Written by TJ and Jaime | 01 August 2010

So the voting is over for the Pepsi Refresh Challenge.  When it ended tonight, Sophia's Cure foundation was still in first place.  It will take a few more days until the winner is officially announced.  We are so proud of the Gaynors for sticking with this after the first loss.  We will know shortly once they tally all the votes if they really won.  Regardless, it's a HUGE victory for SMA.

Nora got a special treat tonight when Grandpa Gooden read to her.  It was really special to watch the two of them together - thinking about how I was in the same position 34 years ago.  Nora adores her Grandpa and I'm positive the feeling is mutual.

As promised I uploaded part II of Nora in her powerchair.  This video was taken in the evening on that first day she received her chair.  We moved out into the driveway after that near wall hit in the first video.  You can see how good she is at controlling the chair already.  We were all standing outside of her field of vision to encourage her to turn to see us.  To our surprise she turned the chair not only once or twice but every time!  It's also interesting to see that when she does turn to see us, she knows to stop moving so she doesn't keep going all the way around.  We are having so much fun watching her in the chair.

YouTube Nora powerchair part duex

Grandpa and I rigged a wooden ball and a piece of cork on the end of her joystick.  We really think she may have an easier time with something bigger to hold on to.  Jaime is busy sewing custom pads to help position her arms correctly so we don't have to use those pillows crammed into her arm rests.  We are all so excited to see how well she does with these little customizations.

The bad news is Nora's heart rate is up - quite a bit in fact.  She sitting at around 140 when she should be in the 110s.  We are watching her closely now for that first sign of a fever.  It can be a dozen other things causing it besides impending sickness. She took a poo today that left us all speechless.  Seeing something that come out of a person so small seems impossible.  So I hopefully the heart rate is poo related.  We'll keep you all updated.

Well g'night Nora fans

Written by TJ and Jaime | 31 July 2010

True to my prediction, this was a Friday to remember!

First, Nora Got her Powerchair!  It's a permobil Koala which is the smallest of the pediatric chairs.  This is just a trial so the chair she is using now, may not be the chair she ultimately ends up with.  A trial is used to satisfy the insurance company that Nora can benefit and actually operate a powerchair.  A successful trials means insurance is less likely to deny the cost of the chair.  Anyway, they fitted an itsy bitsy teeny weenie joystick and Nora only took about 12 minutes before she understood that the wiggly business end of the stick makes the chair move.  She started with backwards circles and then bolted directly into the wall....almost  We are thrilled that Nora has taken to this so quickly!  We do feel that the joystick needs some work to make it more effective, but that will come.  We are also getting a front bumper for obvious reasons. I bet you wish I had video of the event, but since I was at work when it arrived, I didn't get video.   Nurse Kim did though!!!!!

Nora's gets the chair video

Second, Grandpa Gooden (aka Ron, aka my dad) came to visit.  Talk about perfect timing since he got to see Nora's first day with her learner's permit.  He is going to be a huge help this weekend.

Third, Sophia's Cure moved into the #1 spot in the $250,000 Pepsi refresh challenge!  No clue how that happened, but we must have been closer to #1 then everyone thought.  Now we just need to hold that position for one more day!!!!!!!  So, please remember to vote one last time tomorrow!

We took Nora's chair out in the evening to the driveway (less drywall out there).  That video is almost done so I'll get it out tomorrow (Sat)

Written by TJ and Jaime | 30 July 2010

It's the last day of the Pepsi Refresh Project.  Sophia's Cure is still in 2nd place.

We need this last push to make a run for first place.  Vote Here

Nora is going to start her powerchair trial tomorrow.  At noon the powerchair should be dropped off for us to try for a week.  I think it's not the right stroller for Nora but we'll see how she does.  We are getting the Permobile Koala.  I should have some really fun/interesting videos coming soon.

Nora's ramp will be delivered tomorrow too.  We can use it for the powerchair too but it was purchased to help get her existing stroller in and out of the house.

Jaime has had a really tough work week.  She's been coming home late each night and working most of the evening.  She really needs a break.  Speaking of work, I saw my doctor about my sleepiness issues.  It's no surprise that I often am tired from lack of sleep.  Since daycare booted us, we cannot afford the hours for a night nurse any longer so Jaime and I cover that time.  We spend all our nursing hours on Nurse Kim who watches Nora each day.  Having a nurse here all day is great for Nora and us.  I'll save adding more to this subject for another post - in short we are doing really neat things with Nora each day to stimulate her development.  Anyway, caffeine isn't working anymore, so I did some reading and found out there is a drug they give to 3rd shift workers who often have to adjust to 1st shift schedule.  Sounds like a perfect fit, right?  Being drowsy at work is something I cannot tolerate....nor does my boss  Work is important to me and my job lets me have insurance which pays for Nora's equipment and Nurse Kim.  So the doctor wrote me a trial prescription to see if it will work for me, and then insurance denied it when I tried to fill it!  I'm not paying $499 for 30 pills.

It's crazy that insurance would deny it.  Without something I have a huge chance of falling asleep at work or my productivity goes so far down I get sacked then loose my insurance all together.  Sheesh insurance company!  either approve enough hours for a night nurse or allow me to stay awake after working the night shift.  Don't take away both options!  That's just being cruel.

Written by TJ and Jaime | 27 July 2010

Our SMA friends Leah [SMA type-2] and Mom [Kate] were visiting family in Tennessee and stopped by to see us.  I hung out quite a bit with them at the SMA Conference, but that's not where the relationship started.  We first heard about them during our trip to Opry Mills in Nashville TN a long time ago.  This was before we received her diagnosis and we were still puzzled regarding Nora's condition.  Jaime's friend Tammy told us about her sister-in-law [Kate] who's child has this horrible disease called Spinal Muscular Atrophy.  The symptoms were similar to Nora's and I remember thinking how terrible it sounded.  We got back to the hotel room and looked up everything on SMA that night - but dismissed it all because it was genetic and nobody in our family had anything - so we thought there was no way Nora could have SMA.  It's sad looking back now and to that story about Leah and thinking how horrible life was going to be for this little girl.  Fast-forward to the present and ohhh what we would give to have Nora be just like Leah.  What a cruel twist of fate that experience turned out to be.

Anyway, Leah and Nora hung out together and colored.  Well Leah colored and Nora just fixated on Leah.  Who wouldn't  though?  Leah is a precocious 4 and a half year old.  She knows all about SMA too.  I tried to explain that Nora was weaker and Leah corrected me saying "Nora has Type-1 SMA, I am a Type-2"  Then I said "do you still eat with your mou--?" and Leah interrupted saying "no, I don't have a feeding button but if I don't keep using my pacifier to make me strong, they say I may have to someday".  Leah is simply amazing.  She has weakness in her hands and when she colored she just passed the markers to me to take the caps off for her.  She knows her capabilities and isn't the least bit shy about asking for help.    Like when she was eating Mac-n-cheese with an adult spoon and after 3 or 4 bites said "do you have a smaller spoon?  I'm having a hard time with this one".

But to see Leah in her powerchair is to see the real Leah.  She is so nimble with the controls especially since she only travels at Mach II speed.  She is like a typical 4 year old.  30 seconds after putting her in the chair "where's Leah?"  she just takes off and explores.  When we went for a walk she sometimes stayed with us, but most of the time she was about 100 yards ahead of us - just being totally independent.  Jaime and I love little Leah.

I also uploaded another video of Leah's visit on YouTube.  See her in action