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Home again…

February 29, 2012

We arrived home this evening.  As always, we are trying to get settled back in to our routine.  Lucy is on an every six hour antibiotic schedule which means Drew and I will be taking turns infusing meds throughout the night.  Lucy has over two dozen meds that we give a day, each taking at least fifteen minutes to infuse.   We run a pretty tight med schedule around here to ensure that Lucy gets her meds and I keep my sanity. 

Lucy’s blood counts are falling, we are going to wait and see what her body does over the next week.  So far blood cultures are still negative.  We drew separate fungal cultures this time which can take up to 48 days to grow anything.   Lucy had a new gj tube placed today because it would not hold an extension tube in place anymore and was leaking bile everywhere that Lucy went, yuck.  When that was done and delivery of our meds were ensured, we were discharged. 

We made it through another admission.  There are always new challenges that arise with every admission, but we made it.  Thanks for all of your thoughts and prayers, love and support.  We could never do this alone!  


February 28, 2012

I woke up early, showered while Lucy was sleeping, and then heard the resident come in and wake up our girl who has had very little sleep in days...grrrr.  This is one part of hospitalizations that I despise. At around 10am Dr. R came waltzing in, he knows better than to wake Lucy up.  We were so happy to see him we could  have hugged him, he smiled and said I bet you are.  I asked him if he was up to speed on all the events that happened while he was gone.  He proceeded to give me a full report of our girl for the last two weeks, I was impressed.  We discussed our current issues and concerns and formulated a plan.  We tried to schedule a sleep study for Lucy tonight, it was a no go, but worth a try none the less.  We are scheduled for one a month from now, but it would have been oh so convenient to have it done while we are already here.  Lucy’s line cultures are still negative, but we are treating her with antibiotics for her bladder infection.  Tonight was the first time in weeks that her temp went below 100 degrees. 

Tomorrow we need to replace Lucy’s GJ tube in IR and discuss a few more issues with our main doc.  If all goes well, we should be able to go home and finish our course of antibiotics there.

Our day in a nut shell…

February 27, 2012


We spent our day waiting for bacteria to grow, or rather not grow.  Line cultures are still negative, but urine cultures are positive for bacteria, one she has grown before, but not the one she grew two weeks ago.  We think that this bacteria may have translocated from her failing intestines into her bladder.  We also believe that because we are doing ethanol lock therapy on Lucy’s central line the bacteria is not going to grow in her line cultures.  We just finished a course of antibiotics a little over a week ago to treat a bladder infection, and now we are starting another round antibiotics, a non sulfa antibiotic as we believe that Lucy has an adverse reaction to sulfa.  Anyone surprised by this?

Drew and the kids came this afternoon and stayed into the evening.  We watched several movies, not all from beginning to end, but we got the gist of most of them.   We pretended that Lucy’s crib was a boat and that anyone who was not in it was going to be eaten by sharks, this kept everyone entertained for hours.  We bought a balloon, some clay, a sketch pad, marbles, one red die, a green beaded necklace, and a binky clip from the gift shop.  This too kept everyone entertained for a while, as well as kept Lucy’s binky from falling on the floor  We had a picnic lunch and dinner on a blanket in Lucy’s room.  Lots of tears were shed, mostly by Lucy, when it was time for the kids to go home.  This is getting harder on her than it used to be.

Drew and I sat and discussed how we were going to get everybody where they needed to be tomorrow.  It is never easy, but we figured it out.  Tomorrow our main doc will be back and we are looking forward to catching up with him, or better yet catching him up on things.

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

Back in again…

February 26, 2012

Cultures are cooking, labs were drawn, urine collected, and vitals taken.  After a long day spent in the ED it was decided that they wanted to admit Lucy for observation…ugh.  She continues to run a fever, but she is not acting “sick”.  The attending that is on this weekend is concerned about Lucy, I can appreciate that, but I’m not quite sure what keeping us here over a weekend is going to accomplish.

I am going to take advantage of the wonderful nurses here at the hospital and catch up on some much needed sleep.  How’s that for looking on the bright side.  I’m so grateful for iPads, Netflix, and Dora the Explorer, we watched all 24 episodes from season one today, some more than once.   Drew and the girls came over and had pizza with us for dinner, Jack was at a party for 4th and 5th graders only so he missed out on all of our fun.  Lucy has the nurses on the floor hopping getting all of her meds and supplies, and a few extra supplies for baby doll too. 

Not sure what tomorrow will bring, but on Monday our main doc will be back. 

On the fence…

February 25, 2012

That is Lucy’s current stance, all week her body temperature has been on the rise.  It is hovering at or above our cut off for what we consider a fever.  Last night it went higher than it has all week which prompted a call to the on call doctor.  Our options at 11pm were few, she needed to have repeat labs and blood cultures done to rule out infection.  Sensing my frustration, he discussed with me my options for bringing her in, if her temps came down then we could wait until the morning, but if they continued to rise she needed to come in, and so began the back and forth discussions in my head of what to do.   Lucy has been running a low grade temperature for weeks, she has had blood cultures done weekly for the past three weeks with no growth...yet, and neurologically she has a lot of issues going on which we believe is playing a role in her temperature instability.  In addition to the fevers, she is acting manic again, not sleeping.  I would describe her behavior as volatile, one moment she is fine the next she is screaming.  This is so difficult.

Every fifteen minutes or so we kept rechecking her temperature, she was trending down which we took as a sign to bring her in in the morning.  After struggling to find sleep she finally did at around 12:45 pm.

Our plan is to bring her to the ER this morning for labs and cultures.  If her temperature is on the rise  or lab work is indicating infection then we will stay and start treating her as if she has an infection until things prove otherwise.         

Quick update…

February 18, 2012

We arrived home tonight at around 6:30pm, exhausted, emotionally drained, nervous, but relieved to be home.  We are working on the dosing for Lucy’s anti-seizure meds.  She has not had any more seizures since starting her on the Keppra and pheonobarb regimen,  for that we are very grateful.  She is, however, one miserable baby.  Keppra has a nasty side effect of causing increased irritability, this is just what Lucy doesn’t need, more irritability.   I have heard this side effect  referred to as the Keppra attitude.  She was one miserable baby before we started her on these meds.  I’m not sure yet if the Keppra is adding to her irritability, or if  it is something else, or if  her behaviors are heightened because she is exhausted and in need of getting back into her routine.  Because of all of these things, we were given the option of coming home tonight and work at sorting out these issues on an out patient basis.  Although she is clearly not better, it appears that we have stopped her seizures.  We figured if she is going to be miserable and screaming at least we can be miserable in the comforts of our own home.  

Thank you for all of your thoughts, prayers, love, and support.  I am staring at a large pile of laundry and an even larger pile of medical supplies, meds, and TPN bags that need to be put away.  I think I’ll put the med supplies away tonight and save the laundry for tomorrow.     

Our day…

February 17, 2012

Our day started off ok.  Lucy woke up at around 8 am, played with some toys, chatted to people she felt like chatting with, ignored those she felt like ignoring, watched Tinkerbelle for the billionth time, and then started seizing again.  The team was rounding when she started seizing so they were able to witness first hand the type and  intensity of Lucy’s seizures.  In a way I was relieved that they saw them, I no longer have to describe to them what they are like.  She seized for almost five minutes when they ordered a loading dose, the maximum amount, of phenobarbital for Lucy’s weight to stop the seizures.   Since then she has been sleeping, pretty soundly. 


Why is this happening, it’s anyone’s guess.  In theory Lucy did not have enough methadone for it to really have this much of an effect  on her, but we all know how much Lucy likes to follow theory.  It’s possible that this has something to do with starting her on methadone,  but not very probable.  None the less we have discontinued methadone for the time being.  Could it be a paradoxical reaction to the phenobarbital? It’s very unlikely, phenobarbital is a tried and tested medication, this is one of the reasons we agreed to trialing  it with Lucy.  The words disease progression were uttered too many times today for me to count.  I don’t doubt that that could be a likely contributor, but there are also a lot of other things going on that we need to investigate. 

Neurology was consulted today and discussed with me the need to put Lucy on another anti-seizure medication.  Phenobarbital is a sedative.  Lucy is experiencing seizures despite being on phenobarbital.  Increasing her phenobarb dosing to control her seizures long term is not the best plan for seizure control, that is if we ever want to see Lucy awake.   We are going to continue to use phenobarbital at night, but add in keppra  for more seizure control.  Keppra is another tried and tested anti-seizure medication that is not as sedating,  is used to treat varying types of seizures, and it comes in IV form.  For these reasons we are going to be adding this med to Lucy’s already long list of medications tonight. 

I was and still am hesitant to start her on anything new tonight as she has so much going on.  Dr. C who is covering for our beloved Dr. R came in this evening to discuss with me our options, she really helped me to make this decision for our girl.  She believes that the combination of the two anti-seizure medications are what Lucy needs.  We are going to be starting Lucy on a loading dose of keppra tonight, and throughout the weekend we are going to be monitoring her to ensure that the combination of these two drugs is working.  Yes, you read correctly, we are here for the weekend.  

Drew and the kids came over for dinner tonight, unfortunately Lucy was not awake.  This made the kids sad, and Lucy even sadder when she finally did wake up at 9:3o pm tonight asking,  “when the kids coming?”.   Lucy  and I called the kids and they said good night  and I love you over the phone, be still my mama heart.  We are looking forward to seeing everyone tomorrow night.

Sleeping over…

February 16, 2012

We are in a room on our regular floor admitted for observation of seizures.  Since giving Lucy her phenobarbital dose for the night we have not seen any more seizure episodes.  Prior to that we had several episodes that were observed by the admitting team.   We are very concerned that she is having a paradoxical reaction to the methadone…ugh.  She is extremely agitated and irritable one moment, and then distant and nonresponsive the next.  We were very hopeful that methadone would be a great med for Lucy since it is effective at controlling neuropathic pain.  I am looking forward to meeting directly with our palliative care doctor tomorrow and discussing things.  Not sure if I mentioned this, but our main doc is out of the country(he promises this is the last time for a long time) until February 25th which adds so much more stress to our already stressful life.   The doctor that is covering Lucy for him knows our girl well, but apparently didn’t know she was starting methadone yesterday.   I wonder how that detail was forgotten?   I am not sure I am looking forward to seeing her as much in the morning, but we do need to talk.  Hopefully we all come up with some sort of plan to get us to the 25th.

Thanks for all of your thoughts and prayers, love and support!  Drew went home to get somewhat of a decent nights sleep and also so that he can bring me a change of clothes and some things to shower with in the morning.  The kids are all snuggled in bed at our friends the Puff’s house, and Lucy and I are being well taken care of here at the hotel DuPont.       

Too many things…


Times have been tough for our little one, I believe that is the easiest way to sum things up.  This afternoon Lucy was taken to the hospital via ambulance for cluster seizures lasting longer than ten minutes.  These continued for the duration of our ride over here.   For those of you who have ever had a child or seen a child seize, a second feels like an hour, a minute a year, and well ten minutes an eternity.  Like I mentioned in pervious posts, seizures are not something new for Lucy, but the frequency and duration of these seizures are new for our girl.  She has been even more irritable and agitated the past few weeks but especially the  past few days.  We started Lucy on phenobarbital on Saturday and methadone yesterday.  Is it med related, surly we are suspicious,  we don’t really know yet.  There are too many things happening at once, as usual, to say what it is that is causing this.  For now she is sleeping, seizing like she did is extremely fatiguing, and when she is awake she is irritable.  I will update when we know more.  


February 11, 2012

As I sit here, blood is infusing ever so slowly into Lucy so as not to fluid overload her.  She is finally sleeping, something that has been scarce for her this week.  We are praying that this is the boost her body so desperately needs, but I have been told that even though this will help this is not going to “fix” her.  I know this in my mind but in my heart is another matter. Her baseline is changing, I am hesitant to say what her norm is, what was normal last week is different this week.  

Cardiology finally chimed in on all areas of Miss Lucy’s life today which officially got the ball rolling on many things.  We are going to be starting Lucy on some pretty heavy duty meds for pain control, autonomic control, and seizure control.  Lucy has learned to live with a level of pain that is beyond our comprehension.  Acute pain is one thing, chronic pain is another.  As her parents we too have learned to live with a level of Lucy’s pain.  Our hospice team and doctors very delicately pointed out to us that Lucy deserves to be comfortable, and that as difficult as it is going to be to find the right balance of meds, she needs them.  Our biggest fear is perhaps loosing the part of Lucy that makes her Lucy.  The very real reality is that we need to do something for her and we don’t know what that something is going to do to her until we actually do it.  

Our appointment with neurology was pretty straight forward.  Lucy’s previous eegs have demonstrated that she has seizures so there really is no need to repeat an eeg, we just need to decided whether or not we are going to start seizure meds, and might I just add that our options for meds are very few.  Drew and I left our appointment with neurology and parted ways, he went off to work and Lucy and I headed to day med.  We kissed good-bye and said that we will discuss “stuff” later.  As Lucy and I were settling into day med we were greeted by our palliative team and Dr. R, they were all coming to discuss “stuff”, so much for doing it later.  Decisions were made that took some convincing on my behalf , but are clearly in Lucy’s best interest.  We are going to be starting Lucy on phenobarbital tonight because it is an effect seizure med, pain med, and helps with autonomic instability, all areas that she is in need of help with.  In a few days we are going to be adding another med for pain control that will hopefully be a great med for for neuropathic pain relief. 

I want to thank all of you for thoughts and prayers, love and support.  I also want to say a special thanks to all of you who donate blood, without you our little one and so many others would not be alive.  Our prayers are for little to no med reactions, negative cultures, and that Lucy is comfortable.  If you can also find it in your prayers to pray for our three other loves in our life that they too have peace and comfort with all that is going on in our lives.      


February 10, 2012

You’ve heard the saying “worry does not empty tomorrow of all its troubles, but empties today of all it’s strength”.  I’m not really sure who is famous for stating this, but I wonder if this person had a child with a chronic progressive degenerative disease that has them wondering at all hours of the day and night what on earth is going on with their child.  I had strength beyond my comprehension today, and yet I am so worried about our girl.  Something is wrong, different, changing. 

We had a long appointment with our main doc today, which in the grand scheme of things did not amount to any big changes yet, just a lot of what if’s.  We are scheduled to see a new neurologist tomorrow one who specializes in seizure disorders.   When Lucy was a teeny tiny baby this neurologist was one of the first doctors who clued us in that there was something going on with our little one, but steered us toward a neurologist who specialized in neuromuscular diseases.  Ironically our neuromuscular neurologist is steering us back to him. Seizures are not something new in Lucy’s life, but appear to be making a come back.

We are scheduled for a blood transfusion tomorrow, labs and cultures will also be drawn as Lucy is teetering on the fence of a fever.  I am worried that she is brewing something,  add to that worry that our main doc is going to be away for the next two weeks, and I believe that my worry makes me a stronger advocate for our daughter. 

Where I Last Left Off…

February 9, 2012

I apologize for my lack of updates, time is just not on my side these days.  We appreciate so much the love and support that we receive from all of you who know and love us either from the words here on this blog or in real life!

When I last wrote I shared with you that there is a fungus among us.  After several conversations with our main doc it was decided that we needed to investigate other options for treating Lucy’s yeast issues.  Systemic/IV antifungals are tough on the body, most of them come with a list of side effects that are concerning for anyone, but even more so for Lucy.  Her sensitivities to medications are limiting our choices, as well as her failing GI system and liver disease.  Lucy is in intestinal failure, she has little to no function of her GI system and as a result she is unable to take in and absorb meds through her GI tract.  Yet it is her GI system that is overgrowing with bacteria and yeast.  We need to continue to try and treat the yeast and bacteria in an effort to keep it from translocating from her intestines into her blood stream putting her at risk for life threating infections.  It is believed that Lucy’s poor immune function is playing a significant role in her reoccurring yeast infections, these types of reoccurring fungal infections are most commonly found in severely immune compromised individuals such as bone marrow transplant patients, or HIV infected patients, and Lucy.  After consulting with some of the other docs in the diagnostic referral service, Dr. R talked with me about putting Lucy on amphotericin B orally.  Amphotericin B is typically an IV antifungal that is know to have serious side effects when administered IV, it’s nicknamed ampho-terrible.  Interestingly enough when given orally, or in our case via her g and j tubes and swabbing her mouth with it, it should have little to no side effects because it does not get absorbed systemically.  Though not common, we agreed to treating her this way because, frankly, all of our other options come with a long list of side effects that will exasperate many of her existing symptoms and create bigger problems .  Treating Lucy with an “oral” medication means we are taking a gamble that her failing intestines will be able to take in the very small volumes(.5mls) of ampho and move it through her GI system thus coming in contact with the yeast and eradicating it.  In theory this should work; however, any long time reader of this blog knows that Lucy does not follow theory.  Most if not all of the meds we are pushing down her tubes are pouring back into her drainage bags no matter how long I clamp her for.  We are continuing to research options for systemic antifungals.

As for the yeast in her mouth that does appear to be improving ever so slightly.  I am going to venture a guess that oral ampho is perhaps one of the worst tasting medications.  Although Lucy hates the taste of  the med, she is surprising us with her cooperation in allowing us to swab her mouth with it every eight hours.  When we first started swabbing her mouth I said half kidding that there are not enough smelly stickers in the tri-state area to bribe Lucy with for the next two weeks, but she is realizing that it is helping to get rid of the very thick and pasty yeast that has plagued her mouth for months.  She never ceases to amaze us with her ability to tolerate a new therapy, intervention, or medication. Once she realizes how much better she feels she is willing to allow us to do it, although not without tears and some back arching, this is both comforting and heart breaking.  

The last few weeks have been riddled with worry, if it’s not one things it’s ten more things.  Last week her urine had a strange odor to it which prompted me to ask our doc to have it cultured.  On the same day, one of the lumens on her central line clotted off for the second time in two weeks.  We did a dye study on her line but did not find anything obvious that could be causing the line to occlude.  I was not really surprised since seconds before injecting the dye into her line for the study I flushed a med through it first and lo and behold it started flowing like a river.  One sure way  to fix a line…go through the hassle of scheduling a dye study.   We did cultures of her line as well as because we are always suspicious when we have line troubles.  Her line cultures have no growth as of today, which is a good thing, but her urine cultures grew positive for an unusual bacteria that is not a typical  UTI bacteria found in the bladder but rather in the bowel.  Lucy did not present with a high fever which is odd since she has a pretty significant infection, this has us all a little concerned.  She was one miserable girly this weekend, which makes sense now that we know about the UTI.  We started her on IV bacterium to treat the infection, which is now only going to make the yeast issue a bigger issue…ugh, around and around we go! 

Lucy is in need of blood, her counts have dropped pretty significantly in the last few weeks.  We are scheduled to have this done on Friday.  She is also in the midst of another autonomic storm which has affected her sleeping and mine too, motility or lack there of, body temperatures, and heart rates.  She has us all on a roller coaster wondering what on earth we are going to do for her.  Talks between Dr. R and our cardiologist are being had, one of our frustrations is whether or not we can really do anything for Lucy cardiac wise.  We are looking for some guidance as to how high is too high for her heart rates and how long is too long to let them be high.  Pain continues to be a growing issue, Drew and I witness on a daily basis just how strong our little one is when it comes to pain.  On Monday night we had her laying between us in bed, she was twitching and shaking she was in so much pain, but was not crying she just wanted us to lay with her. 

It has been a tough few weeks around here, but we will get through them.  We are scheduled to be at DuPont the next two days for what is going to amount to be loooooong days.  We are in the midst of making some changes to her current meds, looking for more effective pain management.  

My hope is to update more often about the everyday stuff that happens in our life, it’s just that the everyday stuff happens to be everything I have just written and then some.  The then some part will have to wait for another day.  Thanks as always for your thoughts and prayers, love and support, we could not do this alone!