry for Lucy.  She doesn't eat the fry, it's so that she can use it to lick the teeniest tiniest bit of ketchup, if she is feeling up to it.  More often than not she just likes to hold the fry and feel like she is "eating" dinner too.  It's the little things in life :).

Lucy is going to be transported tomorrow morning from our home hospital down to Johns Hopkins, again, to have another stent placed inside of her common bile duct.  She is scheduled for this to happen at around 2pm for all of you prayer warriors out there.  This time this will truly be a permanent stent, the plan is to keep this stent in for the rest of Lucy's life.

This decision was really not a difficult one for us to make.

Without a stent she is miserable, with a stent she is more comfortable.  It's really that simple.

Above all we want our girl to be comfortable!  Of course we do, all parents want their child to be comfortable, but saying we are choosing comfort in this situation means that we are choosing comfort no matter what the circumstance.  We are choosing to accept all of the consequences that accompany this decision - infection, possible rupture of her bile duct, pancreatitis, the stent becoming embedded in the wall of the bile duct, sepsis...These are all very real possibilities, but when compared to Lucy living the rest of her life in pain, this was not as difficult of a decision as you might think.

Do we wish that Lucy's circumstances were different, absolutely!

How we wish we could fix this and so many of the other problems for Lucy, sadly that is just not our reality.

Fixing Lucy's bile duct issues will not fix Lucy, her mitochondrial disease has reeked havoc on too many of her major organ systems for us to even consider putting her through a 20 plus hour surgery.  Fixing one failing organ and replacing it with another failing organ is not going to fix our girl.  This is where Drew and I and our medical team have had to come together and discuss the really tough stuff of life.  Just because we can doesn't mean we should, that has been the gist of the conversations that we have had with our team, in addition to discussing what we as her parents ultimately want for Lucy.  Our goal has always been and continues to be to give Lucy the BEST longest life.  We are hoping and praying for Lucy to have as many good days as possible, which is why we believe that stenting her is the right thing to do.

Tuesday, September 10, 2013

A long day...

We are home and exhausted, it was a very long day!  We have an amazing medical team, a truly amazing medical team!  This afternoon we had four of DuPont's finest all come together at the same time to discuss the really tough stuff of life with me, yet again.

Lucy's cholecystostomy tube was in the correct position and did not need to be replaced today.  This was both a relief and a disappointment.   A tube change would have been an easier solution to her problems.  Lucy's biliary system is very diseased!  We suspect that Lucy has cholangitis, again.  Due to all of the antibiotics that she is on she is not presenting the same way that we are used to seeing in our girl when she has had cholangitis in the past.  Lucy is not feeling well and is in pain, it is so difficult for everyone who knows and loves her to see her this way.  It is excruciating for us as her parents to see her this way.

We also suspect that because of this infection her entire body is not functioning as well as it should be.  We took a closer look at Lucy's bladder issues today via ultrasound and concluded that we need to get Lucy's current systemic infection under control because we suspect that her bladder spasms are related to this current systemic infection.  While we are working on making yet another antibiotic switch we are also going to try instilling Lucys' bladder with medication that will help control her bladder spasms. Topical medications typically used to control bladder spasms are not an option for our girl anymore as we used to use them, but we discovered that they caused severe vision loss in our girl.  Therefore, instilling her bladder, putting the medicine directly in her bladder, is our only other option.

Due to all of these recent findings we have decided to place another stent back inside of Lucy's common bile duct next week.  Only this time we are not planning on removing it, it will remain inside of Lucy forever.  Placing a stent and leaving it in comes with it's pros and cons.  The biggest pro is that Lucy will be more comfortable.  The biggest con is the risk of infection.  No one knows for sure how long a stent can stay inside of a bile duct in a child as young as Lucy before it becomes embedded and ultimately infected.  Placing another stent will give Lucy the most comfort, and that is why we are choosing this option for our girl.

Thanks as always for your love and support, thoughts and prayers.  I did not not intend to post about the Energy for Life Walkathon and these medical issues all on the same day.  Please consider joining our team and raising funds for a disease that very much needs research to help fund life saving treatments and ultimately a cure.

It never gets any easier...

I am  staring at the monitors watching my little one's heart rate, oxygen saturations, and blood pressure, the wave length is constant and slow...so the opposite of what we are used to seeing with our girl.  Something is going on with Luc.  Whatever it is it's sucking the life out of her at the moment  We're just not sure yet what it could be.  I've probably written/said these sentences a hundred or more times in Lucy's life.  Just ask our main doc, he worries every time I write/say it.

Lucy is about to undergo another cholecystostomy tube change, we suspect that her tube has migrated out of position causing her to be in even more pain.  At least we hope that is the cause for her increase in pain, if it isn't then we will need to investigate further.  In addition she is complaining of constant bladder pain.  From a culture stand point her urine is looking OK at the moment, but something is going on that we need to try and figure out.  Our urology team is going to take a look at things while she is under anesthesia today.

As you well know we are very familiar with hospital routines, but no matter how many times we have been in this position before... it never gets any easier.

Your thoughts and prayers, love and support are greatly appreciated.  I will try and update when all is said and done.

She's worth a cure...


(Picture from Last year's walk.)

I know, I know...it's been a while.  I promise a more detailed update really soon, because believe me there's a lot I have to write about.  But for today I have very important matter to talk about...

I am sooooo behind in announcing this, but better late than never...right!?!

It's that time of year again when we join together to show our support for those affected by Mitochondrial Disease...

Please Join us for the Annual UMDF Energy for Life Walkathon to be held on 
Saturday, September 28th, 2013
at the Camdem River sharks Stadium
Registration begins at 7:30am with the walk to start at 9:00am

You can sign up here, or by clicking on Lucy's team logo or the Energy for Life logo on the left sidebar of our blog.  Any of these options will link you to our team page "For the Love of Lucy".  There you can sign up to join our team, or be a virtual walker if you live far away from us.  Either way we would very much welcome your support.  Note you must register as an individual, no family registrations please (this allows us to count everyone!).

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