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!