An “Off” Day

We are bound and determined to celebrate Halloween-at- home this year, and we were going to kick off our home Halloween weekend with a hay ride out to the pumpkin patch, but… it’s snowing, it feels more like we should be getting our Christmas tree.  I stepped foot into a Target for the first time in months on Thursday looking for wings for a monarch butterfly costume that Sophie very firmly stated she wanted to be for Halloween.  Although yesterday she very firmly stated that she wanted to be a “white kitty cat, the very furry kind”, seriously.  Guess where I  found the costumes… right next to the Christmas decorations, what!?!  I already feel behind the eight ball so to speak all of the time, seeing Christmas decorations out so early just doesn’t seem right, anyone else want to rally around this with me?  I have been feeling more than ever that I just want life to s-l-o-w down!

I woke up last Sunday morning fully prepared to go to church when I leaned over to Drew and said lets take the kids to Sesame Place instead today.  It was a warm and beautiful fall day, perfect “Lucy weather”, he smiled and in an instant plans changed and a day full of memories were made.  I am so grateful that we seized the day knowing what I know now.

Lucy is “off”, something is not right with our girl.  She looks, for lack of a better word, “sick”.  She is sleepier, grumpier, sneezier…her symptoms are similar to those of the seven dwarfs.  Her coloring is, well,  whiter than the snow covering our front yard.  Her body temps and heart rates have all taken a dive down.   Clearly her autonomic nervous system is not happy.  We are praying that this is just a bad mito day and nothing worse, but my mama radar is saying something is up.

Hopefully the snow melts in time for trick or treating on Monday, if not our  kids are all going trick or treating as ski bums.

Beach Memories…

Lots of memories were made during our beach vacation… it’s hard to believe that it was just a little over a week ago that we were basking in the sun, with the wind at our backs, and sand in between our toes.  We thought long and hard about how a vacation like this would work for our family.  As you can imagine bringing four children anywhere is challenging, but having one very “special” child, one who can not get wet because she has four IV lines hanging off of her body at any given time, a urinary catheter taped to her leg for most of her day, two drainage bags attached to her g and j tubes, for the next month a cardiac event monitor strapped to her chest, temperature regulation issues, and at times has sensory issues beyond comprehension….well, you can only imagine the challenges that we faced.  Lucy’s first time at the actual beach lasted, at best, ten seconds.  She was drawn to it however, I think watching “her kids” run in and out of the waves and play in the sand with such enthusiasm intrigued her, despite her nervous system signaling her brain that it was too much.  By the end of the week we were ALL able to enjoy the beach for forty-five minutes as a family of six!  We rented a house right on the beach so that our three water babies could enjoy the sun, sand, and surf whenever they desired and for as long as they desired, all the while keeping Lucy on her strict schedule.  While Lucy napped, the mama read two novels and buried her feet in the sand, the daddy built sand castles and hunted for sea shells, and the kids were thrilled with just being at the beach! 

When we left, more than a few tears were shed, and conversations about doing this again next year were had…all signs of a great vacation!

The view from here…

Enjoy!

Sophie sells sea shells down at the sea shore…

That is if we would let her.  Our kiddos have been fund raising machines this year, coming up with all sorts of ways to raise money: pizza night, lemonade stands, fruit and vegetable stands, carnivals, bracelet sales, comedy shows… and in their own way raising awareness for mitochondrial disease all in an effort to “save Lucy’s life”.  For our kids, this is something that they CAN do to make a difference, what we as parents need to do is to gently inform them that raising money for a cure does not mean that there will necessarily be a cure in Lucy’s lifetime, but our hope is that their will be.  This is so hard!

We made it to the beach!  We still can’t believe it, packing and planning for this beach get away was almost too much for this mama who prides herself on doing the impossible.  Lucy’s needs are enormous!  Planning, packing, coordinating shipping of her medical supplies and medical therapies with our infusion company was nearly impossible, refilling prescriptions, staying on top of her med schedule, changing TPN on the side of the road (I have changed TPN in the most unusual places from the Magic Kingdom to a remote parking lot on the side of the road), and packing just the basics for our family of six, was A LOT! 

It took me almost all of the three days I was home from the hospital with Lucy to make this happen.  Drew and I nearly got divorced  (ok not really, but fatigue and stress are not good for anyone much less anyone's marriage) in the process of packing to get away…but we made it… still married I might add.

Even though we had to pack what seemed like our entire house and a hospital to leave town, we need this week away…I need this week away!

Home Sweet Home…

We finally made it home tonight a little before 7:00PM…can you say loooooong day! 

We were discharged this morning at 11:00 AM, just in time to head downstairs for an outpatient therapy appointment with Lucy’s PT.  Afterwards we went straight to the outpatient pharmacy to attempt to pick up a prescription that I normally order from another pharmacy, but did not because we have been in the hospital for the past twelve days.  Lo and behold, the script that the resident wrote was incorrect, so we proceeded to march upstairs to track down someone who could call in the correct script.  While upstairs we were getting ready to begin the marathon of med infusions that occupy a majority of our day when Lucy’s small lumen, the one that would not flush well yesterday, began alarming “down occlusion”.  This occurred at the same time that Dr. K just so happened to be on the floor checking on his patients from yesterday, he was shocked that her line was not flushing as he said it flowed like a dream when he checked it in the OR yesterday. He ordered Lucy’s line to be TPAed (Is that even a verb?), which meant that we had to be readmitted! 

The first round of TPA did not work, so we attempted to try again.  Five hours later, Dr. K came upstairs with the IV nurse to try it for himself, it was then that it began to flush better than it had, and even better we were able to draw back blood.  Discharge papers were signed again and we were on our way home!

Now that we are home we began the process of organizing all of the meds, supplies, and med schedule.  It is going to be a long week as the only time during the day where Lucy is not being infused meds is between 2:00 and 5:30 AM and PM.  I am going to need to take advantage of those three and a half hours twice a day to nap.  The reward for a week like this is going to be a beach vacation!

We are all happy to be sleeping, if only for a few hours,  under one roof tonight!

Still here…

It has been a long day, week, admission…

We went down to the OR around 1:3o.  They were ready for our girl.  Dr. K explained his plan and told us that it was going to take him as much time as it would take him…words that were both comforting as well as worrisome.

(Dr. K made Lucy a tiny gauze heart to cover up her sutures:)

A little over an hour later he came out and told us that things went well.  He had a surgery fellow assist with the procedure, Dr. K said that he was breathing down his neck the entire time.  He explained to the fellow that this CVL surgery is life saving surgery for our girl, without this line she would not be here.  Dr. K is very particular about how he places a line in a kiddo like ours…in other words he told the fellow not to screw up!

When Lucy came out of the OR that is when things became difficult.  Dr. K left her femoral line in because she was still infusing her antibiotics through that line.  The plan was to get her to the pediatric anesthesia care unit (PACU) and pull her femoral line before she woke up fully from anesthesia.  Once they began snipping the stiches on Lucy’s leg she woke up, and might I add, not happy!

They called us back just as soon as her fem line was pulled.  She was unhappy and made sure everyone knew about it.  We were waiting for radiology to take an x-ray of her new line so that we could get the ok to use it.  This is when I noticed that they were only running D5 through an IV in Lucy’s foot!   Lucy is used to triple the amount of dextrose at quadruple the rate they were infusing it.  Needless to say her glucose plummeted and the PACU went on a wild goose chase to find Lucy’s TPN, you can’t connect her old TPN  line to a new clean line because of the risk of infection.  Only here’s the problem, TPN was never ordered for Lucy tonight as we were planning on going home this evening on our home TPN bags.  This caused quite a stir in the pharmacy, on our floor, and in the PACU… what fluid were they going to give Lucy? As we were waiting for a higher dextrose fluid to be ordered for our girl I  instructed the PACU nurse to bolus Lucy 60cc’s of D10 straight into her IV to help bring up her glucose, this is when the nurse told me she needed to get orders to do so…really!?!  Thankfully a nurse who knows Lucy from the ER was working in the PACU, she remembered Lucy’s hypoglycemia issues and agreed to blousing her, telling the other nurse that mama knows best.  Oh the drama…it only continued when they were trying to figure our what rate to run her D10 at, nobody could seem to get an answer.  Poor Lucy she was cranky and irritable and her parents were quickly feeling so as well. 

We left the PACU just as quickly as the nurse could document all that needed to be documented and we were returned back to our room where they immediately doubled the rate of Lucy’s D10 infusion.  When we arrived were still had not received word that we could use Lucy’s new line.  People started panicking as we only had one point of access, in Lucy foot mind you, to infuse everything.   The IV team nurse finally got approval to use the line, this is when we noticed that one of the lumens on her line would not flush…ugh!  The hope was that it clotted off and we could fix it by locking heparin in it for an hour to break up what if anything was blocking the line.  This is when we made the decision to stay another night instead of going home.  

The line is working and we are able to run continuously through it, but it is sluggish.  We are going to talk to surgery about the line in the morning.   As for tonight, we are working on getting Lucy back on schedule with all of her meds.  She is on an every six hour med schedule with her antibiotics that run for two and a half hours, thirty minutes prior to running her antibiotics we need to infuse IV Benadryl and IV Tylenol to help with the red mans that Lucy gets from the vancomycin.  This is in addition to the nine other med infusions that we run daily…yeah, sleep is going to be an issue this week. 

Even though we are anxious to go home, I am welcoming the nursing help tonight as we work at getting Lucy’s meds back on schedule.  We have just “a few” details to work out in the morning, but we should be all set for discharge sometime tomorrow morning.

Our Weekend…

Our weekend actually went by faster than I thought it would.  Medically, Lucy did just what Dr. R ordered…to behave!  Her cultures have all been negative, which means that our fear of reinfection is no longer, and her new line can be placed.  We are scheduled for surgery tomorrow afternoon with Dr. K again.  Afterwards we are hoping to pack up and go home!  I spoke with our home infusion company on Friday and gave them a heads up that Lucy has a few new meds that she will be coming home on, one of the more difficult meds to find will be her IV Tylenol.  This is a relatively new med to become available in IV form, and I for one am thrilled that this med is available for our girl!  Our options for pain and fever relief are minimal as all of Lucy’s meds need to come in either IV or transdermal from.  Our infusion pharmacist has never had an order for IV Tylenol or even knew that Tylenol came in IV form,  but told me if DuPont has it then she should be able to get it.  

On Saturday the kids came to the hospital to help celebrate a birthday for a Wyatt’s sister…unfortunately, we know all too well how being in the hospital can interfere with family/holiday plans.  Our family, combined with Wyatt’s family, and the third mito musketeers family made for one big party.  Three mito kids and all of their pumps, wheelchairs, tubes, and medical needs at one birthday party was a nurses nightmare, but all went well.

Today was a cold and gloomy day, Lucy and I took lots of walks around the hospital to pass the time.  Our friend Cherie came to see us and gave Lucy a mani/pedi.  Lucy chose to paint her toes purple…I wonder what the OR staff is going to think about that tomorrow.

Please say a prayer that tomorrow goes as smoothly as possible as we are looking forward to going home!

TGIF…

Really, I will be saying TGIM on Monday when Lucy can get her new line put in and we can go home.  Today was another day of waiting for bacteria to grow, or rather not grow…so far no growth.  Medically speaking today was an uneventful day, Lucy’s red mans was better than yesterday, but not gone.  We are watching her carefully for any increased signs of reaction.  Her blood pressures are on the rise and we are not really sure why, we are keeping a close eye on those too.  She is on a pretty hefty dose of clonidine so her pressures are concerning, but for the moment we are monitoring them closely. 

Today I had a meeting with the new palliative care doctor that DuPont recently hired.  She came from CHOP and trained with the very established palliative care team there.  She knew about Lucy and our family from our consult that we had with the palliative care team at CHOP way back in December, and was very excited to meet us.  Due to the multiple hospitals stays that we had over the winter and spring I never really followed through with the CHOP palliative care team.  I was very excited to learn that DuPont has started their palliative care program.  It was difficult to discuss all that we needed to discuss without Drew here and with Lucy demanding this and that.  We agreed that we should arrange to meet soon without distractions and with my husband next to me. 

Drew and the kids came to the hospital tonight for our usual Friday night pizza night.  We told them that we were not going to the beach tomorrow…but rather we will be going next week!  Drew and I are rearranging everything to make this happen.

Tomorrow we have a few fun things planned here at the hospital with the kids.  We are trying to make the best of a less than ideal situation.