Thursday, May 31, 2012

Learning to say "P"

Sam likes to use the letter "d" to start every word he uses.  For example, "det's doe doutside".  His speech therapists plays some fun games with him to help him use his other letter sounds.  Today we "painted pictures" and did other things that start with the letter "P" since that is one sound he has been working on.

Tuesday, May 29, 2012


My mom and Sam had a "sleepover" on Sunday night and she asked if she could do all of Sam's medications during her visit.  She's going to come stay with us again in July to help out when baby #2 comes.  Yay!  The help will be greatly appreciated.  This is Sam's current schedule for those who have asked about it and want to learn how to use a g-tube too.  G-tubes are pretty much awesome.

8:00 AM – 250mg RP103 (3 large pills, 1 small pill, 20ml orange juice, 50ml applesauce)
9:00 AM – 3ml carnitor, 1.5ml sodium, 3.25ml polycitra K, 1ml iron, 0.5ml vitamin D, 3ml potassium
9:30 AM – mix food for the day (2 boxes boost, 1 packet phos nak), 16 minute feed on rate 500
12:00 PM – 2nd feed, 15 minutes feed on rate 500
1:00 PM – naptime (don't put him down sooner or he'll throw-up his last meal)
3:00 PM – 3.25ml polycitra K, 1.5ml sodium, 3ml potassium
3:15 PM - 3rd feed, 15 minute feed on rate 500
4:50 PM – last feed until bag is gone, rate 500
7:00 PM – thoroughly flush tube
8:00 PM - 250mg RP103 (3 large pills, 1 small pill, 20ml orange juice, 50ml applesauce)
8:00 PM – hook up to orange juice drip on rate 30 (3oz orange juice, 3oz water)
9:00 PM - 3ml carnitor, 1.5ml sodium, 3.25ml polycitra K, 1ml iron, 0.5ml vitamin D, 3ml potassium
10:30 PM – mix night food and put on rate 58 (1.25 boxes boost, 4oz water, 3.25ml polycitra, 1.5ml sodium, 1.5 packets phos nak, 3ml potassium)
6:00 AM – switch food bag with orange juice bag.  Change rate to 30.

Saturday, May 26, 2012

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.

Tuesday, May 22, 2012

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.


Monday, May 21, 2012

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.

Friday, May 18, 2012

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.

Saturday, May 12, 2012

Friday, May 11, 2012


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