Birthday Wishes from Clemson

Ayden Jane's birthday is not officially until tomorrow, but she was spoiled rotten all weekend by Kayla and her friends from Clemson.  It started Friday night when they arrived and played on the trampoline with her.  It was so fun to hear all the squeals of laughter through the window.  I think Ayden Jane outlasted them!  When they could jump no more they came in to read books.  How cute is this?

The main event was on Sunday.  I took Mckenna to Charleston for a soccer game and the Clemson crew took Ayden Jane on a day of adventure.  The grand finale of it all was a trip to Build-A-Dino.  Ayden Jane told me all about how they kissed the hearts and stuffed the dinos.  Then about bathing them.  Of course she had to explain that they actually used air and not water but still called it a bath.  We walked the dogs after the crew left and Ayden Jane recapped her day for the entire hour.  Some of my favorite quotes were; 

This was the best day of my whole life! 

Today makes me six already because it was the best birthday ever.

I went in and when I saw the one I picked I totally knew that was the one I wanted.

Well you get the idea.  Here are a few pictures of Ayden Jane's dinosaur birthday.

 

September

September is going by so quickly.  I feel like there is so much I should be writing about, but it is all coming so fast I don't get the chance. 

One thing I will be working on in the coming week is ketones.  I am waiting for our blood ketone monitor so we can finally answer the question once and for all, is Ayden Jane generally in ketosis.  I have been sort of slow to measure and track ect., but I just felt like I can tell what Ayden Jane needs food wise.  It is time to figure out if what I see on the outside matches what we think is going on in the inside.

The 'we' in that statement includes myself and a few crazy smart medical minds.  (and good friends)  Here is a very simplified (because that is all I can handle) version of what is being discussed/investigated by these smart folks.  We know that people with PWS have an impaired carbohydrate metabolism.  Remember Ayden Jane and her Krebs Cycle?  So, if they are not good at using glucose for energy, what do they use?  Fats.  Yep, the dreaded word in the American Diet. 

The brain can use glucose for energy, however, in the absence of glucose the liver can metabolize fats and turn them into ketones.  The PWS body has trouble using the glucose properly (although I don't entirely understand the details) so that the brain and body are not fully energized and much of the glucose is stored as added fat mass.  The PWS brain and body seem to use ketones for energy efficiently so now you have a happy brain and body.

So, this is the basis for feeding a person with PWS low carb, and I am talking no grains or sugars and generally low glycemic index fruits and veggies.  Some parents are sticking with as low as 20 grams of net carbs per day.  That is hard.  The diet is actually called a ketogenic diet and is used in treating patients with seizure disorders.  Yep, good for the brain.  We are not likely on a true Keto diet, but likely close.  Ayden Jane eats too much fruit and I add honey to a few things and she has a few carbs in her supplements....  But we definitely have never feared the fat.  In fact, avocados and flax meal and butter and eggs and nuts have been her staples since the start.

So, how does AJ fare in all this?  I discovered this week that she is the oldest kiddo to start eating this way pretty much from the start.  I did not set out to be a rebel or ground breaker.  It was not even my idea to feed her this way!  lol.  I just read what a couple of these brilliant folks wrote and watched Ayden Jane.  She made it absolutely clear that grains and sugars crashed her system.  She made it obvious that she had lots of energy and felt good when she ate fats.  So I just kept it up.  I didn't have any other nutritional guidance until she was 2.  That was the first time Ayden Jane saw Dr. Miller.  She ran an analysis of her diet and gave it a stamp of approval as well as being thrilled with how AJ was doing.

So, coming full circle, I realized this week that it is important to know what is actually going on inside Ayden Jane.  Not just think I know.  I don't expect to change anything but Ayden Jane could provide some information to these smart ladies.

What they are thinking is that Ayden Jane has learned to use fat very efficiently.  Almost preferentially so that her metabolism slips into ketosis quite easily.  Among the questions are is she in ketosis even though she is not on a diet that would create ketosis in a typical person?  What level are her blood ketones?  What level are her ketones when I feel she is really 'on'?  What is she burning for fuel when she goes and goes and goes?  How do her ketones fluctuate over the course of a day?

So, as Ayden Jane works on getting ready for her 5K, I will be using her as an information source.  Good thing she thinks having her blood checked is cool ;)

Odd and Ends

So much is going on and I am way behind.  Let the rambling begin! 

First up, our endocrinology visit and labs.  Hey, lets get the boring stuff out of the way.  We saw Dr. Clarke and our weight and height check was good.  Not perfect, but she is sitting at 90th percentile for height and 95th for weight.  That brings her BMI back in check from our last visit.  Basically she looks good.  Maybe not as lean as I would like, but definitely comparative to our other kiddos at that age.

We drew labs as well.  I thought that we were going to leave with orders for fasting labs, but we just drew them instead.  So the results?  Mostly all good.  Mysterious eosinophils still a big high... IGF1 a bit high and cholesterol high.  Soooo, we are going to draw the lipids and IGF1 fasting to see where that leads us.  I am just not worrying about it until then.

For now we are choosing to just concentrate on the One Small Step walk.  Ayden Jane is having so much fun with it.  She even asked me tonight why all those people are coming to walk just for her.  She also occasionally comments when she looks at her growing chain, "wow Mom, look how many people love me and are cheering me on!"  It is so cute.

Kindergarten seems to be going well.  We did have our first meltdown tonight over handwriting.  I have a feeling it is the first of many to come:(  It is hard for her and she sees that her peers can do it.  I think it is time for me to figure out how to hit it harder.  Any suggestions?

How Food Changed Ayden Jane

Lots of people have asked me about why I chose, back in the day when diets recommended for PWS were more along the lines of calorie control than anything to feed AJ as I do.  First of all, I learned from what L was figuring out with K.  AJ was just diagnosed around the time L went low carb ect. with K and it just made sense.  Also, O was wonderful and accessible by email and the yahoo group was a great source of info. I figure it is easiest for you to just see it.

 

This is Ayden Jane at about 5 months just before we introduced cereals.

 

 

This is at 7 months after 1 month of cereals and other baby foods.

 

This is 8 months after more cereals and baby foods... just diagnosed!!

 

 

Here she is at 10 months after we went zero grain, low carb, pushed fats and started supplements.  The mischief to come is beginning to show.

 

 

Now at 11 months with one full month of gh.  Wow I am growing and getting strong

 

 

This is 13 months.  Just 5 months post diagnosis and loving my new food, supplements (particularly b12 at this age) and all the energy.  I will be walking in just over a month!

 

And finally, Ayden Jane at 22 months showing signs of determination to come :)

 

Running It All

Ayden Jane has a strange tendency to internally rotate her right leg.  It seems connected to the scoliosis issues, of course in a chicken and the egg sort of way.  Jen has been determined to 'fix' this and it is so cool to watch Ayden Jane run now. 

So, how has she been working on it?  First up is the basic orthotic in AJ's shoes.  She has had 'yellows' in her shoes since early on and just when I don't know if they are really necessary we get new ones and I see a good improvement. 

Secondly, she has done leg/hip exercises.  These are cool for me to see because in the beginning when Jen was trying to figure out just what was going on she discovered muscles AJ was just not using.  In fact, didn't really know how to fire.  Now those same muscles are strong and AJ can find them quickly.

Thirdly, we have been using a 'derotational strap' that Jen and Mr. Alan cooked up.  Ayden Jane does not wear it a lot, but she does when we walk the dogs.  (It amazes me what this kid puts up with)  Anyway, at first it gave her leg a lot of support/cueing.  We backed it down a notch now and I am hoping to do away with it one of these days.  What I can say is that watching Ayden Jane run now she looks so much better. 

Ayden Jane is ready to run and Jen and I were talking about 'training' for the 5K.  Jen said she wanted Ayden Jane to take the amount of running slowly up.  That she does not want her to run too much at this point...  Well, Ayden Jane heard our conversation and burst out into tears.  "But I want to run the whole race!  I don't want to have to walk at all!" 

So, where are we with it all?  Last night Ayden Jane ran our whole road 1/4 mile straight through.  She wanted to go more, but that was it for now.  Then we walked 2 plus miles and she begged to run the 1/4 mile back home.  She did it!  "Mom, this is hard but I can make it.  When we go to the race I will be able to run it all."

Fun Visit

We really enjoyed our visit from 'the college kids'.  It was quick and fun and Ayden Jane is EXHAUSTED.  It's a good sort of exhausted though. 

Friday night was a trip to the HS football game.  We left about half time to come home and see Kayla and her friend Allie.  Yea!!  Ayden Jane hung on until 11 pm!  She did not let them out of her sight.  I think she was convinced that if they got more than 10 feet away from her they would be headed back to Clemson.  The girls spoiled her rotten and she loved being the center of the universe.

Saturday morning we sent her in to wake up Noah.  (she did not know he was home)  She enjoyed jumping on him for a bit before moving on to jumping on Allie and Kayla.

Off to the beach we all went...  It was a beautiful day but a bit rough in the ocean for her to show off her surfing skills.  They mostly swam and fought the current.  Kayla has pictures on her phone and I'll post them when I can!

In other news.  Starting tomorrow we will have C and C after school 4 days a week.  Ayden Jane is so excited.  It will be great for her to have friends her age to play with on a daily basis.  The boys are great and I am sure we will settle into a good routine.  My plan is snack and out the door!

I am still waiting to hear on the job front.  I think all the options I have heard mentioned work for me so I am not too worried about it, just anxious to know just what it is I will have to get excited about.

I am thinking it's about to get busy around here!

Surf's Up

We all know that when Ayden Jane gets an idea in her head...  You just have to let her run with it.  Over the weekend she saw a friend her age up on a surfboard.  The seed was planted.  Today when Gary woke up Ayden Jane ran to him and before he even really had a chance to become conscious she was informing him that he needed to take her to the beach and teach her to surf. 

 

Like I said, you just have to go with it when she gets an idea in her head!  Now, Gary has never surfed a day in his life and they took Mckenna's board which is a great board to practice on as a kid who is just starting, but not an easy board for a total beginner.  Not that any of that was going to stop Ayden Jane!  Gary had her climb up, pop up to a squat and then stand while he steadied the board.  It was pretty cool.  She didn't ride the wave far, but in her mind she was surfing and that's all that mattered!

 

 

 

 

 

 I'd say she rocked it for a 5 year old, let alone a 5 year old with PWS!

Labor Day and Stuff

So what do you do when the air conditioner maintenance guy has you trapped?  Write a blog post...

It's Tuesday after Labor Day weekend.  We had a great long weekend.  Lots of beach and pool time and a good bit of dog walking.  The energy stream just keeps flowing out of this kid right now.  The cold is running it's course but I have hopes it will pass.  I ordered more pharmaNAC (which I should have already had on hand) so starting tomorrow I should be able to double dose her on that.  Plus, she is so good at blowing her nose!!!  Hooray!!  She does not act sick at all.  In fact, she is running me into the ground!

Speaking of running me into the ground.  Mckenna has her first Cross Country meet today!  On Saturday we decided to head out and see if she can make the whole course.  She had not actually completed the entire course at one time yet.  I was really proud of her!  It was HOT and she definitely was not feeling good at around 2 miles.  She plugged on through and she finished the entire thing.  Hopefully the confidence will carry her through today!  (and I survived the run.  It has been a while!)

 Kayla and Noah sound like they are off to a good start for the semester.  Neither sounds like their course load is going to kill them (Kayla is only taking 3 genetics classes, public speaking and Spanish. LOL)  Both sound happy.  We here on the home front miss them for sure.  I think we had adjusted to Kayla being off at Clemson, but it is really new that Noah is not around.  The younger girls definitely miss him and even the dog keeps checking his room to see if he is home.  Glad we will be able to go see him soon.

As for me?  Well, there is a possible job on the horizon that I would LOVE.  A lot would have to fall in place (will require an act of God) for it to happen, but I am trying to be patiently hopeful.  If it comes through I will fill you in on the details!!

Kindergarten Update

Ayden Jane is rocking Kindergarten.  I have only heard positive feedback so far from her teachers and even from other parents who have seen her around school.

A friend who has seen Ayden Jane around school during these first 2 weeks said that she, "seems so happy and full of energy.  She has the Kindergarten thing down."  Wow.  Could I ask for a better review?

Realistically, I don't know whether AJ is talking much in school or just taking it all in.  I have my suspicions that she is keeping to herself a lot, but that's okay too.  She is seated next to her friend C from down the street.  They are a great combination.  They are pretty similar in the need for structure and their ability to analyze (talk repetitively about) a subject.  Having C by her side gives her confidence even though I don't really think that they play together much during the day.

I am doing my best to just stay out of the picture for now and just let them get to know Ayden Jane.  I will get in the mix in a few weeks to see if there are any areas we need to work on.  I really want the teachers to tell me anything we can work on.  It is a bit challenging because I am asking them to pick her apart and tell me her weaknesses.  Usually parents want you to tell them all the good stuff! 

Only down side is that Ayden Jane woke up this morning with a stuffy nose.  Please say a few prayers that we can kick it quickly and will not head down the dreaded sinus infection path!