Thursday, June 28, 2012

Goodbye Preschool.

Sam had his last day of "pre-school" today so we had to take a photo.  It's really a therapy group that helps him interact with other children and motivate him to eat.  He's definitely a social eater and the program has helped him a lot.  We're sad to say that the program has been discontinued.  (btw, Sam will cry if I say this out loud but that dog is not ours.  We're dog sitting for nine days)
Here are Sam's amazing teachers, Catherine and Amanda.  They were so sweet to him and made him feel comfortable. I was really worried the first time I dropped him off but he didn't even cry... I might have.  He absolutely loved attending class and I'm sure he'll miss seeing them twice a week.  Sam's speech and eating habits definitely improved when he started going.
Every kid got a bag with stickers and a pinwheel.  Now begins the search for a new and affordable preschool.  I'm about to recruit the neighborhood kids to come to my house for free school/art/snack/whatever group so Sam can still have plenty of interactions with other kiddos.

Sunday, June 24, 2012

Meet the Babies

There is a complicated and possibly arbitrary algorithm known only to Sam by which he chooses his most trusted companions.  These friends become close members of an elite entourage that have to accompany Sam just about everywhere he goes.  You never know who is going to make the cut, but they all have one thing in common.  They are all beanie babies, which is appropriate, since Sam refers to this bunch as "My Babies."

Meet Annie, the leader of "The Babies."  Annie was formerly known as "Baby Dog," and she has been around longer than all the others.  She joined Sam's side on a trip to Hurricane in the summer of 2011.  Nick Worwood generously gifted Annie to Samuel, even though the dog didn't belong to him.  Annie was Jared Worwood's property, but after some wise advice from Dr. Danny, Jared gave the transaction his blessing.  I thought it was a very nice gesture by Nick, but I didn't fathom how attached Sam would become to this spotted puppy.  She has survived many episodes of vomiting, many rides through the washing machine, and at least 10 airplane rides.

Baby Elephant (pronounced Baby Dornit) was the next baby to join Sam's club of friends.  He came from the Bountiful collection, around the time Sam was still obsessed with elephants, making elephant sounds, and watching "Born to Be Wild" at the Clark Planetarium.  Sam often shows Baby Elephant how to play soccer and march.  He has joined Annie on every trip to Stanford so far.
Bagheera, more commonly referred to as "Gheera" was the third member to be inducted into "The Babies."  This sleek black panther seems to drop in and out, much like his wild relatives.  Sam is often content with the original duo Annie and Baby Elephant, but when he needs strength in numbers, Bagheera is an able and willing body, and a good friend to have around.

Pascal joined the crew fairly recently, during one of Sam's "Tangled" binges.  Pascal accompanies Sam when he has assumed his Eugene alter-ego, and Sam frequently deploys him to sit on Ashton's shoulder, transforming her into "Princess Tangled."  Pascal is a much sweeter friend than his namesake, and is often requested at nap time.
Bobby is the newest member of "The Babies."  He was a gift from a nurse at Stanford.  Sam had to go without Henry last time, and although he was brave, the blood draw and urine bag were very distressing to him.  Bobby rushed to Sam's side when he was in his time of need, earning him a privileged spot in Sam's inner circle.  Sam named him Bobby after I suggested several options, all of which Sam shot down.  The last name I suggested was Bobby, to which Sam scowled and flatly said, "No."  "Then what's his name?" I asked.  "Bobby," Sam replied.  And it stuck.

Wednesday, June 20, 2012

Frog Syringes

We use a variety of syringes to give Sam his medicines every day.   He's almost always good about letting us give him his meds but just for fun we glued 20 cent froggies to the tops of his syringes.  He's really good about helping push the liquids through his g-tube medicine port.  The frogs are just another way to make medicine less of a chore every day.

Monday, June 18, 2012

three flights in three days with an almost-three-year-old: RP103 update

We went on our fourth trip to Stanford last week.  Ashton wasn't able to go because she started having pre-term labor contractions the day before (she was 32 weeks pregnant).  She spent most of that day in the hospital while they monitored her progress.  She didn't progress into full labor, so they sent her home and recommended she not go to California.  So I got to take Sam all by myself.  I'd never spent so much one on one time with him, so it was a really fun and special trip.  Ashton had never spent so much time away from him, so we had to google video chat whenever we could so she could get her Sam fix.

We arrived in Palo Alto on Sunday, June 10.  Sam is a champion flyer -- he's done it so many times now.  He always konks out right after takeoff and usually wakes up right before we start our descent.  We got settled in at our hotel and Sam got pretty hyper.  He tripped over a cord and nailed his forehead on the desk in our room.  He got a big old goose egg, which he started trying to "get off" with his hand.

We spent all morning Monday at Stanford.  We rolled in to the clinic at 8:00 a.m., just in time to give him his medicine.  It was just my luck that his g-tube button clogged, so I had to take off the tubing and use a little ramrod and slip tip syringe to open it back up, all with Sam flailing on my lap.  He HATES it when we mess with his button. Suvarna, the study coordinator, walked in the clinic lobby during this little episode.

After we got his medicine in the nurses started getting his vitals.  I didn't know how he would do without Henry there giving him moral support, but he did great!  He let them take his blood pressure, kept the thermometer in his mouth and hopped on the scale like a boss.  Next it was time for the blood draw.  He always fights it, and cries a little, but once the needle is in he usually calms down.  I think it's the anticipation that kills him.  He did pretty well this round and filled up all the tubes pretty fast.  After that we had to put the urine collection bag on him.  He hates this part most of all, even more than the blood draw.  He always fights and kicks and screams, but we were finally able to get it on.  It must be pretty uncomfortable to wear because he always starts walking like a grandpa penguin once it's on.  With the bag on we were ready to walk over to the children's hospital to get the required ECG.  Sam held Suvarna's hand the whole way.

As soon as we got in the ECG room Sam started fussing, but I just took his shirt off and threw him on the table.  This is the part where we really need Henry's help.  I got him to lie down somehow, and then he lay perfectly still while the tech put 13 stickers on his chest and abdomen.  He barely moved at all and we got a good ECG read on the very first try.  That was a relief!

Next I had to get the pee-bag off.  They gave me some adhesive remover wipes, which helped, but he still screamed the whole time.  Once that was off the worst was definitely over.  Then we just had to wait to meet with Dr. Grimm.

Dr. Grimm is the bomb.  He was on service at the hospital that week, so he was very very busy.  But he took time off to come over and meet with us.  He never acts rushed and he always takes plenty of time to answer your questions.  I had a lot of questions this time:

Question 1: At what point do we need to raise the dose on his RP103?  His last levels from Stanford came back really high, which was extremely stressful for us.  Have we been undertreating him for the last 3 months?

He said that everybody's level fluctuate, and that you have to get high levels on two consecutive visits in order to change his medication dosage.  He noted that Sam's cystine levels had been very low when we rechecked shortly after, which indicates that the levels definitely fluctuate.   He felt that most of the time Sam's levels are probably just about right.  

Question 2: How do we monitor his kidney function, since he isn't in kidney failure?  Cystinosis causes Fanconi syndrome, so how do we measure if that is progressing?  Sam didn't used to have problems with acidosis, but now he has to take Poly Citra K to keep his blood pH neutral.  Is this a sign that his Fanconi syndrome is getting worse?

He said there are three main ways we monitor the Fanconi syndrome.  At this age, we look at his phosphate levels and we look at his growth.  If his phosphate levels are adequate, then his proximal tubule is currently reabsorbing enough.  If he is growing, then he is also reabsorbing enough, and it's unlikely that the Fanconi's is getting worse.  

The third way they monitor progression of Fanconi's is to measure protein in the urine.  He said we would start doing this when Sam is a little older, like 5 or 6.  Because Sam has a finite number of nephrons, and many of them were damaged prior to his treatment, as he gets older those nephrons have to do more work.  Eventually this can lead to hyperfiltration damage, which results in protein leakage.

Dr. Grimm felt it was unlikely that Sam's disease has progressed because his phosphate levels look good and he is growing.  He said that the need to start Poly Citra K was probably more reflective of him getting bigger and his nephrons have to work harder to reabsorb more bicarbonate.     

Question 3: What is the best way, in his opinion, to test a newborn for cystinosis?  Do we just measure the cystine levels, like we did with Sam, or is it worth it to do genetic testing as well?

This is something we've asked just about everybody, so it's always nice to get another expert opinion.  He felt that the routine cystine analyte test was the most reliable way, and testing cord blood or infant blood should be just fine.  This is what our nephrologist, Dr. Nelson, thinks as well.  He didn't know much about the genetic test, but he said if it was cheap it wouldn't be a bad idea to do it, just as a confirmatory test. 

Question 4: When is the best time to measure electrolyte levels?

Dr. Grimm said the most important electrolyte you measure is bicarbonate, but that you don't need to get super serious about it unless the child isn't growing.  He said the best time to measure bicarbonate is right before his next dose, when his blood levels are theoretically the lowest.  This way you see the worst levels.  

Question 5: What is your opinion on growth hormone shots?

Many children Sam's age are already on growth hormone shots, so we've looked into getting that going.  Dr. Grimm said Sam appears to be doing very well on growth and that we could wait.  He said we should help Sam have the most normal experience possible, and adding daily shots probably isn't the best thing unless absolutely necessary. 

So we had a great visit at Stanford.  Then we packed up went to the airport and flew to Newport Beach to spend one day with family while they were vacationing there.   Sam was so excited to see his cousins, especially Abby.  He chanted her name for the rest of the night.

On Tuesday we hit the beach and let Sam play in the sand.  He splashed in the water a bit too, but one wave ruined everything when he got saltwater in the face.  He just doesn't handle oral surprises very well.  He started coughing and gagging and threw up a little in the sand.  Then he wanted to take all his clothes off and run around in his diaper.  We all decided we'd had enough beach and headed back to the resort to swim in the pool.  Sam had a blast in the inflatable rocket ship Lauren bought him.  He made me push him all over the pool so he could throw a sponge ball and Seth's fiance Hilary.  Good times.

Sam was very sad to say goodbye, but we had to hit the airport Tuesday afternoon.  He cried Abby's name all the way through security and all the way to the gate.  He perked up when he saw our airplane through the window.  We were some of the last to enter the plane because I had to stop and give Sam a feed.  As we walked down the aisle the male flight attendant asked where our seats were.  I told him row 22 and he picked Sam up and carried him to the back of the plane.  Sam was pretty stunned and all the people at the back of the plane got a kick out of it.  I found a spot for our luggage and joined Sam.  He was out cold in minutes, and he slept all the way back to Utah.  He didn't wake up till I got up to exit the plane.

Three flights in three days with an almost three-year-old.  I had to change all the diapers, give all the meds and all the feeds.  I didn't sleep much, and when we got home I was pretty exhausted.  It was a fun male-bonding adventure though, and it definitely helped me appreciate everything Ashton does for Sam everyday.  She is a supermom.

Saturday, June 16, 2012

Friday, June 15, 2012

It's All Fun And Games Until Someone Breaks A Window

Our day started off great! Sam had a little swim party with friends in our front yard.  Yay!  Wahoo!  Fun!    Somewhere in there he found the garden shears in our shed.  Scary, but no one was hurt.  A little later he shattered a garden pot.  That's okay.  It was only 50 cents.  After that he decided to play drums on our living room window, which ended in disaster.  Go to your room, Sam.

These pictures don't do it justice.  I've heard that little boys break windows and get into other sorts of shenanigans but I think we may be starting off a little bit early.  Here's to a glorious future with two little boys!

He feels really bad about it.  Sort of.  He offered to use the money in his "chicken bank" to buy a new window.

Wednesday, June 13, 2012


Cottage cheese, peas, pretzels, and gold fish are Sam's staple licking foods


 I always know when Sam is going to eat food because he asks for it and then tells me to go get my camera so I can photograph him eating.   He is now putting spoonfuls of cottage cheese in his mouth and sometimes he washes a few curds down with water.  He's doing so well.

Friday, June 8, 2012

Garbage Town


We started out with A LOT of garbage on our kitchen counter.  Sam chose the boxes he wanted to use for buildings in his neighborhood.

I cut out a variety of shapes in different sizes and colors so this project could be a learning project as well.

I made the houses and he glued them to the paper and then added squares, rectangles, circles, etc... to make doors and windows. 

He made a pond with "green stuff in it" and "cat tails"

Sam says the macaroni and cheese box is Mormor's house and "Chelsea is in the garage"