T+1367 Graduation, Gratitude and Gaps

 An update!  Once again I'll try to get caught up before the end of the year! 

Torie had a great year at Preschool, and graduated in May.  She enjoyed making new friends and having something "Just for me to go to." 

We really appreciated the VERY small class size and all the individualized support from her teachers!

On the subject of being appreciative....as most of you know Torie and I go to the hospital once a week for her enzyme infusion or ERT (Enzyme Replacement Therapy).  We go to the Pediatric unit for it, and they access Torie's MediPort, draw labs, and infuse her enzyme.  We're there for about 5 hours every week.  I am so grateful to the Pediatric Nurses at St. Mary's Hospital.  They are so kind and caring and efficient and knowledgeable and competent. 

 Torie is always excited to go to the hospital.  Weird, right?  But the volunteers have a Popcorn machine set up and sell bags of popcorn.  And no one loves popcorn like Torie does!  So it's "Hospital Wednesday, Enzyme Wednesday, Popcorn Wednesday!"  She gets her popcorn, we go to admissions (where they put stickers on her armband and they register her as soon as they hear her voice in the lobby!) and we head up to Peds.  She loves being the Elevator Operator for everyone.  As soon as the doors to Peds open she runs in and says Hi to everyone, usually with hugs.  She has her routine down, and they have all adapted to her way of doing things.  She sits and watches TV while she's accessed, and she tells her nurse, "That didn't hurt at all!"

 I can't imagine how awful it would be to have to come once a week for 2 years if she dreaded it.  I'm not sure I could drag her in kicking and screaming once a week.  But these nurses have made it so easy for Torie and I.  I am so fortunate to call these awesome nurses my colleagues and friends!



Torie has now lost her top 2 teeth and her bottom 2 teeth leading to the "Gaps" portion of this post.  She loved her visits from the Tooth Fairy and she's always checking to see if she has any more loose ones "So I can get some more money!"

T+1196 MPS Day and Other Stuff

 Happy MPS Day!  3 years ago today Torie was T+100.  Yes, I really do still keep track.  I think I probably always will.  Our lives centered around these numbers for so long.  We don't count meds anymore (there are none!), ANC (Absolute Neutrophil Count) has gone by the wayside, we've been home for over 2 years, no weekly clinic visits, but those T+ numbers still matter.  I have been grateful beyond words for 1,196 days that someone was caring and giving enough to donate their child's cord blood to give Torie the enzyme that she didn't have. 

Oh, this poor neglected blog!  Gone are the days that sleep was out of the question until I updated!  I guess that's a good thing.  But for you loyal followers, never doubt that you are so appreciated!  Prayers and happy thoughts, along with faith, hope, trust and a bit of fairy dust have gotten us to where we are today.  So now I have a ton to fill you in on, so be patient and read on!

March 24th we went to Minneapolis for Torie's 3 year/6 month study check.  We were so lucky to spend time with Joseph, Kim, and Pat, and with the Carman's, Josiah, Torie's Un-Birthday Twin-Triplet, The Mommy (Rachael), and The Daddy (Jim).  Our schedule this time was really light, so we had a lot of time to play!  We did all the never-miss-in-Minneapolis things...Mall of America, Ronald McDonald House visits,seeing Jerry,  Punch Pizza, and Juicy Lucy's.   

 On to the medical stuff....Torie did the treadmill!  The hotel had an exercise room that Torie saw when we first got there.  She told me "I have to practice my treadmill."  She only did about a minute but at least it was her idea!  She wasn't thrilled about the real test, but she did 6 minutes at 1 mile an hour at a 6% incline.  And Papa and I worked for that 6 minutes!  Dr. Polgreen was pleased with her results though, and her EKG and Echo are very stable.  Her PT (Physical Therapy) eval went much better this time; she was standing upright!  In September she had PT about 45 minutes after she woke up from OR.  Much better this time!  And her measured walk went to the vending machines this time.  (Papa Tom is a pushover at vending machines, and we have a cute little red-head who is well aware of it! Hello, popcorn!)  She made Dr. Orchard a popcorn sandwich with her leftovers, and he was pretty pleased with her progress. 

That brings me to the not so positive.  Torie has now been in the ERT (Enzyme Replacement Therapy) study for 6 months (as of the end of March).  She has developed antibodies to enzyme.  And this may be reflected in her engraftment level.  In September her CD15 level was 79%.  Her most recent is 70%.  Dr. Orchard feels this may be due to antibodies to enzyme.  Torie has never been 100% engrafted.  If her engraftment level drops more than 20%, we will stop ERT.  We're not sure what this means for the enzyme that she has been making on her own since transplant.  (Let me just interject that I hate antibodies!  Hemolytic anemia, Evan's Syndrome, engraftment levels, Nephrotic Syndrome, etc.) 

Ryker mows for Vic-Torie!

I don't know if we have seen any great improvement from a neuropsych standpoint.  Torie has had some advances, but she is also showing some behavioral issues.  It may be because she is just acting like a 3 1/2 to 4 year old and she is 6 months older than her last testing.  And we aren't sure if preschool has made a difference (I'm sure it has to some point) or if it is a maturation age.  Their recommendation is that she starts kindergarten in the fall.  Which is great because Torie loves school!
  

 

 OK.  Anyway, we will continue to put our faith and trust and little bit of fairy dust in Dr. Orchard.  He has gotten Torie, and so many other kids this far.  And you might be able to tell that she is pretty crazy about him, too.  Dr. O is just another of our silver linings.


I think one of the things that frustrates me about MPS I, Hurler's, is the unknown.  We don't know what will happen.  We don't know what Torie's future holds.  We don't know how long her future will be.  We just don't know. 

The week we were in Minneapolis was Super Hero week at Fairview U of M/Amplatz Children's Hospital.  Torie is my Super Hero.  She runs and jumps and chatters and laughs and hugs and kisses and smiles.  She gives and receives love.  She brightens a room when she walks in.  She is tough and funny and generous.  She has taught me to be a better person and to be present and to appreciate the little things.  She has taught me that MPS I is not a disease but a blessing.  Would I wish this on another family?  Not a chance.  But she has taught us to be a special needs family and I wouldn't trade her for anything! 

Joseph

Josiah



Torie is my...

Super Hero!

T+1176 Six Cakes

About a year ago, Torie said, "Mama, my next birthday will be 6.  I think I need 6 cakes."  And so, of course, she got them.  And this year she did a much better job of ordering them.  She said "I need pink and purple cakes.  They need to sparkle."  And they did! 

I can't believe that it has been 6 years since I met Torie.  She was born by C-section because she was breech.  Lexie and Dr. Ellinwood let me come into the OR suite.  She was delivered and handed to the attending pediatrician.  She swaddled her and handed her to me.  I loved her right away, but I never imagined the road we'd travel together!  Kind people often say, "She is so lucky to have you and your family."  But really, we are the lucky ones.  We are so fortunate that she picked us!  We treasure her every day!

T+1159 The Tooth Fairy

 Torie went to the dentist in January and they told us she had a loose tooth!  She is getting pretty good at dentist chairs now.  Her 1st visit, last year didn't go well, and she had her first cleaning under anesthesia in the OR.  Torie is not fond of having people look in her mouth, and she hates lying down for exams.  Unfortunately, the dentist is both at the same time!  But this time she actually sat in the chair and had her teeth cleaned.  She still has no cavities, just some demineralization.  I'm so glad she loves to brush!

So anyway, we talked about her loose tooth. A lot.  "Mama, I have a loose tooth."
       "I know.  Pull it out."
       "I can't.  It's stuck in my mouth."

And so on.  Frequently!  We left Arizona on February 5 to head home.  The conversation started again...
       "Mama, I have a loose tooth."
       "I know.  Pull it out."
       "I already did."
       "WHAAAT?  Where is it?"  (We were in the car and I was picturing tearing it apart to find that 1st precious baby tooth!)
       "Right here.  I have to put it under my pillow so the Tooth Fairy will come see me."

And that was that.  No tears, no crying, no bleeding, just that precious little baby tooth.  The Tooth Fairy did come and "Mama, the Tooth Fairy left me 2 moneys!" 

T+1158 3rd Un-Birthday!

 February 4th was Torie's 3-year transplant anniversary!  It doesn't seem that long ago!  We celebrated in Mesa with our Arizona family.  They have been such a huge part of this story and they have supported us every step of the way.  Torie can never get enough "Duzin" (cousin) time!

 We had an Un-Birthday party with Makinley, Chase, Laikyn, Brielle, and Cooper.  When we first started talking about going to Arizona (Papa Tom, Torie, and I), she told us, "I really need to see my Duzins.  They are my Duzins and my best friends."  I think Grandma Flake is so pleased!

 We had a great pizza party at Organ Stop Pizza.  Torie had never been there, and Tom and I hadn't been since Makinley was a bitty girl.  It was Laikyn's first time too.  The place hasn't changed a bit...even down to the organ player!

 On Torie's to-do list?  "Watch Cooper play soccer.  Watch Kinley and Brielle dance.  Hug Laikyn."  I asked her, "What about Chase?"  She replied, "Oh, my Big Duzin is taking me on a picnic."

Arizona sledding with Big Duzin!

 We didn't get to watch the girls dance, but we had a great picnic.  We had a sleepover with Brielle and Cooper at the hotel, complete with popcorn, movies, and bubbles.  We ate tons of great food at Auntie Heather and Uncle Colby's, we had some Laikyn hug time at Auntie Erin and Uncle Jason's, and generally just hung out.  We are so grateful for our Arizona family!  "We wub dem!"

T+1157 She's Back!

Our "Tute wittle wamb" in her Christmas pageant.

 Why in the world do I put off blogging for so long?  The "no news is good news" line certainly fits, but this is ridiculous!  I get so far behind that it just gets harder and harder to start.

Torie is fantastic!  She is in preschool and loving it.  I don't know what will happen when summer rolls around because she would go to school every day if she could. 

She continues on her weekly enzyme infusions and she handles it like the trooper she's become.  She knows "Hospital Thursday, Enzyme Thursday" is just part of the routine now.  She has been getting infusions for 6 months now, so only 18 months, or 76 weeks, to go!

Our holidays were excellent, with the highlight being the marriage of Lexie and TJ-Dad!  They were married on December 21, and their little family is settling in well.

"Hospital Thursday, Enzyme Thursday"

Rykerand a lemon.  "My brother is a squishy-face!"

"Mommy and TJ-Dad"

Looking kind of like Cindy-Lou Who!

T+1001 Part II

 

 Ok, back to the tale of the summer.  Ryker had his 1st birthday on August 9th.  Torie made him a beautiful cake with plenty of blue frosting.  We all enjoyed it, but from the looks of Ryker, he appreciated it the most!  Ryker has to share his birthday with Stacey and Matt's new baby, Piper.  Torie was so excited to meet her, and she had to have a party for her.  She picked out a balloon and a cupcake, and of course we sang Happy Birth Day.  Throughout Stacey's pregnancy, Torie insisted that the baby's name was Rosie.  She has surprisingly been calling her Piper since she's gotten to know her better!

 Over Labor Day Torie and Papa Tom and I went to Arizona to see the "Duzins."  Torie's Baby Duzin was born August 24th, and that story will have to have it's own post.  Baby Laikyn is the newest addition to our family.  Torie kept telling Auntie Erin "She's a wittle tutie."  And yes, she's right!

Torie got to have a hotel sleep-over with her duzins Brielle and Cooper one of the nights we were there.  They went swimming, watched a movie, ate popcorn and had a great time!

Laikyn Elle Beckerman, AKA Baby Duzin

Cooper, Makinley, Brielle, and Torie

One of our days there was spent at the Aquarium.  We all had a great time, and it was so much fun to see Torie with all her Duzins.  She is absolutely crazy about her whole Arizona family and they are all so good to her.  She misses them all the time, and tells me about once a day, "Mama, my Duzins are far far away.  They my nice Duzins.  I love them!"

Chase, who Torie has renamed Big Duzin!

The middle of September we went to Minneapolis to start a study being led by Dr. Orchard and Dr. Polgreen.  The study is for kids at least 2 years post-BMT, and will look at the effects of having weekly ERT (enzyme replacement therapy) for 2 years.  Yeah, that's NOT a typo.  Once a week for 2 years.  Torie's NeuroPsych results in February were disappointing, but not surprising.  She had lost points from the year before.  She is making 50% of a normal level of enzyme.  After everything that Torie has been through, I would hate for this study's results to come out phenomenal, and have to say "I wish we would've..."  We just want to give her every chance we can.  And besides, we have huge admiration for Dr. Orchard, and we trust him with Torie's life!

Papa Tom was never able to spend much time with us in Minnesota, and he really wanted a turn.  So Mama-Great sat this trip out, and Tom got to sample some of our favorite places.  Torie loved taking him on the roller coaster at Mall of America.  We went to the Minnesota Zoo, and had lots of great food.  We also got to spend some time with our friend Amelia, and her mom Brooke.  She is doing so well!  And what a gorgeous girl! 

2:15, Recovery Room

 Tom was both amazed and exhausted by the schedule we had that week.  He definitely developed a new understanding of all Torie's visits, tests, clinics, docs, and appointments.  Torie had to have MRI's done, and she has to have general anesthesia for them.  They were also able to do her DXA (bone density) scan at the same time.  We tried to get it in radiology, but she was having nothing to do with that!  She had a Physical Therapy eval the same day as OR.  She woke up about 2:15, left the Recovery Room at 2:45, and we made it to PT by 3:00.  I think her results may have been a bit skewed since she could hardly stand up on her own!

All of her docs remain quite impressed with her.  Her echo showed some more thickening of the mitral and aortic valves, with more leaking.  Dr. Braunlin thinks Torie may have to go back on Enalapril eventually.  But maybe enzyme will help with this.  Her latest engraftment levels from the week are pretty stable;  CD3=90%, and CD15=77%.  And Torie failed the treadmill test due to a lack of participation.  She got on for a minute, but when they pulled out the face mask it was all over!  We will go back in March to repeat all the tests, and again at 12 months, 18 months, and 2 years.  And I thought 16 doses pre-BMT was bad.  Geez.  It's gonna be a long 2 years! 

3:00, PT!

 The week after we got back from Minnesota, Torie started preschool!  She loves going to her school, with the exception of not getting to ride a bus.  Oh well.  She has school on Monday, Wednesday, and Friday afternoons.  She has OT and speech on Wednesday mornings.  And we spend all day Thursday at the hospital for infusion.  For a 5-year old, she's got an awfully full schedule! 

So, that kinda catches you up on our girl.  She is so energetic, and vibrant, and full of life.  She brightens every room she walks into, and she is a constant source of entertainment and laughter.  She is truly a joy.  And we owe you so much for those constant happy thoughts and prayers.  They really do work!  Vic-Torie!