New School Prep

The big day is just one week away!  Ayden Jane attended a sweet little preschool for the past 3 years and was known and loved and grew so much.  The staff all knew her and enjoyed her quirkiness and automatically double checked when food was around to be sure Ayden Jane had what she needed. 

This year is the big transition to Kindergarten.  Not only is she now going all day she is in a new school which is MUCH bigger than her little preschool.  There is no way all the staff will know who she is, and really most of them will not have a need to.  (although if I know AJ they will know her soon enough) 

There are a few key folks, however that need to be clued into all that is Ayden Jane.  I emailed her principal and mentioned my desire to check in with some of the staff.  In particular, I want to meet with the folks in the cafeteria, the school nurse, the special area teachers and, of course, her teacher.  The special ed. teacher that will be the point person for her IEP has already been filled in and won't be actively teaching Ayden Jane so I want to make "AJ's rules" clear to those that will see her day to day.

I heard back and she told me when to come in (Monday) and that she would let all of these people know.  I offered to do it in small groups, one on one or in one big group if they prefer.  I am happy to repeat myself as many times as necessary - whatever forum means they are listening!  I am having a little trouble deciding whether to let Ayden Jane ride the bus home or pick her up from school.  Basically our neighborhood is directly across from the school so 'picking her up' simply means walking over and getting her.  That car line is just too dang long!  If the bus brings her she will be dropped off at a corner a little bit closer to our house.  Just means I darn well better be there when the bus gets there!

Well, I guess I am off to gather the necessary papers!  Maybe make my own "Ayden Jane Rules" poster.

The Awesome and the Exhausting

What a few weeks!!  I have been playing with Ayden Jane's supplements over the course of the past month or so.  First we added the carnitine in early July.  It has allowed her to get so much energy out of her diet that her hunger dropped and her energy skyrocketed.  The naps went by the wayside and her goofy, fun self resurfaced. 

I also decided to play with a supplement that is not often used in PWS, but I tried when AJ was little and really saw some good from.  Okay, Dr. Miller suggested I bring it back...  D-Ribose.  I have no way of sorting out what has caused what but here is what is up.

Ayden Jane can now write!!  Okay, maybe I did not make it clear.  One month ago Ayden Jane could read and add and count to a hundred, but could not even write her own name.  The connection was just not there.  She knew the letters, the sounds... but could not make what was in her head come out her hands.  She would cry in frustration, "but all my friends can write their names." 

Let's try it again.  Ayden Jane can WRITE!!  I hope you are doing the happy dance now.

Here is what I came home to the other day.  It is just the beginning of what went on!

 

 

This was the title page to a whole story she was working on.  Mckenna helped her.  At first she just spelled the words out loud to AJ then she wrote out sentences for Ayden Jane to copy.  AJ jumped up and down and cheered for herself.  "I made a 'W' for the very first time!  It is perfect.  I am writing!"

 

It is truly nothing short of amazing.

 

Of course, there is always a 'but'.  During the past week since the brain hand connection is suddenly working, her speech is painful to listen to.  There are two things that seem to be going on to cause this issue.

 

1)  Her ideas are elaborate with lots of detail and connections...  Her brain is soooo much faster than her mouth right now.  Result?  Saying the first half of a sentence 5 times really fast while the brain is working on the rest of it.

 

2)  She is just plain talking so fast her articulation is awful!  My mom actually asked me if she was going back to speech soon because she can't understand her anymore.  Frustrating.

 

History tells us that she takes 2 weeks to process these sorts of spurts and integrate the new skills more seamlessly so that means I have one more week.  Puts her ready for Kindergarten by the first day of Kindergarten! 



This is one of the craziest spurts yet and the tornado of activity and speech issues and general going way too fast and too loud seems proportionate to how awesome the spurt is.  If I make it through one more week it should be smooth sailing to start the school year!

Ayden Jane Runs for Her Future

Ayden Jane continues to amaze me.  We were walking the dogs the other day and talking... and talking... and talking...  I think that is part of the reason Ayden Jane likes to walk so much!

Anyway, we were talking about Mckenna and running cross country and how far it is for Mckenna to run a whole race.  I mentioned the One Small Step 5K coming up in October to raise money to help the doctors like Dr. Miller find ways to help her and keep her healthy.  It will be hosted by her new friend Ellie's family in Spartanburg SC.  Ayden Jane got soooo excited and told me she wants to run it too and help Dr. Miller fix her Krebs Cycle!

So, my 5 year old wants to train for a 5K to help fund medical research.  Her 'training' is going pretty well and she is already beginning to receive donations to her cause!  She started jumping up and down and cheering when I told her!!  I need to keep a map or a list or something visual so she can see how many people out there are cheering her on in her quest.

Here is the link to her fundraising page. 

Ayden Jane's 5K

AJ, like any 5 year old, doesn't really get the values of things, but I know she will count how many people are rooting her on!!

Keto May Hold Answers

Summer is flying by.  So many things to post about lately... so little time.  One of the things that is being discussed in the PWS world is the ketogenic diet.  It is really fascinating.  I am not a nutritionist by any means and really do not know much about food.  Honestly, I could eat the same thing every day and be perfectly happy.

That being said, I do notice how food effects Ayden Jane.  It has been pretty obvious for a while now that Ayden Jane uses fat for physical and mental energy.  Also, that she does not use carbohydrates for much other than to store fat.  How do I know these things?  I'll start with the carbohydrates.

When AJ eats a heavy carb load, she tends to struggle to get words out.  She gets almost sleepy and honestly a little mean.  Dr. Miller explained the Krebs Cycle and it made sense...

So, why do fats work well for Ayden Jane?  I think the answer lies somewhere in the information on the ketogenic diet.  I have not done the food logging and all to figure out how close to keto we are, but as I have 'listened' to people who are truly keto and others trying to get there I am realizing just how much fat Ayden Jane is already eating. 

The idea of a keto diet is to eat a high percentage of fats (good fats like avocado, coconut oil, nuts...) and limit carbs and protein so that you sort of force the body into using fats as it's main energy source - ketones.  Ayden Jane's diet is not truly keto genic because she eats too much protein and because I have not really searched out all the hidden carbs.  For example, onions.  Ayden Jane loves them. 

I really enjoy following all the discussion and I am happy that for now, we are fine without going all out keto.  (which is really hard work)  Also happy that if Ayden Jane starts to have trouble we can go a bit further with her diet.  For now, I will continue to keep her carbs low and focus on ways to get good fats into her.  I am determined to get more creative for her and am almost looking forward to learning to cook better this fall when quantity is no long and issue.  Almost...

Just When You Think You Have Things Figured Out...

Prior to heading to Dr. Miller we upped Ayden Jane's carnitine.  It was a missing link in her energy chain.  It was awesome to see her so full of energy!  At this point, the energy is not so huge, but she is still calm and happy and easy.  She is learning and funny.  She is not hungry.  She can go a long time between meals and still think clearly.  It is awesome.

The past couple of days there have been some spurts reminiscent of when she was little.  Just sort of leaps in skills.  I haven't noticed these sorts of leaps much in a while.  I guess I sort of figured that they wouldn't happen much now as she has accomplished most of the major milestones. 

Okay, so what am I talking about?  Here goes.  First up was at the swimming pool last night.  I know that Ayden Jane is always swimming.  She has been a good swimmer for a long time.  However, the coordination of a true freestyle has eluded her.  Her ability to motor plan alternating arms, kicking her feet... all together and then let alone breathe!  Last night I was speaking with a friend at one side of the pool.  Ayden Jane was swimming around doing her own thing and a nice woman (who I think was a bit worried that my small child seemed to be so unattended in the pool) asked Ayden Jane if she could swim.  AJ apparently told her she could and decided to prove it.  She swam a solid, controlled freestyle, took a breath TWICE and crossed the whole pool.  So cool!

The other shocker of the day came earlier on our way from the beach to the pool.  Ayden Jane started asking me how to spell things.  I have tried to get her think this way before and I don't know if she could because she just didn't want to play that game.  I turned her questions around and got her to try to spell a them.  Of course she could not spell them perfectly but she did a pretty good job of spelling them phonetically.  I was really surprised! Yep.

That's a lot of 'jumping' in one day!  And to think I was beginning to worry that her energy had dropped.  I am guessing that it hasn't really dropped, but been diverted to developing new connections.  Hoping for a fine motor jump soon!

The Winds of Change

The theme going into the fall is change.  Kayla is heading into her junior year at Clemson and is preparing for MCAT's and feels the clock ticking on decisions as to just what she wants to do.  Noah is heading off to his freshman year at Coker and his first venture into independence and managing his own time and money.  Mckenna is headed for middle school and all the fun and challenges of becoming a teenager.  Ayden Jane is off to Kindergarten at 'big school.'

Whew! At close look.  Kayla knows she wants to work in research.  She had a tremendous time shadowing at UFL and really enjoyed the specialties of rare disease research.  She can see herself specializing in something 'rare' and helping the patients and families...  On the other hand, there is so much out there and she really only knows the small amount she has seen.  She is planning on volunteering at Shriner's in Greenville this school year.  She has a scholarship which requires volunteer hours so it will 'kill two birds with one stone'.  We pray for her to hear God's voice in finding the right direction and fearlessness in taking those steps.

Noah is headed off on his own.  He has tremendous leadership skills, however he always takes a bit of time to 'get his feet under him.'  He will likely not enjoy the feelings that come with being a freshman on campus and trying to figure out strange computer programs, where to get help, how to....  He has a fantstic opportunity that he has truly earned both academically and athletically. I pray for a smooth transition so that all his potential can quickly surface and he can soar.

Mckenna is 12 and entering 7th grade.  Here that means her first year in middle school.  She is running cross country for the high school, still playing club soccer and should have a more challenging academic year.  In addition she plans to continue her art classes and is beginning to take up guitar.  Oh, then there is chamber choir and leading worship for the middlle school youth group.  Wow.  Now that I write it that is a LOT!  In a way, I feel like it is Mckenna's year.  I know that sounds odd, but Ayden Jane is older, finally healthy and in school a longer day.  Last year Noah was a senior and we did a lot to not miss his 'lasts'.  Gary spent a lot of time with Noah, just the two of them looking at schools or just hanging out as guys.  Mckenna is about to take over the role of oldest and I think she is ready to really come out of her shell.  (oddly enough the kid is shy and awkwards at times.  How a beautiful, brilliant, artistic and athletic kid can be shy and lack confidence I will never understand :)

And, of course, Ayden Jane starts Kindergarten.  I am excited for Ayden Jane and oddly not concerned about her.  Well, other than the frustration she will experience trying to keep up with the ability to write.  I believe it will be a big challenge.  Luckily, her social skills, reading skills and math skills are ready.

And me?  Well, I will have a lot less laundry to do and grocery shopping will be significantly different!  I really need to go back to work, but that does not seem to be looking like it's gonna happen.  I have big plans to learn to feed Ayden Jane (and the rest of us) better quality and shift AJ's diet a bit more toward fats for her brain.  I will keep a couple of my friends kids after school and substitute when I can.  I can't help but hve the feeling that there will be a significantly differnt feel.  I pray for patience and trusting God for the right job at the right time and that I will go where He sends me to make that happen. 

Gary has some decisions to make professionally as well so it promises to be a big year for us all. 

Florida Wrap-Up

I am feeling a bit drowned in medical stuff again.  It seems the feeling comes with the territory and I know that the feeling comes a goes, but it is still not fun when your in the middle of it. 

First up is easy.  Ayden Jane has a sore at the corner of her mouth.  Mckenna used to get them all the time!  Luckily, AJ does not have any desire to pick at it so we were just letting it heal and not thinking to much of it.  When we saw Dr. Miller she took one look and asked if Ayden Jane had it long.  Of course, I was quick to let her know it was not caused by the lovely skin picking habit of many PWS kiddos, but that had nothing to do with where she was going.  She said that those sores are a sign of zinc deficiency.  Crazy.  I used to have AJ on a zinc supplement and had just ordered some more because I was working through all the supplements I had used in the past.  I started it today (the sore has been there about 2 weeks) so we will see!

Next up, carnitine.  I am still at a dose of 1000 mg/day of CF and 500 mg/day of acetylcarnitine.  Dr. Miller is happy with that dose (yea me, I can do math).  I thought that AJ was beginning to smell a bit fishy which can happen if you take a bit too much carnitine, but Wed. we realized that AJ had a raging UTI.  I am keeping the dose up and waiting to see ....  I don't mind a bit of a fishy kid in the summer when we are swimming and playing, but I don't want to send a stinky kid to school.  The added energy is about to exhaust me, but how can I be frustrated with my PWS kiddo being too full of energy and have no need for snacks.

Dr. Miller brought up the idea of a prebiotic to go with AJ's probiotic.  Way too soon for me to know what I want to do with that thought yet, but it will keep me busy a while.  As far as I can tell, a prebiotic is basically food for the probiotic so it seems like it would be a good idea but I am sure it is more complicated than that.... It always is.

Lastly, Wednesday we met Jen (PT) at Mr. Alan's (Orthotist) office to put together a 'derotational strap'.  What is that you ask?  Basically, we still have trouble with Ayden Jane's right leg wanting to internally rotate at the hip.  AJ she has stretched some ligaments ect. over time by sitting in a poor position with that leg.  It even accentuates her scoliosis when she gets back in the bad habit.  The idea with the strap is to hold her in proper position so her muscles get used to working that way.  For us, we have been wearing it when we walk the dogs.  So that means however long it takes us to walk 2 miles every day.  I have to say that the kid is again amazing.  She just takes the added hardware and extra work in stride.

Dr. Miller wants me to check in by email in a month with dosages, hts and wts and a quick report.  We may then look to play a bit more and see if ribose does anything for her now since it really seemed to help when she was little.