Friday, June 30, 2017

Food and a Changing Metabolism

Food is such a tricky subject as it comes to PWS kids. Well, families. Food 'rules' or routines fall into place whether they are totally planned or not. Breakfast, lunch and dinner with appropriate snacks. Quantity worked out for the individual and managed from the outside. Types of food - low carb/high fat for Ayden Jane. This includes zero grains or simple sugars and consciously choosing things like avocado. Pairings of certain foods. No fruit all by itself. So blueberries with nuts. Or apple slices with almond butter. You get the picture. It's hard for me to even remember what must seem like rules to others because we have been at this for 9 years. It's our normal.

So what happens when metabolism changes? What happens when the body can begin to use stored energy a bit and not NEED more food the second it runs out of fuel? What happens when you are suddenly awake and active more hours in a day and have so. much. energy? How do you know how much you are supposed to eat? How do you know when you are supposed to eat if you are in the middle of having fun?

These are the questions we are working through. Yes, they sound like wonderful things to be working through and they are, but they are also confusing. Ayden Jane has never had "PWS hunger". She learned to understand the cues her body gave her in regards to food and just knows what an appropriate portion size looks like. So, what do you do when the cues are different?

We have come across this twice this week. First time was when we were at the water, bouncy, obstacle course this week. It was HARD work. I did the first session with her and I was tired! She did a second session and it was 1:00 before we hit the car and ate our packed lunches. Then we stopped at a friends to swim in a pool for a bit and by 3 Ayden Jane was tired out. She wasn't sleepy and didn't say she was hungry but she was ready to just sit for a little while. We stopped for an errand and by nearly 4 I decided that even though lunch was not long ago she might need supper. It took her a minute but I said the words chik-fil-a and she decided maybe it was a good idea. After she ate she perked up and said she had no idea she needed supper already.  She followed it up with a chia bar and was asking me if it was okay. I asked her if she was still hungry and she said just a little. I told her that was her answer. A little hungry so try a little extra. She was baffled by the whole thing and has asked me about it a couple times. She can't figure out how she could have been so hungry and yet not had the feeling she usually does to let her know she needed to eat.

The next time came last night. Gary grilled and with 6 of us in the house (and one being Noah) that means grilling a lot of food. There were hot dogs, burgers, chicken, corn on the cob, asparagus and grilled apples. Ayden Jane had half a hot dog and half a burger with some corn and cherries. Later Gary mentioned the asparagus and apples and asked AJ if she wanted any. She said she had already eaten her supper. I told her it was fine if she wanted some but she repeated her supper was over and could we save some. I thought she was worried it would be too much food so I reassured her again. She got a little exasperated with me and said, "I already had my supper and I don't NEED any more. What's that word for when you are not hungry any more, you are ..... (I said full?) YES. I'm full so save some for me to have tomorrow when I am hungry."

Oh my. She is learning to read new cues in her body and even needs a new vocabulary for them. So tonight she referred back to the chik-fil-a thing. She asked me how she will know when she just needs more food and if she will get the feeling of enough if she doesn't need more food.

I wish I could answer that question by saying you can just trust your body. I honestly don't know if you can kiddo, but you amaze me with the positive attitude you keep in the midst of it all.

Wednesday, June 28, 2017

Fun Times


Today Ayden Jane and I went to a fun place with 2 of her friends. She had a blast and worked hard!
Just imagine a giant a bunch of bouncy things, now get them all wet and slippery. Next, make them wobble a bit as lots of other people are on ones all around. 

Yes that is Ayden Jane jumping off the top

Now add some slippery things to climb, a few ropes and "rock" walls that lead to tall jumps. 

Friday, June 23, 2017


I feel like most of what I have to say is the same thing over and over. Ayden Jane is doing great, but still has a lot to catch up on. She can understand more, learn more, move more... but her starting point is several years behind.

It's entirely exciting and amazing.

It's entirely painful and frustrating.

There are so many great moments.

Her coach noticing the change in her this spring and commenting that she seems to be "outgrowing the quirks she had from whatever that thing is called I told her Ayden Jane has."

The parent of a friend we haven't seen since last fall telling me, she must have learned a lot and worked hard in third grade, They had a great conversation.

Doing pretty stinkin' well in her first swim meet moving up to swimming 50's with turns!

A grandparent and medical professional saying, "Ayden Jane's swimming looks great this summer. Her whole body is so much more organized."

Watching her make friends with a sweet girl on the swim team who simply offered encouragement and friendship. Next thing I know they are chatting away and slip off to play hopscotch.

Reading the book Wonder with her and being amazed by her insight into the characters feelings and motives.

Then there are the times where the amount of information she can take in, multiplied by the depth she wants to understand, divided by the square root of the number of questions humanly possible ... makes my head hurt.

I am sure it must be overwhelming in that brain and body. So much new information coming in. So much to organize....

Go sweet Ayden Jane go. The determination, the love of learning, the joy of life. So much fun to watch. 

Friday, June 9, 2017


A few people have asked me this week how Ayden Jane is doing. It's a simple enough question but if I am to answer truthfully and completely, it's a long answer. So, here goes.

Energy wise, Ayden Jane is doing GREAT. She is up around 6:30 or so and goes strong all day long. At times it seems like she has too much energy. She will go from playing in the ocean for hours to swimming in the pool for a bit. You would think that would make her tired, or at least calm. I think she is just really enjoying the feeling of energy. That probably sounds weird, but until this summer, she would go pretty well, but we would have to watch for crashes in energy. It always was an effort for her to keep up. She was determined and did not want to miss anything so she would hang in there, but she just goes with ease now. Of course, she is also talking non stop and super fast so it would be nice if she came with an off switch.

I told her the other day that I was going to hide her Pitolisant because I can't keep up. She grinned and told that wouldn't work because she is too smart for me now. I'd say she is enjoying the added energy.

There are other areas we are watching for improvement. One is tone. Ayden Jane is low tone, but not too low. Lots of kids with PWS have significantly low tone. I think Ayden Jane's tone has improved by watching her sit. She used to sort of melt into the sofa and you could see her posture fall apart. Now she looks more typical. She stands up better without leaning into me and she can get going easier. When your tone is low, you sort of need to tell all your muscles to turn on, gather strength and then move. Every time... The muscles turn of the moment you are done actively using them so you have to round 'em up every time. I can see Ayden Jane get going much more quickly now, she can hop out of the chair, change direction, and generally just react more typically.

A great things is that Ayden Jane just feels good. She is happy. She is relaxed about food, now trusting her body that she can hold it together if supper is late or a snack is missed. I think she was more fearful of feeling bad because she ran out of fuel than she was actually hungry (before Pitolisant). This made knowing when and what food was coming always lurk in the back of her mind. She is freed from that now. I asked her the other day if she was okay or we needed to get a snack. It was the first she seemed sort of spacey in a long time. She looked at me like I was crazy, said she was fine, and ran and jumped in the pool.

There's more, but that's all I can think of now.

Tuesday, June 6, 2017


It has arrived. Summer is here! By 9 AM on Saturday - first school free day of summer, Ayden Jane asked me a bit impatiently if we could go to the beach YET. I put my list of things I had put off all spring to do after school was out on hold... and we hit the beach.

We arrived at the beach before 10 with a packed lunch in the cooler and the day ahead of us. I figured we would be out there a couple hours. Well, Ayden Jane found friends, boogie boarded, played longer... About 2:00 her friends were heading off the beach so I was able to tear her away, but of course she wanted to swing by the pool. Lets just say we made it home about 4:30.

So, that seems pretty amazing as it is. I mean, 6 hours of playing in the ocean and pool. I thought for sure she would collapse and be down for the night. Just to prove me wrong, however, she asked if we could go for a bike ride after supper! I mean, did we really need to do every summer activity in one day! Thankfully, I survived and lived to write about it.

We are working on getting into a summer routine of sorts. This week is pretty much making things up as we go. No camps, no arranged play, just making it up as we go. That pretty much translates into Ayden Jane looking up the weather and telling me we NEED to go to the beach early because it might storm later and she NEEDS to get her wiggles out.

We are getting a small, soft top surf board. It basically looks like a big boogie board with fins in the bottom. She is determined this summer she will be surfing. I am sure she will fall a lot while she figures it out so I just want her to fall on something that will not hurt her. She just loves the ocean. Once she figures it out I see lots of time spent trying to capture a video of her riding a wave! It could be an all summer long project.