Monday, May 23, 2011

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, May 14, 2011

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.

Friday, May 6, 2011

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!


Friday, April 29, 2011

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, April 18, 2011

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

Friday, April 15, 2011

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!