Tuesday, October 30, 2012

Hurricane Update...

Thank you to all of you who have inquired about our well being and how we weathered the most recent storm, hurricane Sandy.   Our biggest fear was loosing power, which would have made taking care of our medically fragile daughter with severe anxiety issues that much more challenging! Thankfully the hurricane came and went with little to no damage to our property, neighborhood, and friends and family.  Our hearts go out to those who were not quite as spared from the effects of the storm, it is going to take quite some time for areas around us to recover.   Schools were cancelled on Monday and again today, as was Drew's office, due to the huge storm and the fact that driving on the roadways was not allowed unless you were an emergency vehicle.   As the eye of the hurricane approached our area all of us sensed the drop in pressure, our ears popped similarly to when you are coming down for a landing in an airplane.  Witnessing the power of this awesome storm reminds us just what little power we really have.  Tomorrow is Halloween and all of our kiddos are excited for all of the festivities.  Here's hoping that it stops raining in time to trick or treat tomorrow evening!

As always thank you for all of your thoughts and prayers, love and support.


Monday, October 15, 2012

Life in the fast lane...

"October already",  I keep saying this over and over, where did September go!  Jack even said yesterday "I can't believe that it is the first week of October already Mom!"...errr," Jack, it's the second, no the third week of October already!!"  Oh shoot me now, it has to be genetic, referring to our poor concept of time, I blame my mother for this... because I can, she is my mom :).  Some days I just want to pull the reigns and bring our life to a screeching halt.

Drew and I both felt like we were just beginning to get into vacation mode on the Friday of our vacation. Then on Saturday we packed it all up, loaded it back in the car, and by 11am and were on the road again back to reality.  Reality hit hard when on Sunday afternoon Lucy's temps starting climbing along with her heart rates, all signs of a legitimate fever.  Life in the fast lane,  shoot (actually, I may have said an even stronger word), I began unpacking one suitcase and started packing another.  Drew was watching the end of a football game when we both looked at each other with that all too familiar look in our eyes, we knew we had to kick it into high gear as life was about to get crazy again.  He headed in the direction of the grocery store and I to our laundry room trying as hard as we could to get things back in order quickly.  I don't know about you, but as much as I would love to leave my house in tip top shape before a vacation so that when we return home I don't get that overwhelmingly feeling of not only do I have to unpack the car, but I have to clean my house too, we never do. Our house looked like it had been ransacked, add to that mess a cargo jet full of luggage, tote bags, sea shells, and medical supplies galore and now you have a better idea of what our house looks like, still.

We put Lucy down for a nap with the thermometer reading 101.1 and heart rates in the 150's.  Fully expecting her temps to soar, we started putting plans A through Z in place.  Everything changed in an instant; my mood, my heart rates, my blood pressure, every muscle in my body tightening...it is amazing how quickly stress can seep back into life and take over.  I called the on call service to see who was on, hoping and praying that it would be our main doc because maybe, just maybe, I could talk him into letting us stay home at least until the morning.  I mentioned in my previous post that before we left for our trip Lucy was not doing well, she has been "off" for quite some time and we're  not quite sure what to make of it.  Believe me when I tell you that there are all kinds of theories being thrown out there, but the bottom line is, we just don't know.  Recently I have sent several e-mails to our main doc with the first sentence reading,  "Don't cringe, but I have a theory...", he appreciates it :).   When Lucy woke her temps had come down just enough to allow us to stay home, but  I was given strict orders to bring her to the hospital first thing in the morning for a variety of labs and cultures, or sooner if her temps went up even a tenth of a degree.  Agreed!

All week we have been watching and waiting, theorizing, and predicting, but the bottom line is we still don't know what's causing Lucy's fevers.  In between trips to DuPont and worrying us to death, Lucy has moments of just being Lucy that gives us all  peace and comfort, but in the blink of an eye that can change; a seizure strikes, an acute episode of pain hits, heart rates soar, temperatures rise...  Her blood cultures are negative so far, call it a sixth sense but both Dr. R and I agree that what we are seeing in Lucy is not looking infection related, if it is she is not presenting like her typical protocol for infection.

Her urine is now growing pseudomonas, along with enterococcus, and staphylococcus.  The psudomonas is new, the other two bacteria took up residence in Lucy's bladder during the better part of the last year.  It is unlikely that these bacteria are the cause of Lucy's fevers because Lucy's urine analysis looked decent enough. We are going to hold off on treating the pseudomonas for the time being, as pseudomonas is one difficult bug to try and get rid of, it likes to morph into an even more potent bacteria.  We are hoping for a peace treaty of sorts to occur in Lucy's bladder, we just finished treating her for yet another UTI last Saturday, the poor girl has been battling one UTI after another for months!

Seizures are the pits, how's that for a Brady Bunch description.  We have been adjusting Lucy's Keppra dosing since she started on the med at the beginning of this year, maxing her out a 1100mgs of Keppra twice daily (BID).  Yes you are reading correctly, that's a ton of Keppra, but that is what it takes to keep her from having clusters of seizures for days at a time, in addition to using phenobarbital and Valium.  We attempted to cut her dose back to 1000 mgs BID, but the cluster seizures returned.   Lucy has broken a record (again) for the highest blood Keppra level of anyone our main doc has treated, this is not something that we are proud of.  However, we are extremely limited in our choices of anti-seizure medicines, there are only three IV seizure meds available and the third one, Vimpat, we have been saving as a last resort.  Our palliative team mentioned to us early on that Vimpat is a good palliative med for when we "get there", referring to the end of life.   In no way do we feel that Lucy is at the end of her life, but the time has come to consider it.  Dr. R has never used Vimpat before, our neurologist has used it sparingly in patients with severe seizure disorders, but not in a patient as young as Lucy or as medically complex as our girl.  It is a relatively new medication that has not been well studied in pediatric populations.  To say we are nervous to put Lucy on this med would be an understatement.  Our main doc fears that Lucy's extremely high blood Keppra Levels could be contributing to her fevers and some other neurological issues, and thus we need to attempt to reduce her levels.  To add to our predicament, there is a serious IV Valium shortage, we use IV Valium for many reasons, one of them is to help stop prolonged seizures.  Valium is the only medication that works for Lucy, the ONLY medication!  Changing or adding a new seizure med could mean that we loose seizure control and thus our need for IV Valium will increase, at the present time we only have a few vials of Valium available to us so we are using what we have sparingly. This is something that all you prayer warriors out there can be praying for, that we will continue to have access to IV Valium and the many other medicines that our little one needs but are on the nationwide drug shortage list, our friend Wyatt and his family would greatly appreciate your prayers for this as well.

Fungus, it's bad, enough said!  We are looking into a new anti-fungal to treat the fungus that has plagued Lucy's intestinal track and mouth for the better part of the last year, but it is not as easy as putting her on it and watching her to see if things improve.  Nope, that would be too easy.  We have to weigh things like growing a resistant fungus, and not being able to treat a fungal line infection if/when it happens because the fungus we are treating in her GI tract becomes resistant to the medicines that we are using to treat it.   This is a very real reality as she has already become resistant to one anti fungal . There are far fewer anti-fungals than there are antibiotics, our options are limited right off the bat.  Infectious disease and Dr. R have been discussing this, he filled me in on their conversation the other night knowing full well that I would spend some time researching this potential med before going to bed, I think he uses me to do his research :).  You want to bet that I had a few questions about this med in the morning that prompted a phone call to Lucy's infusion pharmacist, she is wonderful and always eager to answer or look into any questions that I have.  We will discuss our options and questions with Dr. R in clinic on Thursday.   In the meantime, Lucy's compounding pharmacist is making an amphotericin lip balm in addition too the oral ampho that they make for Lucy in hopes sparing her lips form the intense pain of pealing fungus from her lips every day!   Too bad ampho is the color of mustard.

As we begin the third week of October, Jack, we are hoping that our life can slow down just a little please. Halloween is coming, costume ideas are the topic of everyday conversation, six pumpkins are sitting on our front porch waiting to be carved, and Lucy's favorite scents of pumpkin and cinnamon are filling the air in our house.  September and October have notoriously been difficult months for our girl and Drew and I are keenly aware of this.   We are scheduled to be at DuPont a lot this week for various clinic appointments and follow ups, and as long as we come home every night I am fine with the appointments.  Your thoughts and prayers, love and support are always appreciated!

Monday, October 8, 2012

A Beach Dream....


Oh my it's been a while since I have posted... rest assure that things with all of the Marletts have been busy and chaotic as usual, well almost.  We just returned from vacation, one week at the beach in the Outer Banks of North Carolina!  The house we stayed at was called A Beach Dream, and that it was.  Things were not looking well for Miss Lucy before we left, and to say our medical team was a little nervous about us leaving I think would be an understatement.  Lucy was battling yet another UTI, seizures, and clinically just didn't look well.  She received a blood transfusion just prior to leaving, and our beloved doc extended her antibiotic coverage an extra week for "travel insurance", he is amazing like that!  We took our mini ICU on the road, along with all of the usual stuff you need to go on a beach vacation, coordinated the most complex shipment of meds and supplies, had our emergency protocols all in place, and headed for the beach.
Thanks to all of you for your love and support, thoughts and prayers...we made it to the beach again this year, another answered prayer!
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