IV Dose #2...

Tuesday, September29, 2009

Torie, Mom and I headed over to Denver on Monday and stayed with my Aunt Collette and Uncle Dick. Well, with Dick. Collette again said "Oh yes, I'd love to have you stay" so she went out of town again! Huh. Maybe it's me. Anyway, it was a beautiful drive over...tons of yellows, golds, greens, and oranges. I think sometimes my travels have caused me to forget what a great state we live in. I'll have plenty of opportunity to admire it in the upcoming weeks, though. This was our 1st infusion at The Children's Hospital (TCH), and we'll continue weekly until Minnesota is ready for Torie to start stem cell transplant. Hopefully mid-November. They are working on a donor match as I post. Anyway, we arrived at TCH bright and early Tuesday morning. We had just barely gotten to our room in the infusion clinic when Uncle Shaun showed up! And he stayed all day with us. What a great guy. He hates hospitals. We're pretty lucky to have him. Kristin was our nurse du jour. She came in and Torie stuck her leg out and said "hug leg" for the blood pressure cuff. What a great little patient already! But it never takes long with kids. She had no problems with her infusion, and no reaction. Dr. Thomas stopped in to say hi and it was nice to visit with her. Too bad we didn't get to see Janell, the Metabolic Genetic Counselor. She's done so much for us, she seems like an old friend already. Riley, another little Hurler dolly was there the day before. Maybe one of these we'll get to meet her too. So, we finished up about 4:00 and headed back to Collette's and guess what? She came home! While we were there! I made my Grandma's secret recipe fried chicken and biscuits and had a fun relaxing evening. We were back on the road this morning to come home so I can get some laundry done and get ready to head back next week.

We've been having some problems with Torie's Hickman catheter (Her central IV line). The last few days she's had quite a bit of blood oozing from the insertion site. OptionCare (the home supply company) sent 100 unit Heparin to flush her line with. Heparin is a blood thinner that prevents clots from building up in her line. If it clots off it won't be able to be used, and she would have to have a new one placed. And we don't want that. So, after talking to Dr. Mike, we're going to try 10 unit Heparin and see if that helps.
She's been so good about not pulling or tugging on her line or the dressing, but here she's showing her line to Uncle Shaun. She's such a brave sweet tough trooper. We are so lucky to have her and I hope with these posts you can feel like you know her a little too. Thanks for reading and stay tuned. All we hope for is lots more to come!

The most wonderful time of the year...

...Fruita Fall Fest! You were probably thinking the title had something to do with my last post, but no! Fall Fest is the end of of September every year. Rides, games, shopping, and FOOD! It's the only time of the year that one can find Greek food in Fruita. Yum. Last year Torie was too little to go on rides, but not this year. After our week in Disneyland she's an old pro. The first thing she said was "Mo rides!" and was ready to go. She rode a giant chicken (for Aunt Lettie who finds Mike the Headless Chicken hilarious) with Mama-great, a strawberry with me, the slide with Stacey, but best of all,

was driving a car all by herself!
Stacey, being the faithful Mesa State alum that she is, supported the corn stand. Torie, being the faithful corn-eater that she is, did too. Those girls can chow!We all had a great time and can't wait till next year. If you've never been, definitely worth checking out.

Tomorrow Torie, Mama-great, and I head to Denver to The Children's Hospital for IV ERT. We're hoping she does as well with dose #2 as she did with the first one. Her Hickman catheter - an implanted infusion device - works great. It's like a big IV that goes into her chest and ends in the right atrium. These are great for kids...no pokes! Blood can be drawn through it and when it comes time for her stem cell transplant, it will be given through it as well. Lexie is doing a great job flushing it (to prevent blood clots from forming) and Torie doesn't seem to mind it. She doesn't pull on it or mess with it. Not crazy about dressing changes, but at least she loves "banny's" (Bandaids). What a good girl she is.

Thank you all for your comments on Torie's Story. Your well-wishes, kind words, prayers, and support are appreciated more than words can express. Please keep 'em up...we'll take all we can get!

Torie Snow and the Flakes

Sounds like they should record Christmas music, doesn't it? Uncle Eddy (Tom's older brother) and Aunt Lanny stopped to see us on their way from Wyoming to Arizona. We were very excited to see them since all the other Flakes stopped when Torie wasn't here. We had a great visit with them. The Flake's are all so wonderful to us...we sure wish they were closer!

Wednesday, September 23, 2009

Tom and I just got back from the Grand Mesa. We spent the last 2 nights in our RV with our very dear friends, the Mazon's. Fall has already hit and the colors were beautiful. It did get super cold, doncha know. Good practice for winter in Minnesota! Torie stayed home, but here are some pictures from last year....

Mesa Lakes

I forgot to post this picture from UMMC (University of Minnesota Medical Center). Day of discharge, on our way out of the hospital....

"Flowers for the cute little red-haired girl" How handy that we happen to have one of those!

September 20, 2009

Torie was very excited to be discharged Friday evening after her IV ERT was done. She tolerated it with no problems. Let's keep it that way! We packed up and left RMH early Saturday morning, caught our flight back to Denver, and headed home. Aunt Collette met us at the airport with my car and presents! Our thanks to "Lettie" and her friend Sheryl...so very kind to think of us.

We met so many wonderful people in Minnesota. Torie's "care team" Dr. Orchard, Teresa, Eileen, Dr. Kendra and Dr. Julie, Marie, Beth, Laura.....so many I can't name them all. The plan now is for Torie to continue IV ERT in Denver at The Children's Hospital until mid-November, then we will return to Minnesota to start stem cell transplant. Very scary, but absolutely the best place to be.

Through all of this I am so thankful for my family. They are the best people in the world. Tom is a wonderful husband...his willingness to support my various travels and holding down the fort while I'm gone. I'm so glad that he loves Torie the same as the other 4 grandkids. My parents are so smart, so strong, and so reassuring. Shaun is just like my dad...a rock. Tough enough to carry everyone else and still so caring. Collette and her family are there for us for anything we need in Denver. And the Flake side is supportive in their well-wishes and prayers - especially Torie's 2 awesome Aunties Heather and Erin. We have friends that we can lean on and for almost anything. I wish it hadn't taken this life-altering event for me to appreciate how many important people I have in my life. All my thanks to all of you.

A quick P.S. - Ronald McDonald house collects can tabs for a money-raiser. Please save your can tabs and I will take them to Minnesota when we go back in November. And don't forget to put your change in the RMH collection boxes at McDonald's if you can. Believe me, it goes to good use!

Home again home again jiga-a-jig!

September 19, 2009

Back in Fruita! Ronald McDonald House was great, but there's no place like home. I'm gonna try to give a quick recap of the rest of our week. The schedule I posted? We had 3 days that looked pretty much the same. Monday was kinda rough...figuring out the shuttle was easy compared to figuring out what all the initials on the schedule were and then trying to find them! I'm so thankful I wasn't on my own. It definitely took all 3 of us to navigate our way to different clinics and appointments. I would have been totally overwhelmed. Mom is the most supportive person in the world. Not to mention an excellent mediator for Lexie and I! By the time our days of appointments were done we were all ready to head back to RMH. Torie didn't get her usual naps, but she was a trooper! Kids are such better patients than adults. She'd get done with an exam and say "mo games?" Unfortunately, her great attitude makes me look really immature when I start whining. So I had to suffer in silence. Waaa! Anyway. There's so much to do at RMH that it was difficult to not make some time for playing...

I think Torie and Lexie enjoyed meeting Jerry more than anyone else. Jerry is the RMH therapy dog. He is a super sweet mellow Labradoodle with an incredibly high tolerance level! Kids hug, lay, pet, pat, pull, etc...he just lays there!

Wednesday evening we had wrapped up our appointments and had some extra time so we went out to Maggiano's (one of my top faves) for a celebration dinner. Super deliciously yummy! When we got back to RMH, it was sleep study night. Respiratory brought equipment and she was hooked up for the night.

She sure didn't like her "kitty whiskers" but she never tried to take the off...even in her sleep! That'll make a Grandma proud. She cruised through the night with no problems at all. I wish Mom and I had too, but I think between the two of us we checked her every hour. Yaaaawwwnn!

Thursday was OR day. By the time she left pre-op holding she was getting hungry and a little cranky, but a little bit of Versed changed that. She left Lexie singing "Yo ho yo ho (Pirates of the Caribbean) T-O-I-E!" We spent a very long day waiting for her - she had about 6 different procedures - but everybody was great keeping us updated. A bit of a scary time with the breathing tube, but all in all, everything went well. Intrathecal ERT at 12:58pm. I can't explain the feeling knowing that we are underway with treatment. Part relief, part hope, part faith, and perhaps a side of anxiety of the unknown. But just knowing that treatment has begun feels like we'll have her longer. She felt kinda puny after, but she got to her room on 5D (the Pediatric Bone Marrow Transplant - BMT -) and settled in pretty well. Lexie stayed the night, and Mom and I headed back to RMH. It was "Parents Night In", a special dinner that volunteers host about 3 times a year. Fabulous! Candles, flowers, steak, wine...Incredible. Those Minnesotans have huge hearts! Mom and I both bawled for a while...Again just relief I guess, but definitely tears of joy.

We enjoyed our dinner very much but when we got back to the hospital Torie was eating Cheerios and drinking apple juice. I think that was one of the best looking dinners we'd seen...just nice to see her getting back to her usual. She loves her groceries!

After getting settled at Ronald McDonald house (RMH), we were up bright and early Monday morning to start our week of appointments and evaluations for Torie to be part of the research here and for treatment. She saw "the clown" outside on the bench and said "Ronald McDonald Duck!". Funny baby! We caught the shuttle to the campus so Torie had a nice bus ride. This place is huge! But everyone is so helpful, we got great directions anytime we needed to go somewhere. So...here's our schedule:

Monday 8:00 Consultation w/Dr. Orchard, BMT Clinic, 5th floor PWB
9:30 Labs and Calendar Review, BMT Clinic
10:00 Surgery Consult, Peds Clinic, 4th floor PWB
10:30 Chest & Hip X-ray, Clinic 1D, 1st floor PWB
11:00 EKG, Clinic 1B, 1st floor PWB
12:00 Lunch
1:00 Anesthesia Consult w/Dr. Beebe, check into 3C and ask Dr. to
be paged, 3rd floor Hospital
2:00 Echocardiogram, Gold Room, 2nd floor Hospital
4:00 Cardiology Consult, Peds Clinic, 4th floor PWB

And that was just the first day! The rest of this last week was pretty much the same! Crazy busy. But we managed to get through the day but we were all glad to get back to RMH and go to bed. We have met lots of really nice families there, and 3 other kids with Hurler's Syndrome. It's so nice to talk to them and hear how these kiddos live and thrive with Hurler's.

Torie is tolerating her ERT beautifully! So we're hanging out waiting. I sure you're anxiously awaiting more of my drivel, so I thought I'd let you know about our week here....

Torie, Lexie, Mom and I left Junction Saturday evening, drove to Denver, and stayed at my Aunt Collette's house. Like any smart hostess, she left and spent the weekend in Ft. Collins with friends. But my Uncle Dick picked up the slack, made bagels and cream cheese for breakfast, and my cousin Fachon took us to the airport. We arrived in Minneapolis-St. Paul, got our rental car and found our way to Ronald McDonald House. What a great place! They provide linens, towels, cleaning supplies, everything. They also have volunteer groups that come in almost every night to cook dinner. And they clean-up! WOW!

September 18, 2009

We came back to Univ of Minnesota Children's Hospital to Unit 5D to find our spunky, active, crazy, back to normal baby! She's doing great! Right now we are waiting for her 1st dose of IV enzyme. Laronidase (Aldurazyme{TM}) belongs to a class of drugs called enzyme replacement therapies (ERT) that provides people with sufficient quantities of an important enzyme that they cannot create on their own. The main ingrediant in laronidase is a protein that is identical to a naturally occurring form of the human ezyme alpha-L-iduronidase. Laronidase replaces the missing enzyme and and restores sufficient enzyme activity to break down glycosaminoglycan (GAG) build-up. In a clinical study, laronidase ERT decreased abnormally high GAG substance in urine and decreased liver size, improved lung problems and corrected symptoms such as sleep apnea and airway problems. However, laronidase ERT alone alone has not shown to benefit the progressive neurocognitive decline or improve survival. This is the reason that Dr. Orchard and the University of Minnesota are doing research and clinical trials giving laronidase Intrethecally (IT). Laronidase given intraveneously (IV) does not cross the blood-brain barrier. By giving the enzyme IV and IT, neurocognitive abilty is being being preserved.

Wow. That was lengthy! But you know how windy I am! So IV ERT is in progress while I type. It feels like every drop that goes in is buying our girly just one more day with us.

September 17, 2009 - Late
Torie did great today. She's now in room at Univ of Minnesota Children's. She got her 1st dose of Intrathecal enzyme at 12:58. We're on our way. We feel so fortunate to be here. Minnesotans are super, doan cha know? Ronald McDonald house is an amazing place. I'll post more tomorrow..I've got a week to catch up on in one day! I'll do my best. All my thanks to CAE for her blogging guidance and support!

In the Beginning...

Okay, I'm gonna try to get this started. Be patient, and hopefully I'll improve with time. First off, let me tell you who's who in the story...I'm Cheryl, Torie's Grandma. And Lexie's mom. Right now I'm in the Surgery waiting room at the University of Minnesota Medical Center. My mom is here also, so we have been amazing everyone with 4 generations traipsing about. For anyone who isn't up on the timeline of events leading us to this waiting room, I'll try to give you a brief timeline. But, you know me, brief is so subjective!

March 2009

Bronchiolitis that just wouldn't go away. Grandma-Great (my mom) took her to Docs on Call, Chest X-ray showed some scoliosis (spine curvature) so we were referred to Dr. Deering, a pediatric orthopedic specialist in Grand Junction. She took more X-rays and found a "gibbus" which is an outward curvature or Kyphosis. She also found that Torie has bilateral absent acetabulum, or in other words, no hip joints. She felt it best that Torie wear a hip abductor brace, the "Cruiser". In Torie speak: a "woowoo". She also referred us to Dr. Manchester, a geneticist from the Children's Hospital in Denver who does a specialty clinic in Grand Junction every other month.

In the "woowoo" with Grandma-Great on Snowy Range

August 6, 2009

I went with Lexie and Torie to see Dr. Manchester, the geneticist from Denver. After answering numerous questions from Melissa, a genetics counselor, Dr. Manchester came to see Torie. After some looking and listening, he sat down and told us that he thought Torie has a lysosomal metabolic storage disorder, most likely Hurler's Syndrome, that she'll probably need a bone marrow transplant, not live past age 10 or so, and the universe went crash....

Mom called as we were walking out to the car. Lexie and I were both crying, so mom joined in. She wanted to be here with us, so she and dad left the cabin in Wyoming and headed back to Fruita. It was so nice to have them back. We didn't feel as alone and of course Torie was thrilled to see "mama and papa". (She calls Tom and I mama and papa too. Lexie is mommom and Scott is Daa-ie.)

August 17, 2009

Mom and Dad ended their summer in Wyoming early to spend more time with Torie. Lexie and I took Torie to the Children's Hospital in Denver to meet with Dr. Thomas and Janelle from the Metabolic Clinic. My brother Shaun went with us also. He is always so calm and rational, so he was a very reassuring presence to have with us. They were very certain of the Hurler's diagnosis but they drew blood work to confirm. They seemed to feel that the best place for treatment was the University of Minnesota. We had a lot to think about.

August 31, 2009

After lots of phone calls and dealings with lots of people, we found out that insurance may not cover to have Torie treated in Minnesota. Heart-breaking news since Minnesota is the only place in the world to do Intrathecal Enzyme replacement. Research is showing this is saving Hurler's kids from the neuro-degenerative effects that accompany this disease. In essence, it's saving their brains. Torie is so smart and bright and funny and observant that this was what we felt she needs. Like I said, heart-breaking.


September 2, 2009


We flew out of Grand Junction for a week in Disneyland for Lexie's birthday and for some fun before the unpleasantness of treatment begins. Shaun met us in Denver, a total surprise for Lexie, and went with us. Torie loves flying on planes "inna sky". We were exhausted when we got there but ready for big fun the next day!

September 3, 2009

Disneyland was great! Our 1st ride was Alice in Wonderland. Torie got off the ride and told Lexie "WOW!" She loved all the rides but Dumbo and Small World were high on the faves list.

Tom and I took Torie back to the hotel and Lexie and Shaun stayed to play at Disney. About 4:30 we got a call from Teresa Kivisto at the Univ of Minnesota...Torie had been accepted into the program to receive Intrathecal enzyme and stem cell transplant! This time it was tears of joy! Disneyland really is magic! We started planning our trip to Minnesota.

The theme of Disney's parade is CELEBRATE! and we had every reason to!

September 8, 2009

Lexie's 22nd birthday! We sang "Happy Mommy" and enjoyed a last day at Disney. We flew back to Grand Junction on the 9th, and turned around to go back to Denver on the 12th.

So I guess that catches us up some. Torie just got to recovery and we get to go see her. I'll post more about our week here shortly.

Lexie's horoscope for this week:

Saturn - the planet that's caused you misery these past 2 years - finally does you a favor when it conjoins the Sun in Virgo on the 17th, freeing you of a stubborn problem. It'll still cost you time or money to resolve the issue, but the price is worth it for the peace of mind you'll get in return.

Weird, huh?