Jenna has the stomach flu...

After a great weekend at the Natalie's Wish event in Orange County we flew to Palo Alto for our final week of the drug trial; and wouldn't you know our Jenna got the stomach flu. It hit her hard, quickly, and when we went in for the routine labs required for the drug trial, the doctor recommended a trip to the ER for the day. There she could receive fluids and the potassium so important to keep her on track. We were disappointed that after this whole day in the ER, her blood chemistry levels have declined further (even though the tough little bird looked quite good to me) in spite of the IV fluids. She is being moved from the ER at Stanford Children's to the Pediatric Unit, where she will spend the night with Kevin by her side. Patrick and I returned to the hotel in a cab and he is already asleep. The sight of the ER and the sight of his sister IN it had him pretty shaken. I myself am so tired and worried and weary of Cystinosis I'm kind of in the clouds...
Thanks for keeping our Jenna in your thoughts. She didn't shed a tear the whole day in the ER...just watched the IV being put in, watched a movie, took a nap and chatted with nurses as they tended to her. : )

Teresa

Saturday at Home

It is a wonderful day.  The wind is blowing and it's not much like Spring, but the kids and Kevin and Tucker and I are all HOME and I revel in each little thing we do that is part of our weekend routine here.

Last night was a significant one because Jenna, who is on the 12 hour medication, slept from 9pm until 8am this morning!  This was the first night in her life that her sleep was absolutely uninterrupted by water refills or medications.  While I suspect this won't happen all the time, it is really great to know that it's possible! 

We leave for our 6th Natalie's Wish event in Newport Beach on Thursday.  On Sunday we fly directly from there to San Jose for our final week of the trial at Stanford.  One more long week and we're done! 

I will continute to keep you posted.

Love, T.

We made it!

In spite of all my fear and loathing; yesterday was not so bad.  Both kids had a good cry when their lines were put in...but after that hard part was done the rest of the 12 hour day was just fine.  Patrick watched the clock very carefully all day--keeping the nurses on course for his hourly blood draws.

Now the switch.  Jenna took her first dose of the new medication last night and will continue on it unitl the end of the study.  Patrick reverts back to the 6 hour medication schedule until the end.  The final day of testing, May 25, will be like yesterday--a full 12 hours of constant bloodwork. At that point we'll enroll the kids into the long term study and they'll both get the 12 hour medication for at least 2 years or until FDA approval.  Kev and I look forward to May 26--that first day when both kids will be on the new blue pills!

I'll admit I'm a little weary to write much....here are some videos and  images worth a thousand (or more!) words....click on the arrow to view youtube videos....

Prior to our departure last week we got to visit Steve Chamberlain at his Horse Ranch.  The kids rode a  pony and got to feed a baby goat...Thank you, Steve, for the really fun day. 

After the drive here and the first day at the clinic this week; I really loved this moment.....



 Coyote Point Park was spectacular! 

Aunt Nancy spent two nights this week and kept us all sane!

Multi tasks watching a video and preparing pizza Lunchables.

Jenna on her perch at the clinic yesterday...

We love Nurse Cindy. The kids picked out little cakes at a wonderful specialty market at Stanford.

 Jenna wanted Cindy's name written on hers!

Cystagon and a Wedding at 3am

I have been remiss in letting our followers know how Patrick is doing on the trial medication! I am pleased to say he is tolerating it very well, and aside from the fact that he has to take 7 rather big pills for each dose, and the fact that he has to take them with orange juice (he's not a fan); it's all fine! He is still waking in the night with a wet bed and a wet pull up-but I've heard with time it may become a bit less of a problem. It's nice that he's waking on his own terms, instead of us shaking him to wake for medicine. He still throws up, but it's not a sick tummy heart wrenching kind of vomiting (maybe we've become too used to it?). He simply coughs, gags a bit, throws up and then goes about his day. I hear that may go away with time, too.


We have had a nice respite from days at the clinic, celebrating Easter with family in Idaho and enjoying a quiet Spring Break at home in Sacramento. We will have a couple of families over to barbecue tonight, the kids and I have had fun working in the yard and cooking in preparation.


A story. Jenna and Patrick are at the funny age when they become aware of romance and act silly with their friends saying "I'm so in love with (insert anything) I want to MARRY it". They have not been to a wedding, and have just recently begun to understand that it would be very uncool if they one day married each other. Last week we had Patrick's favorite dinner, spinach and cheese ravioli; and while savoring the fare he said, "I love this spinach ravioli so much I want to MARRY it!". I tell this story for a reason.


While Patrick enjoys the full nights rest that comes with the time release medication, Jenna continues to take the "old drug" every 6 hours around the clock. Last night I visited her room at 3am and jostled her from her sleep and gave her Cystagon. She usually wakes MUCH easier than Patrick (Kevin says she's like a cockroach in the sunlight...all scattered and crazy when we wake her); but last night she was really sleepy. She was sopping wet from knees to neck as I scooped her up and gave her the meds; her head bobbing about while she fought me. I whispered "Jenna, right now there is a girl named Kate getting married and she is becoming a princess for real! Do you want to go watch the wedding on TV?". Her eyes flew open and she peeled off her wet pull up, and ran her naked self into the living room. We wrapped up on the couch and with great excitement and curiosity Jenna watched the first wedding of her life. She was riveted, and like the rest of the world marveled at Princess Kate and her dress, her veil, her hair, her tiara, her proud father and her Princely groom...all of it. We agreed that Kate is a very special Princess.


As Jenna walked back to her room to go back to bed she looked at me and said: "Wow, that Princess Kate is sure beautiful; I think Patrick is going to want to marry her instead of that spinach ravioli."


Last night it was a great blessing to give Jenna her meds at 3am. To share a Royal Wedding, live, with my six-year-old daughter was pretty special.


Love, T.



Thanks for coming to spend time with us Aunt Nancy!





Aunt Nancy always makes us laugh!





Sprinkles Cupcake Shoppe after an arm pinch!



The trip to Claire's Boutique with little pals Mary Margaret and Cate

Patrick chills outside a shoppe in Boise, ID--give you one guess what Kevin was doing....



Jenna, Patrick and Grandma Jan watch a movie



The impressive YMCA in Caldwell, ID.  The kids loved the H2O Slide!







Monday the 18th

Bribery seems to be the ticket.  For Patrick it's money or food (he made $1 today); for Jenna, a trip to Claire's Boutique at the end of the week to shop for some bling is getting her through it.  I am becoming completely at ease with this method, considering both kids did really well for their bloodwork today.  We have had a relaxing day around our "home away from home" ie: Marriott's Residence Inn.  Kids have been hot tubbing, out to lunch, a little school work and a nap for all of us!  Kevin left for Sacramento today so the kids and I are on our own tonight and tomorrow morning until Aunt Nancy arrives to help out until we depart on Wednesday.

Patrick has been taking the delayed release medication for 4 nights now and has slept through the night each time!  He wakes up soaked, but who cares?  I am thrilled for him and for us!

We had a wonderful day yesterday--boating in the San Francisco Bay with our hosts the Diepenbrocks.  Many thanks to Mike and Jim for the indulgent day!  Check out our photo journal to see fun pictures of the outing!

Love to all of you who are checking in and saying prayers!

Teresa

Long Day

Today there were 3 other children at the clinic who have Cystinosis, two of them teenaged boys and a bit later our friend Tina Flershinger from Clarkston, Washington showed up for her visit.  The boys were VERY occupied with their electronic games; we got nary a glance from them; save a big grin for these pictures.  Joey's mom Mary and Bailey's Grandpa Tim were fun for Kev and me to visit with as we wandered from clinic to main hospital and to one more location for our appointments with Dr. Grimm.  He is the head Pediatric Nephrologist here at Stanford and took a long time with us, giving us a thoughtful response to our myriad questions.  Jenna and I joined Tina and her father Mark at "The Fish Market" for lunch and then to Great America for some roller coaster rides with Patrick. (Jenna decided the mall could wait while she enjoyed an afternoon with Tina.)

I have a headache.  Talking about Cystinosis all day with families and doctors is educational, helping us understand the nuances of the kids' mysterious disease; but it's a drain.  I know it's a drain on the kids too...they show it in funny ways; Jenna especially is needy and a little more vulnerable than usual.  She had a terrible time with the blood draw again today.  They didn't have to poke her more than once this time, but it took about an hour to calm her to the point that a blood draw was possible.  She actually hid in a cabinet in the clinic room and shut the door behind her, hiding from us and the nurses.  When it was all said and done we got a laugh out of that.

We will stay in Palo Alto this weekend as we have to be here Monday, Tuesday and Wednesday for clinic visits. The next couple days will be fun family days. We just gave Patrick his first dose of the RP103 medication...a big deal! Even more fun is that we only have to wake one of the kids at 3am tonight. Patrick can sleep and sleep--as long as he likes! His hotel room, however, may be a lake in the morning!


Here's the day's photo gallery:


A harpist heads to a concert at the Stanford Children's Hospital Atrium and gives J & P a try.

 


It was wonderful seeing J & P playing with Tina today.  We are excited to see her at Natalie's Wish!

Joey, Bailey, Patrick, Tina and Jenna....could all be related!

 


 

 


 

 

"Randomization" Day

We are in Palo Alto and will head to the clinic this morning for a pretty long visit.  The kids were officially accepted into the study and the actual drug research part of it begins today. Of course the purpose of the trial is to test the efficacy of the "RP103 Delayed Release Cystagon"; so the patients spend half of the study on their existing drug and half the study on the RP103.  Wouldn't you know that the computer randomized Jenna and Patrick's participation to opposite schedules?  Would've been a treat to have them on the same protocol the whole time---but this will keep our minds sharp. 

Patrick will take his first dose of the 12 hour medication today and will get to experience it for the next 3 weeks!  Wonderful! Jenna will remain on the 6 hour schedule and they will switch in 3 weeks.  At the end of all this, of course, we will take home the 12 hour meds for the long term. 

Our boy is absolutely obsessed with roller coasters.  He knows how to go on to You Tube and watch the videos that people have taken from the front seat of roller coasters around the world.  Today he'll get to experience one for real as his Dad will take him to Great America Theme Park after the clinic visit.  Jenna thought it would be more fun to do a "girly thing" with mommy, so we will stick close to Stanford Mall.  I am thrilled with this scenario--I don't care for theme parks; but quite enjoy a Mall!

Here's a link to one of Patrick's Favorites:
 http://www.youtube.com/watch?v=0RPcNwlcAoI&feature=relmfu

Fun to have Kevin here.  Thanks to Heather for the fun gift bags that kept the kids busy and happy on our way here; and thank you to Aunt Nancy for coming to take over at the beginning of the week when Kev leaves!

Stanford Trial Travel Schedule

A few family and friends who are interested in visiting have requested our schedule...I thought I'd note it here.

April
Thurs 14-Wed 20

May
Sunday 1-Wed 11th (we may come home for Friday and Saturday night--TBD)
Sunday 22-Friday 27th

May 5 and May 26 are the longest days and will require the greatest amount of effort for all involved.  Bloodwork will be required every hour from 9am to 9pm those days...so we'll need to stick close to the clinic, and will have to be rather quiet as the kids will have an "IV Saline Lock" in their blood draw site. (Yuck)

Home!

Yesterday's appointment was much better.  Jenna, instead of being terrified to go back, worked hard to prepare herself for a successful experience.  She woke and drank a lot of water without even being reminded, ate a big breakfast and had a great attitude.  I heard her chanting to herself in the bathroom: "I know I can do it, I know I can do it, I know I can do it...".  No tears yesterday--just celebrations of success.  After their appointments we found a pretty place on the Stanford campus to get their bikes out of the car. 

We still have to wait until mid week next week to be certain the kids can be in the study.  The purpose of the last three days was to get consistent blood work that will be evaluated to determine their average cystine levels.  They must have levels below 1.0 to participate.  Since they began taking Cystagon in 2006 they have never had levels above 1.0 (Jenna's was 2.5 at diagnosis, Patrick's 3.8).  The blood samples take a lot of time to prepare and evaluate after they are mailed to Scripps...so we will wait to hear.

Jenna and Patrick are happy to be back at school.  I am thrilled to be home! 

Hard Day

We slept in a bit more, got ready a bit later and didn't take as much time for food and water; and it was a rough one.  No blood was acquired from Jenna, as two attempts to draw, by two different nurses, resulted in nothing but a meltdown by our little lady.   The poor girl is so darn brave (hasn't cried at a blood draw for over two years), and she was caught off guard today for one reason or another.  I blame it on a lack of water this morning, and will get her up bright and early tomorrow to get her on the right track.  I pray all goes well tomorrow; that she doesn't hurt, and that she is able to overcome the trauma of today.  Our new friend (and trial coordinator) Xiao xiao, was very supportive and kind today--and made the decision to forego the bloodwork after two failed attempts; claiming that patient comfort is the most important thing of all.  He didn't like to see her upset; nor did Patrick, who fights with his sister constantly, but asked to leave the room and became rather frightened when she started to cry.  I am sure my headache right now is related to the stress of the morning.  We all react...just in different ways.

Jenna, Patrick and I bellied up to the counter at a place called "Slider Cafe" after their appointment and had breakfast sandwiches.  Our wonderful server (Mieka was her name), created a beautiful plate of strawberries and whipped cream for Jenna, who loved it enough to order seconds.  It was nice for Jenna to be so indulged after her rough go this morning. We also met Bob, a young MBA graduate who sold us his 2nd generation iPad as we ate breakfast...it was a wonderful adventure!

We planned to go to the beach and introduce Tucker to sand and surf; but I hit the wall.  We had a quiet afternoon, the kids did schoolwork with Grandma, and Jenna and I had an outing to the dollar store.  She spent $4 on a picture frame and lipstick for Grandma, crazy straws for Patrick, and a chew toy for Tucker.  Jenna was emotional all day today, and we were ALL exhausted.  Retail therapy did Jenna and me wonders--it was well worth the four dollars!

The kids and I miss Kevin and look forward to getting home tomorrow.  I realize after our first extended visit here that this will surely be a challenging couple of months; but will be well worth it in the end if our kiddos may feel better!

Love, T

Feeling Settled

 While we still have to wait until Wednesday of this week to know if the kids are accepted into the study, I did learn from Xiao xiao that the powers that be at the Pharma company have made an exception to the weight requirement and Jenna will be allowed to participate if all other benchmarks are met!  This is great news!

We are at a Residence Inn in Newark, because every hotel in Palo Alto is filled with fans and teams at the NCAA Women's BBall Finals.  It's a quick jaunt over Dumbarton Bridge (the kids still get a kick out of it); and with traffic it takes 30 minutes to get to the clinic. We got there on time today (we were lost and late and flustered last time) and we were out of there in an hour!  The visits this week are quick ones; with blood draws and a check of vitals and off we go.  Both kids were ultra brave today for their "arm pinches"...not a tear to be seen.  Patrick hid himself under the chairs in the waiting room of the clinic; making me wonder if I'd have to create a scene prying him out.  The promise of a trip to McDonalds after his blood work was enough to set him free.

Poolside with Grandma Juanita and Tucker-the-Dog

Grandma Juanita is here with us and she is so helpful.  She stayed at the hotel this morning with Tucker (we brought the dog with us to this pet-friendly hotel), and she finished all the laundry before we made it back from our appointment!  Before heading to the pool for fun, Grandma spent time with each of her grandchildren (separately) doing homework.  I must admit that it is very convenient, when the kids are going to miss 22 days of school, to have a woman who taught Kindergarten for 30 years tutoring them!  She is so patient and creative with Jenna and Patrick!  I am so grateful for her help.



Brave days call for big treats. 
Lunchables?  They were over the moon!



We had a picnic by the pool today for lunch. I typically don't go for Oscar Mayer Lunchables, but as you can see today I made an exception.  Late this afternoon we headed into beautiful Palo Alto to walk along University Avenue and peek into a few shops before dinner.  We discovered a quiet, expansive Business Park on the way home where the kids were able to ride their bikes and blow off some steam before bed.  Tucker got to play fetch, too--so all creatures were happy.

Thanks to family and friends for checking in and for the well wishes.  We are all getting the hang of it!

Love TJ

Day 1

We are home after visit #1.  It is one of 6 that we'll take between now and May 27th, assuming the kids are accepted into the study.  The car travel time is the hardest part.  The kids are restless and they argue, they are thirsty, have to pee, have to poop, are hungry, "can I hear a different song"?  This is not unique to our children, of course; but it makes it no less annoying.  I have to practice breathing peacefully.  I need to start yoga classes again.

It takes 10 minutes from hotel (where we all slept like babies) to clinic.  There is currently a building under construction for Stanford's "Clinical and Translational Research" department.  Until then studies such as the "RP103 Delayed Release Cystagon Trial" take place in a rather small medical office building about 100 yards from Lucille Packard Children's Hospital.  It is a beautiful campus and the kids will have fun riding bikes around it on future visits. We walked from clinic to the main children's hospital during our visit so the kids could have EKG's done.  The EKG, as you might imagine with all those wires and what-not; was a very scary thing that ended up being quite fun...mostly it tickled. 

At tuck-in tonight I learned that Patrick's top three experiences of the trip were: 1.  The pool and hot tub (and bathtub!) at the hotel. 2.  Our lunch at California Pizza Kitchen today. 3.  The EKG! 

Jenna's were 1.)  Meeting Xiao Xiao Gao, (pronounced "shao shao gao", he is the drug study coordinator; an efficient and very accommodating young host who aspires to be a surgeon).  2.) The hotel pool and hot tub. 3.)  The DUMBarton Bridge.  Seriously.  Yesterday, upon entering our hotel room, she asked: "well where is my little welcome basket?".



Jenna weighed in at 41.2 lbs.  Xiao Xiao can't tell us much about our chances; but it seems likely that both kids will be accepted.  The data from today's and next week's visit will be given to the Pharma. company, who will make the decision based on their own and the FDA's guidelines.  Dr. Minnie Sarwal, the pediatric nephrologist who saw the kids today is going to recommend that Jenna is a good candidate for the study in her professional opinion   I will be able to share the official answer next Wednesday afternoon.

The bummers were a change in medication schedule to 9am/3pm/9pm/3am which will be hard to get used to and will have us waking the kids up twice a night.  And Dr. Sarwal was concerned about the kids' cholesterol levels and that they are on statins at such a young age.  She is ordering special blood work for next week; to evaluate further. These are good things to learn as we go.  To see some of the best doctors in the field for so many visits will surely be enlightening.

Love, TJ

Homework on the way to Stanford lasted about 10 minutes.

The kids with Xiao Xiao and Daddy

They made a little pal while waiting at the Cardiology Clinic

Jenna's EKG (required for ANY type of drug study)

He was terrified, but it wasn't so bad!

Lunch. The binoculars were a big part of the trip. Jenna wore them everywhere; like a little birdwatcher.

We had a little lemonade stand in the sunshine when we got home!

 

 

 

 

 

 

 

Drug Study Trip #1

We arrived in Palo Alto this afternoon and are settled into our hotel.  The kids are sound asleep at 7:30pm after a hearty dinner at Max's Opera Cafe at the (really fun) Stanford Shopping Center.

The drive took 2.25 hours and was relatively easy; but only because I had Kevin's help.  We had to stop 2 times for bathroom and food breaks.  My friend Sydney gave us wonderful directions through Stockton, Pleasanton and over the Dumbarton Bridge (the kids loved that name--repeated it 20 times each).  The weather was cloudy all day, clearing just before sunset.  It is supposed to be sunny tomorrow and we are all ready for that! 

We are staying at the Marriott Courtyard Hotel in Los Altos.  We are about 10 minutes from the clinic.  We are to arrive there at  8:30 am tomorrow and I have no idea what the day will be like.  How long?  What will we do?  Who will we see?  Will the kids have bloodwork tomorrow?  Will they give us a better idea if Jenna has any chance at all, considering she is still a slight 42 lbs.?

After tomorrow's appointment we will head back home for a normal school week.  Grandma Juanita arrives to Sacramento on Saturday and will travel back here with us next Sunday for our second, and much longer, visit.  We will be here through Wednesday of next week.  At the end of that visit the "screening period" will be over and we will know for certain if Jenna and Patrick have been accepted into the study.

I am grateful that Kev is here with me for this first trip.  There are so many logistics to learn.  Once I have the lay of the land I will feel better about coming back.  We have two rooms that adjoin and the hotel has a sundry shop, laundry room, pool, hot tub and bar.  What more could one need...besides maybe a kitchenette?  We're going to be dining out an awful lot. 

Tucker the dog is having a sleepover at our (dear) neighbor's house.  Here's hoping he's a good boy.  Jenna had a really tough time leaving her dog.  I wish he could come on these visits with us; but the hotel won't have animals...

I will post tomorrow after our inital visit.

Love, Teresa