Oh What a Week…

And it’s only Wednesday!  Medically speaking things have been stressful, and today’s VCUG and urodynamics study results only added to the stress.  The results confirmed what has been suspected for some time, Lucy suffers from a neurogenic bladder.  Even though we suspected this; hearing it, discussing the results, and comprehending what we are going to have to do for Lucy is difficult.   The nerves in Lucy’s bladder do not signal her brain  to empty when it is full.  We are going to have to “help” Lucy empty her bladder.  From what Lucy’s urologist told me, Lucy’s results showed that she has most likely been suffering with this for some time. 

The study was not pleasant but Lucy did better than she would have done last week or even at the start of this week.  I believe that this is because over the weekend we started Lucy on Risperdal, an antipsychotic medication that we are using to help with the agitation, irritation, and manic episodes that have been plaguing our little one’s life recently.  Since starting her on this med, Lucy has finally gone back to sleeping at night and even napping during the day.  This is a welcome change since it has been over a month since Lucy has had any sort of sleep pattern.  Of all of the meds that Lucy has been or is on, this med (because of the type of med that it is) was the most difficult med for Drew and I to decide to put Lucy on.  This is not just because of the type of med it is, but also because of the side effects that this med can cause.  Most worrisome for Lucy is irreversible tardive dyskinesia, a movement disorder .  Dr. R and I discussed the use of this med all week, and on Friday he and I decided that its benefits could out weigh the risks.  I have literally been nauseous this week just trying to adjust to all of this. 

Yesterday after our regularly scheduled appointment with Lucy’s orthopedics doc, Dr. R wanted to see Lucy in clinic again to discuss some things.  He is concerned about our girl, in his words “she is not going in the right direction.”  Lucy is up almost another kilo of weight, this has us all concerned.  She is third spacing or retaining fluids and has been since getting sick in April and we are not sure why.  Dr. R ordered a ton of labs yesterday to help us pinpoint a cause or possible causes for this issue.  Lucy is anemic, she has been for nearly a year, but her blood counts are not terrible for Lucy, in fact they are up slightly from last week.   Lucy’s albumin level is low, but before infusing her with albumin we are going to be doubling her daily Lasix dosing to see if we can drawn off some of the fluid this way before bringing her in for an infusion of albumin.  Lucy’s liver numbers are rising even more, but this could be because we ran a lipid bag with her TPN on Monday .  Lucy’s skin is scaly and peeling, indicating a need for essential fatty acids that can only come from the lipids in her TPN.  Lucy’s triglycerides are high but not nearly what they were when she was lipemic.  Lucy has gone from running lipids daily before getting sick in April, to running lipids once a week and just TPN fluids the remaining six days in the week.  So many changes, so much uncertainty.

In addition to all of the medical chaos, we started with a new nurse this week.  So far so good.  I can only imagine what she must think of our of our crazy,  busy, exceptional life after coming in on all of this medical drama this week.

Tomorrow Lucy starts back with her new speech therapist here at our home.  We have had a few sessions with her since transitioning Lucy from our county’s birth to three program (EI), to our county’s intermediate unit (IU), the birth to age five program.  When Lucy turned three she aged out of EI and transitioned to the IU where we started with a whole new team of therapists, rules, schedules, procedures, assessments… I miss  Lucy’s old team not only because we were all familiar with one another and with Lucy’s issues, but because they were exceptional!  On Friday of last week I met with Lucy’s service coordinator and the OT who preformed Lucy’s original assessment to help them finalize Lucy’s individualized education plan(IEP).  In the midst of all of this changing of therapies, Lucy has changed so much from a medical stand point that nearly all of the goals for her IEP have to be revised…ugh.  Tomorrow we are hoping to accomplish this.

All of this and it is only Wednesday…      

Rough Week…

My heart is hurting as I type this, our little one is struggling.  Her health is declining,  she is changing both physically and mentally.  There are moments when Lucy is Lucy, and moments when Lucy is extremely irritable and agitated, screaming inconsolably for hours.  Common everyday situations can set her off, and there is really nothing you can do to calm her.  Dr. R was able to experience first hand last Monday Lucy’s “new” behavior.  He has witnessed her behaviors in the past, but for over an hour he was able to really experience what we have been describing to him, and we all agree that this is new and much worse than ever before.  Lucy has always struggled with sensory issues, obsessive compulsive behaviors, and irritability.  She is not diagnosed autistic but has autistic-like tendencies which we have learned to work with and continuously try to manage.  These new behaviors are different, heighten, severe!   We know that Lucy’s brain chemistry is different, she has paradoxical reactions to most medicines that we have tried for her.  Her neurological system is affected as a result of her mitochondrial disease.

We are not sure what exactly is causing Lucy’s new issues…is it a side effect of a medication, a paradoxical reaction to the morphine, or disease progression?  It could be any or all of the above.  We need to wean Lucy off of the morphine in an effort to isolate the cause of her heightened behaviors.  Dr. R is hoping that by weaning her off of the morphine that we will see a decrease in the intensity of these behaviors.  At the same time, we are in need of achieving some level of pain control.  It is thought that Lucy could be experiencing neuropathic pain, morphine is not necessarily the first drug of choice for neuropathic pain.  I have a love-hate relationship with narcotics, they were absolutely what she needed to get through her most recent situation, but I hate what they have done to her and the weaning process.  It is awful seeing your child, your baby, go through withdrawal.  We are weaning her slowly but aggressively. 

This is so tough…mito sucks!    

Back to Reality…

We returned home from the land where dreams come true late Saturday night, actually it was more like very early Sunday morning.  We had a fabulous time and are so grateful that we had the opportunity to go on this trip of a a life time!  We have so many wonderful memories that continue to be talked about over and over by every one of us.  This was our hope, what we wanted most of all…lasting memories! 

Our trip home was not without stress, our flight was delayed due to mother nature.  Lucy’s last TPN bag had enough fluid to last us until at best 11:00pm. When our original flight did not take off at 5:00pm Drew and I started the silent panic, you know the looks that are shared between husbands and wives that if spoken would say something like holy s**t!  If we had another TPN bag with us we would have changed her out but due to the craziness of our life and all of the extenuating circumstances surrounding everything about this trip, we just had enough TPN shipped to us for the time that we were in Florida.  A new shipment of TPN was waiting for us at home.  When at 7:30 we were still delayed I was beginning to panic,  Drew and I thought about our options and came up with the idea of cutting Lucy’s infusion rate in half in an effort to stretch the bag.  We called our infusion company and spoke with the on call pharmacist about our predicament and what we wanted to do.  Our biggest issue was hypoglycemia, Drew and I knew that if she ran out of fluid that her glucose would plummet into the 20’s or 30’s quickly, so our hope was to keep her running at a rate where we kept her glucose above 50.  The pharmacist on call was not the pharmacist that we work with usually, but it didn’t really matter as everyone at our infusion company knows Lucy, her issues keep everyone there employed.  Due to our extenuating circumstances she nervously agreed to our plan and helped me to reprogram Lucy’s TPN pump.  This change was what saved us.  Our flight finally left Orlando at 8:30 pm, and when we arrived home at 12:30am Lucy had just minutes left on her pump.  This was all way too close for comfort, but we made it…just barely, but we made it!  On Monday morning our usual pharmacist called and applauded us for our idea, though it was not something they have ever done before, it worked. 

There has been a whirlwind of events around here since coming home that are keeping us on our toes.  Jack brought back a souvenir from Florida, which warranted a trip to our ped’s office first thing Monday morning.  He has a staff infection on his face and back of his left leg that is classic yet not commonly seen…oh yes, leave it to us to have something so unusual.  A script for antibiotic ointment and instructions on Lysoling our home were given and we were on our way.  Lucy had an appointment with Dr. R and the lab on Monday as well.  She too brought back a souvenir from Florida, a urinary tract infection.  We started her on IV antibiotics in hopes of getting rid of the infection before next Wednesday when she is scheduled for her urodynamics study and VCUG.  Sophie also had a little souvenir that we brought back from Florida  just in case, she had a tick bite in her head. According to Helen our nurse, it had to have been there for some time as it was pretty big.  The good news is that it is not a dear tick, the kind of tick that carry Lyme's disease, it is a wood tick the kind that literally fall out of the trees this time of year in our neck of the woods.  Thankfully there is nothing really exciting to report on when it comes to Megan. 

I am trying to settle back into “normal life”…if that’s even possible.  Tuesday and today are the first two days in a row that I have spent at home in over a month!  I have so much catching up to do I don’t even know where to start…I seem to be doing a little here and a little there with no real progress in any area.  Lucy’s med schedule, medical needs, and dramatic behavioral changes are overwhelming!  We have had some promising leads on qualified nursing which I am hopeful will be an answer to our prayers, but at the same time it is another new change that we will all need to adjust to.

I will post a medical update soon as there is too much to write about tonight.  I am also working on putting our pictures together from our trip, hopefully I will be able to post those soon.

Making Memories…

I have wanted to sit down and write so many times this week, but we have been too busy making memories!  Let me preface this post by saying that I am not sure that I can write in words adequately how we have been feeling,  After coming straight from a very difficult admission to the land where dreams come true, our emotions have been all over the place!  We have truly been blown away by all of the staff and volunteers, affectionately called angels, at Give Kids the World.   They sincerely want to make your visit here extraordinary!

During our first night we were here “an angel” who was handing out warm cookies and lemonade on our way back to our villa asked us how we were enjoying or time here.  He asked the kids if they knew what the number one rule was.  Megan replied,  “Do you mean the golden rule?”.  He said, “No, I mean the number ONE rule.  The number ONE rule is to… HAVE FUN!”.   Then he asked the kids if they knew what the number TWO rule was?  Megan replied,”umm, no?”.   He replied, “The number TWO rule is to… HAVE MORE FUN!”.

The facilities at GKTW are absolutely amazing, from the 2 bedroom 2 bath fully equipped villas, to spectacular handicap accessible pools and water park equipped with PVC wheelchairs so that all can enjoy. 

Beautiful gardens and nature trails with paved paths so that those of us with kids in chairs can enjoy it too.  A  kid’s theater with a new venue every night.  An ice cream parlor open all day for ice cream whenever you would like, with adorable tables and chairs in every restaurant that are kid sized and designed to look like or are made from something sweet.

Handicap accessible doors on everything,  even all of the public restrooms (I have not had to do the hold the door open with my foot  and spin around backwards to catch it with my backside maneuver all while I push Lucy’s chair in the door; ).

A kids spa where we have had nails painted, makeup applied, and a few or more airbrush tattoos applied on just about every exposed (yes, I have a few rules this week) body part. 

A fam favorite has to be the carousel,we have ridden on it at least 50 times, we are being recognized for this around GKTW !

The carousel is one of only two handicap accessible carousels in the world!  Lucy has her choice of riding either a turtle or a snail, she prefers the snail, she says it goes faster. 

Oh and the train, they have a little kids train that too is handicap accessible, Lucy’s chair is able to be wheeled right on!  Our little girl love, love, loves trains, without going into detail we have not had the best of luck riding on trains with her wheelchair, but here she can ride in her chair and as many times as her little heart desires!

Our kids have been spoiled all week, all four of them.  Everyday there is a special surprise waiting for them in the morning, it’s like waking up to Christmas every morning.  Speaking of Christmas, today is Christmas at GKTW.  Every Thursday they celebrate Christmas complete with snow, sleigh rides, and Santa.  The reason for this, some of the kids that are here may not live to see another Christmas.  Our kiddos have been looking forward to Christmas all week, Jack is questioning how they are going to make it snow in 98 degree weather,  it has been  HOT here this week.

Reality has hit us on many occasions during our vacation of a life time.   Lucy’s pain level and tolerance for things has been excruciating at times.  It has been eye opening to Drew and I as well as the kids, she is not the same as she used to be.   After finally arriving in the Magic Kingdom on Monday around lunchtime, Lucy made it loud and clear that she was most unhappy in the happiest place one earth!  Our other kids could see that she was miserable and all three said with compassion and sincerity that if we had to leave that would be “ok” with them…really, they had only just arrived and were willing to leave to make Lucy happy!  That moment, though obviously not the best moment, will forever be ingrained in my memory.  What child would be willing to leave the Magic Kingdom without having done anything?  We have been blessed with truly remarkable children!

Disney cast members approached us as Lucy’s screams indeed sounded like we needed to call 911, the members were kind and compassionate allowing us space to do what we needed to do all while trying not to disturb the other guests.  I called Dr. R and without too many words spoken he doubled her already increased morphine dose and told me to give it to her every two vs. every four hours.  I love that he just did this, although I think the sound of my voice and the screams from Lucy in the background helped him with his decision.

Our time at the Magic Kingdom was better after that.  Lucy was more comfortable and able to go on many rides with her chair.  We went on everything she could possibly go on in Fantasyland and Tomorrow Land.  In Adventure Land and Frontier Land Drew and I divided, he took the older three while I took Lucy to the nurses station to med her and change out her TPN.  An hour later we tried to meet up for the parade and fireworks show; however, that was impossible as crowds had gathered everywhere except around Lucy and I.  I managed to park us right in front of a magnolia tree where we had absolutely no view of the castle, but because of that we had a lot of space around her wheelchair.  Just as the fireworks show began Lucy fell asleep…she missed it, she missed seeing the “pops”, one of the two things she had been talking about seeing for weeks.

As the music started, I looked over and saw Tinkerbelle flying from the castle and at that moment the tears began flowing.  It was as if at that moment I realized that no matter how how much I want my little girl to see it all, it just might not be possible.

Happy Mother’s Day!

Indeed it is! 

We did it, we accomplished what seemed like the impossible!   We arrived at Give Kids the World around lunchtime! 

We are here!

Don’t even ask how many bags and suitcases we brought with us…it was too many to count.

 

We made it through airport security with dozens of vials of IV meds, liters of TPN bags, at least 100 syringes of sterile saline, bags of needle syringes, and with Lucy of course.  Security was kind and compassionate, they did what they had to do but with understanding and respect.  I was impressed with how well they handled our family’s needs.  I’ll be the first to admit we are a TSA nightmare. 

The kids all helped pull or carry something through the airport.   When we arrived in Orlando we were greeted by a volunteer from Give Kids The World, who also brought a cart with her to help us with what she could only assume was going to be a lot of luggage.

All of the kids did well on the flight, even Sophie Lucy.  It was not easy moving Lucy in and out of her chair, she is in pain and moving her makes her even more uncomfortable.  After several e-mail conversations with Dr. R we increased Lucy’s morphine dosing considerably.  We received a shipment of more meds around midnight as we realized that with the increased dose we would not have enough meds to last us through today.  We are praying for pain control for our little one, as it is unbearable to see her hurting!

To say that I am emotional today would be an understatement.  Being here, at this amazing place, with my family… is truly a blessing.  

Thanks for all of your thoughts and prayers, being here is an answer to so many of them! 

Happy mother’s day to all of the moms in our life!

Give Kids The World…

Anyone who has had the privilege of staying at Give Kids the World, can hardly talk about it without sheading some tears of joy.

The Today Show did a segment about Give Kids the World and it’s founder, Henri Landwirth, last summer.  Click on the link below to see it for yourself…

http://www.disunplugged.com/2010/08/21/video-give-kids-the-world-featured-on-nbcs-today-show/

Home…

Just a quick post to let you know that we are finally home, we arrived home at around 10 pm.  As we were leaving the hospital Lucy left screaming from pain and fatigue.  We were able to administer her night time meds before we left which made it just a little easier for us to come home and put her to bed in her own crib. 

Again, the enormity of the last three weeks hit me when we were loading our girly up in the car with even more tubes and bags hanging off of her than before.  She is so fragile, I would be lying if I said I wasn’t nervous about bringing her home.

Please pray that tomorrow brings a more pain free day for our girly.