Cystine Levels

Every time Sam goes to California for the drug trial, they take a blood sample to measure how much cystine has accumulated in his white blood cells.  That's how they track how well the drug is working.  We are always stressed out about his levels because Sam's tummy doesn't empty very well, and the drug will get activated too soon if it comes in contact with dairy or fatty foods.  This is a problem since the drug is extended release (every 12 hours instead of every 6), so if too much gets activated too soon, then he won't have enough left in his system to hold him over till the next dose.

His last cystine level came back at a 2.4, which is REALLY high.  He is supposed to be below 1.  Our doctor at Stanford thought it was most likely a lab error, and didn't recommend boosting Sam's dose.   We took him to Primary Children's Hospital to get the same test immediately after we got the Stanford results.  It takes about 4 weeks (at least) to get the results of the test back, so we've been pretty nervous for the last month. 

Our nurse practitioner called us two days ago to let us know Sam's cystine levels were 0.55.  That's low!  We wish they were down that low all the time.  So we're relieved to know that Sam's actual levels are probably somewhere in between, but it's still unnerving to know that they fluctuate that much.

The good news is Sam is a hyperactive, always-on-the-go little boy, and from the outside, he seems to be doing great.  We hope his kidneys are doing just as well.

Super Sambo

Born To Drink Apple Juice

 

 Samuel has NEVER liked juice.  In fact, he's never really liked anything in his mouth expect for maybe a salty goldfish cracker now and again that he'll spit out after a few seconds.  His g-tube supplies 100% of his nutrients every day.  Every day feels like a battle just trying to get him to put something in his mouth.  We try not to over pressure him.  He is very stubborn.  Tonight at dinner he "had some peas with his salt" and then demanded some water.  We told him he could have water after he took one sip of apple juice (pick and choose your battles sort of thing).  He refused at first but finally caved.  Stephen and I started discussing fun rewards and charts we could make as incentive to get him to put food in his mouth.  When we mentioned purchasing the movie "Born to Be Wild" as a reward Sam piped in and shared that he thought it was a good idea.  He has seen the movie in the theater nine times now.   Stephen quickly drew a palm tree with an orangutan on it and every time Sam took a sip of apple juice he could color in one space climbing up the tree.  We thought he would stop around two or three sips and we would be working on this chart for the rest of the week.  Stephen and I were both shocked to see him take over 30 sips of apple juice (more than half the juice box).  All three of us were so excited we rushed to Target to buy their last copy of "Born to Be Wild" on DVD.

 

 

Puhhhlay Doctor

Sam and Tallinn are both very knowledgeable  about doctor equipment.  I was impressed

After an hour of playing doctor they moved on to bouncy balls.

I probably shouldn't have encouraged this.

another ER visit

With two minutes to get out the door for pre-school I quickly sat Sam on the kitchen counter to put on his "water" shoes.   Unfortunately, Sam's g-tube extension slipped between the counter and the stove so when I plopped him off the counter, out popped his completely inflated button.  Ouch!  First time ever.
His button has popped out before but only when the balloon had deflated and wasn't tight enough to stay in his tummy.  Sam just stared at me and grabbed his belly like "What was that?!"  It wasn't until I told him we had to put it back in that he started crying.  I tried laying him on the bed, then the couch, put a movie on, bribed him with the movie theater, but he would not hold still.  I sat on his legs and put both my feet on his hands to get him to hold still.  I'm sure he'll be able to write many lovely memoirs of his mother after these experiences.

He would not hold still and I could not get his button back in so I told him we had to go to the hospital.  He was pretty bummed that he had to miss pre-school and I think he was pretty scared that he would get an IV at the hospital.  I asked him if he wanted to say a prayer to help him be brave and he asked me to do it.

On the way to the hospital I called the g-tube clinic and they told me to bring him to the ER.  Worst place EVER.  Luckily we were the only people there.  When the nurse called Sam's name he stood up and shouted, "No!  No way!  I don't!"  This was going to be pleasant.  After explaining to him that he was just getting vitals he quickly turned chipper, Oh, oh yeah, mom?  Okay."  After that we were quickly whisked away to the Rapid Treatment Unit (RTU) where the pediatrician could not get his button back in either.  A specialist from the g-tube clinic came and dilated his hole so that his button would fit back in.  During all this Sam looked completely miserable,  clenched every muscle, but held very still.  Afterwards he sat up and said, "We're all done.  Let's go Cafe Rio".  Well done.  You were brave, Sam.

happy mother's day!

                song: ho hey, by the lumineers

Alphabet!

Recently, I read an article by Dr. Trauner who studies learning differences in children with cystinosis.

http://www.cystinosis.org/filemanager/file/Cystinosis%20Article%20Library/Nervous%20System/Accommodations.pdf

 Ahhhhhhhhh!!!!  Just joking.  I'm not scared.  Sam is such a smart kid.  He knows the entire alphabet and is getting better and better with his numbers.  He tells some pretty funny jokes too.  This article motivated me to pick a couple of different activities to work on every day.  We spent almost two hours last week making a mural on our wall and then sticking letters all over it.  Almost every day Sam will say, "let's do this again!" and starts pulling off all the letters so he can put them all up again.
I do sense that school may be a bit of a challenge when that time comes but since we're aware of these possible difficulties we'll be able to work harder with him.  If you ask him where he's going to school he'll say, "BYU!"

 

Sam's favorite free websites

Curious George's Busy Day

Starfall

elmo and sam

we made this movie after watching "being elmo."  turns out it's hard to make movies with two-year-olds, but he did pretty well.

Keepin' The Blog Alive

 

We're so sad that we could not make it to the CRF conference in Newport, CA this year.  Sam has grown so much since we were there last year.  One of Sam's nephrologist's was kind to let us know that the CRF announced the okay for the first bone marrow transplant trial.  This has come sooner than I thought.  Yay!!!  Also,  I hear the CRF raised $1.75 million last night.  All funds go towards medical research.  Thank you CRF!

On a sadder note...  Annie or "baby dog" is missing.  Any information that can lead to her whereabouts will be greatly appreciated.  This too is HUGE for Sam.  Thank you.

Our desktop is dead and our laptop has no more space

 

So these photos are from weeks ago.  As soon as we can get photos off my camera we will show you our/Sam's new toys. 

Samuel discovered the storage space where all his g-tube extensions are kept and decided to link a bunch together.  He's pretty good at figuring out how to connect and disconnect his tubes now.

Sam refuses to give up his crib for a big boy bed.  

Henry and Sam had fun pulling ALL the leaves off this tree.

This is a picture of me.  I helped give myself the arms but Sam made sure I was wearing one shoe.

Sam is moving on to chapter books.  We finished The Tale of Despereaux in four nights.