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Homeward Bound…

January 29, 2011

Lucy’s cultures have not grown any new bugs for the last 48 hours, which means that we can make arrangements to go home!  We do not need to pull and replace her line because her cultures in the last two days are negative for any of the bacteria.  We are going home on a 14 day course of IV antibiotics.  The susceptibilities came back and Dr. R was able to find ONE antibiotic that will cover both the gram negative and gram positive bacteria.  Even better, it is dosed every twenty-four hours vs. every six like we originally thought!  In our world, the number of times you have to administer a drug in a day can make or break your sleep pattern.  Lucy is already on an every eight hour dosing schedule with one of her IV meds, an every six hour med would mean sleep depravation and/or a need for night shift nursing.  Speaking of nursing, as Lucy’s needs are increasing we are having a difficult time finding qualified nurses who have IV certification, and being fabulous with children is of course a must.  We will continue to search.  I wish I could recruit a few of the nurses here on the floor, wouldn’t that be wonderful.

I spoke with our infusion company and Lucy’s TPN, IV meds, and equipment are being delivered here to the hospital sometime late this afternoon or early evening.  Our infusion nurse will come here and show me what needs to be done to take care of her at home, and then we will be able to sign the discharge papers and take this show on the road.

We are so grateful for the team of doctors and nurses that take such great care of our girly.  We are especially grateful for our doc who believes and trusts that we are capable of taking care of our girl in the best place for her…our home.

Thanks for all your thoughts and prayers, we are once again blessed to be able to take our girly home.

More bugs…

January 28, 2011

We learned today that Lucy’s line culture from Tuesday grew gram positive rods.  Dr. R said that these bugs most likely translocated from her bowels as well, because of the type of gram positive bacteria that grew.  We are keeping her on the Vancomycin, the antibiotic we are using to treat the gram positive bacteria, until the susceptibilities come back.  Lucy has a reaction to Vanco called red man’s syndrome , we learned this back in November when she was on Vanco to treat another infection.  In order to counter act the red man’s we have to give Lucy IV Benadryl thirty minutes prior to giving her the Vanco, and then infuse the Vanco at a much slower rate.  For Lucy, this means that we are infusing it over a two hour period of time. 

We started Lucy back on her TPN last night; however, her TPN is not compatible with the IV antibiotics we are using.  We have to turn off her TPN and switch her over to D10 when she is getting her antibiotics in order to maintain her glucose levels during the time we are infusing her antibiotics..  In addition, she has several other IV meds that we are infusing that are also not compatible with her TPN.  All of this switching back and forth is a bit confusing and makes for one complicated set up.  In case you didn’t already know, complicated is Lucy’s middle name.

Lucy’s urine culture came back growing an impressive number of gram negative bacteria.  This earned us a trip to down to ultrasound to have a bladder and kidney scan.  We have been very suspicious  for some time that Lucy is not emptying her bladder entirely when when she does go.  Dr. R believes that Lucy could be suffering with a neurogenic bladder, a condition where the nerves and muscles don’t function correctly in the bladder.  It is not uncommon in kids who suffer with chronic intestinal pseudo obstruction to have a neurogenic bladder, the two kind of go hand and hand.  We are going to follow up with urology in a few weeks, once the infection is cleared, and do urodynamics study to test the function of her bladder. 

After an impressive snow storm hit our area yesterday, dumping a whopping 18 plus inches of snow, my mom was finally able to fly in this afternoon to help us.  My mom, Drew, and the kids all came to the hospital tonight to have dinner and to attend our monthly family support group meeting.  It was nice to see everyone, I miss them and so does Lucy.  She asks all the time “Where’s Dada?” or “Where’s kids?”.  Normally I answer her with “Dada’s at work and the kids are in school”, except this week the kids and Dada have all been stuck at home due to the snow.  This kids are scheduled to have a two hour delay tomorrow, but should be back in school for the first time since Monday. 

There is talk of going home soon on fourteen days of  IV antibiotics, if we have negative cultures tomorrow.  Dr.R told me that he felt confident that we could handle all of her IV meds at home.  It won’t be easy, but we can do it.  Where there’s a will there’s a way…and oh do we have will!

A Day in Our Life…

Just a glimpse of our time here…




A set up like this, commands respect.P1267684





January 27, 2011

We had a relatively uneventful night, which was just fine with me after the day that we had.  Dr. R came in this morning and talked to me about the bugs growing in Lucy’s line.  What we saw happen to Lucy yesterday was gram negative sepsis, very scary!  When we started pushing antibiotics through her line it caused the bacteria in the line to spread throughout her blood system, thus causing Lucy to become septic. He referred to gram negative rods as “the bad guys”, gram negatives are bowel bacteria.   Dr. R believes that the bacteria translocated from Lucy’s bowels into her bloodstream.  This is what we have been afraid of, Lucy’s bowels are failing, despite all of our efforts to push trophic feeds and control her small bowel bacterial overgrowth (SBBO).  We are waiting for the susceptibilities to come back so that we know more precisely what type of gram negative bacteria we are dealing with.

Lucy’s urine has also come back positive with gram negative bacteria.  The good news is that the IV antibiotics that she is on should take care of both the bacteria in her line and urine.  What has us concerned is the fact that Lucy’s white cell count is only minimally elevated.  Lucy’s immune system is so compromised that it is not responding appropriately to the bacteria in her system.

We took a trip over to the sleep lab this morning to have an EEG done of Lucy’s brain.  Yesterday’s seizure activity, tonic clonic seizures, was a different type of seizure than what Lucy typically experiences, absence or petit mal seizures.  We believe that yesterdays seizures were brought on as a result of Lucy’s high fever.  Nonetheless, an EEG was merited considering the severity of what happened yesterday, her having mito, and the regressions that Lucy has experienced this year. 

Now that we know that Lucy has a line infection, we need to get rid of the bacteria from the line.  We need to have two negative cultures in a row to even consider keeping or replacing the line.  Dr. R is going to do everything he can to save the line.   He is fearful of having to replace it, he feels that if we do this with every line infection we are going to run out of access, places to place the lines.  He admitted to me that running out of access is one of his big fears for Lucy, mine too.  I also have to admit that I am fearful of keeping this line, it is subpar now that it has been repaired once, he knows this too.  Also our need for a double lumen (two ports of access) is here, as we are running IV infusions several times a day in addition to TPN twenty-four hours a day.  We will decide on Friday what the plan will be.

As for today, it is snowing and the kids are at home with Drew because of the snow.  My mom was on her way to our house this morning, but all flights have been canceled until tomorrow..ugh.  Lucy and I are keeping warm here in her at the hotel DuPont.

9:00 Update

January 26, 2011

After only eight hours, cultures have come back growing gram negative rods, which means a line infection.  It must be a pretty potent infection, if after only eight hours we have positive cultures.  We are still waiting to learn what if anything else grows, typically you need to wait another 24 to 48 hours.

Lucy is on two different IV antibiotics, one to cover gram negative bacteria and another to cover gram positive bacteria.  In addition she is running several other IV meds. It is going to be a busy night of meds and untangling of her lines. 

She continues to complain of belly pain.  We are draining both her g and her j to help alleviate some of her pain.  Her fevers have come way down, nothing like we saw this afternoon. 

Drew was with Lucy and I all day, but is back home with the other kiddos tonight, as I work the night shift here.  Our friends the Lanahans took the three other kids today as the kids had a day off from school for a teacher in-service.  We are so blessed to have such loving and amazing friends. 

As always, we are so thankful for all the thoughts and prayers said for our family and children!   

3:00 Update


It has been a difficult day… to say the least.  We arrived in the ER at around 9:00 with the knowledge that we were getting admitted.  I had a feeling that Lucy was getting a fever when I put her to bed last night, higher than normal heart rates clued me in, so at midnight I stated the process of doing laundry and packing.  We woke this morning at 5:30 to run an IV infusion, and have been running ever since. 


Lucy had a very high fever 104.9, this fever is causing her to have tonic colonic seizures that are scarring the crap out of all of us!!  We are very suspicious that Lucy has a Line infection, Dr R even went so far as to say he was 95% sure of it.


Her GI system is completely shut down, what very little function she has, which means no feeds or PO meds.  We have converted as many of her meds IV as possible,  we have already done this at home.  When going over her med list with the nurses I was asked “so she takes twenty-five different meds?”,  my answer, “umm…yes, but not all at once or all the time.”

Right now our goal is to monitor the number and duration of her seizures, try to keep her fevers down as best as possible with IV Toridol and rectal Tylenol, and keep her as comfortable as we possible can. 

(I took these pics last Thursday.  Isn't’ she beautiful!)

Good Morning…or Not

January 25, 2011

We were woken up early this morning to high heart rate alarms and a girly with a 102.2 fever.  We are getting things organized and heading to the DuPont ER to be admitted.  We’ll update when we know more.

TGIF…or Not

January 22, 2011

We have yet to go a week since April of 2009 without having a medical appointment, or two, or more, or an urgent need of some sort in which we had to go to the hospital.  We almost made it a week this week, when suddenly things changed.  Lucy’s central line broke when an accidental trip on the line caused the line to snap!


Things became very urgent this afternoon when this happened! Within seconds the line was clamped, medical supplies were gathered, Sophie ran and buckled herself in the van without socks and shoes on because she did not want to waste any time looking for them bless her heart, and plans were made to get to the DuPont ER STAT.  Our biggest fear, aside from her bleeding which was under control once the line was clamped, is Lucy’s hypoglycemia.  Lucy cannot be off her TPN for longer than a few minutes before her glucose levels begin to plummet.  When we left our house her glucose level was 105, when we arrived in the ER twenty five minutes later her level was 50.  Dr. R notified the ER that we were coming and once we arrived they were ready and waiting for us.


Once in the ER a peripheral IV (PIV) was necessary, because obviously we had no central point of access anymore.  Finding a point of access was a struggle, as usual, and even our backup go to spots are no longer options for us.  Eventually, the IV team was able to find a spot deep in the bend of her elbow, and although it was a rather uncomfortable spot for Lucy it allowed us to give her D10, the highest dextrose solution that is able to be given in a PIV.  Lucy was given a 60cc bolus of D10 straight into her IV, and was then started on a continuous drip at a little more than twice the maintenance rate because our girly is used to a much higher concentration of dextrose than you can give in a PIV.  Once things with her glucose stabilized, we could get on with the next big problem, repairing her line.  Because of where the line broke they were able to repair her line in the ER with a repair kit,  another prayer answered.  I kept thinking on the drive over if we needed an OR time slot our chances of getting one on a Friday afternoon were slim to none… and our chances of getting one over the weekend were even less. I chose to stop thinking beyond that point.  Of course nothing can be that easy for our girly, the size of the the line that Lucy has did not fit with the size of the repair kit.  For all you knowledgeable central line people out there reading this, Lucy has a 5 french(fr) single lumen Broviac, we had to use a 4 fr repair kit to fix her line because the 5 fr was just too big to connect to her line.   We glued the 4 fr repair line on and were told that we had to wait four hours for the glue to dry before we tested the line to see it worked. 


(I took this pic tonight so that I had a visual reference to look back on for future reference as my memory is not what it used to be, it also happens to be a very befitting pic  to post here:)

Dr. R met with us in the ER and  jokingly asked, “So who did it?”.  We also talked a little about a conversation that I had with Dr. Shoffner’s nurse earlier in the week.  Dr. S is looking into trialing a medication not typically used for motility but for hypotonia, Neostigmine.  Dr. R is going to give Dr. S a call next week to learn more about this, but it doesn’t sound like Dr. R is a fan of this med for our Lucy for several reasons.  Instead our conversations have been more focused on the need for some sort of bowel surgery for Lucy.  What surgery is the question and would Lucy be able to recover from a surgery of this magnitude.  We have a lot to think about, but for tonight we were just focused on getting her line repaired and going home.


While literally waiting for glue to dry, we were visited by some of our favorites in the hospital, and even went upstairs to meet another fellow mito family that I “met” online on a mito forum.  I just so happened to read their blog last night and commented to Drew about so many of the similarities between their child’s issues and Lucy’s that I felt compelled to get in contact with this mom.  Who would have guessed that this opportunity was going to happen tonight.  There is something so comforting when meeting others who can really relate to what you are experiencing.  The mito community is really and truly amazing , and we are so grateful to be in the company of so many amazing families!

After another very long day, prayers were once again answered and Lucy’s line repair appears to have worked.  We signed the discharge papers at 9:30 pm and headed home to gather the rest of our family from the Puff’s, who once again took care of our other three children like they are their own.  What would we do without the Puff’s… I don’t want to begin to imagine.


January 20, 2011


Believe it our not we are still here… just have not been here, on this blog all that much, for all sorts of reasons….and I miss it.  Writing on here is very much a sort of therapy for me, it helps me to organize my thoughts, clarify some of the everyday and not so everyday experiences of our crazy and very fulfilling life,  and share and document our journey…our lives…for us and you too. 

I have been struggling with how much I want to share with all of you about what we have been feeling and experiencing.  In all honestly, I don’t know that I have the ability to put into words some of what we have been feeling… because at times we don’t even know what we are feeling.  We are learning, processing,  really trying to feel it, embrace it, and find words for how to describe it.  We are trying to live in the moment…trying to live in the here and now!  This is much harder to do than it sounds.  It requires us to be in touch with our feelings and embrace them.  As emotional as I am, it can be difficult to really feel what I am feeling and appear like I am sane, or better yet, not insane.

If you have followed me this far, I will try and give you an example of what I have been trying to explain.  If you are a long time reader or even just a recent reader, you know that Lucy’s health has taken a dramatic turn for the worse in the last few months.  Pardon me for using a a really bad metaphor, but we sort of walked off a cliff in October(thinking back, we started walking towards it last spring) and haven’t stopped falling since.  Although, at the moment we don’t feel like we are descending as rapidly.  The months that have followed have been filled with one medical crisis after another.  We have had some very serious conversations with Lucy’s doctors about “quality of life”, and because I am writing to you about feeling what we are feeling in the moment, let me just tell you that it just plain sucks that we had to have those types of conversations, especially so close to Christmas.  There is something so magical about the holiday season…it has the ability to bring out the the best, and more emotional side in us.  The holiday season does something for our memories, the time of year acts like a sort of time capsule, you remember what you did this time last year, two years ago, even a decade ago.  Maybe it’s the tradition, or the spirit of the season… but somehow this year was wonderful and extremely difficult all at the same time.  You see, although nobody is even saying it to us in these words, we are keenly aware that Lucy may not be here to celebrate Christmas with us next year and that makes us want to celebrate the here and now more than ever!!  Mito is an incurable, progressive, degenerative, disease, I have written those words on this blog on several occasions, I understand the words, it’s the feelings that accompany the process of seeing this disease progress and take over our little one’s body that is just plain difficult to understand!

Please, please don’t think that we are giving up hope or that I have lost my positive personality…I think that we are trying to balance optimism with reality, if there even is such a thing…as balance that is.  I think that sometimes when looking from the outside in, it appears that something is clearly one way, when in reality when looking from the inside out, it is clearly another.  We have never been in this position before.   You expect that you should feel a certain way about one thing when really you feel an entirely different way.  I think that what I am trying to say is that I am going to be more honest with how we are feeling…dare I say raw with some of the emotions that we are experiencing.   I have been struggling with how much I should share with you, how much I should put down in words.  Please know that these are our feelings, and although you may feel differently about something unless you are walking in our shoes and living our life you cannot know what it is like, and I hope that you never have to…I mean this in the most polite and sincere way.  As far as I know, there is no book on how to raise a child with an incurable progressive degenerative disease, I think that Dr. Sears might be missing the boat here. 

It is not easy saying what you really feel or feeling what you are really saying…it is my hope that I can share what it is really like for us to live our life.  I feel a change happening in our lives and in turn on the pages of this blog.

Happiness is…

January 10, 2011

Drew's Dream Come True

Seeing my husband so happy!

Why is my husband so happy?

Because… he was able to see his beloved Packers play the Eagles in the wildcard playoff game today…and the Packers WON!!!! 

Today would not have been possible had it not been for the kindness and thoughtfulness of friends, and our wonderful nurse Helen. Thank you!

For a short time Drew and I were able to forget about all of the “stuff” that life has thrown at us, and root for our “home” team, the Packers.

I was so happy to see my husband so happy, it’s the little things!

And Happy New Year…

January 3, 2011

Good-bye 2010…


hello 2011!PC317552

Here’s to a new year, may it be filled with hope and peace!

Merry Christmas…





From our house (hopefully, yours is a little bit cleaner than ours) to yours, Merry Christmas!