Wednesday, August 24, 2016

Third Grade Day 6

As far as I know, Ayden Jane is doing well in 3rd grade. The first week started on a Wednesday so she had just 3 days. She handled it well and didn't seem too worn out. Of course, then she went into the weekend by going to the sleepover on Friday night...

This week is her first full week, and by full I mean FULL. She went into it a little tired still from the weekend. Then Monday she had soccer practice. She is still new to that so it's a lot. Then Tuesday she had friend over and played late.... By the time she had her homework done and was showered it was after 9.

Today is Wednesday and wow did it catch up to her. She really just needed a nap, but would not cave on that. In the end, she did all she could do and was in full meltdown and asleep by 6:30.

It is going to be a challenging balance this year. School, homework, after school activities and good old hang out fun. Ayden Jane wants to do everything ... but really cannot function when she gets too tired. It's tricky. She will push herself to the brink - and then dump on me.

Meanwhile, I am feeling like the slack mom. I screwed up some communication and Ayden Jane will miss a couple doses of gh just as she is trying so hard to adjust to the new busy schedule. ugh!! It should get here tomorrow. Just makes me so sad/frustrated that I didn't keep track of the details so that this happened. I know it sounds like no big deal. Okay, I know it really is no big deal, but somehow it just hits really hard. Pretty much all of Ayden Jane's meds, supplements, diet, PT, OT, exercise .... is my responsibility and I know each little piece adds up.

Here's hoping that she has a great nights sleep and tomorrow goes better.

Saturday, August 20, 2016

Third Grade

First day of third grade!! It was an interesting start to the school year. I took a last minute job to fill in for a friend who is unable to start the school year so I was not even able to take Ayden Jane to school on her first day.

Gary took this quick picture of our girl all ready to take on third grade. It sounds like all is well so far and she is doing great.

She also had her first slumber party at her friends house this weekend. They went over to a pool and played. The parents were ordering pizza so I told them I would bring some of Ayden Jane's pizza over for her to eat about the same time. When I came in, Ayden Jane saw me and asked, "mom, what are you doing here? You don't have to pick me up you know. I'm spending the night."

She wanted to handle this one all on her own and did not want mom around to cramp her style.

I did stay a bit to chat with MY friends but not too long.

Later I stopped by to give the gh shot to her. I tried to get her to slip off the sofa where the girls were just starting a movie so we could do it quick in the other room. Ayden Jane was not interested in that. She wanted me to come to her and just get it over with. So, on a sofa with 4 girls she pulled out her thigh and I gave her the shot. One of the girls eyes were so wide I thought they might pop right out of her head! As I left the room, there was lots of what was that?! Ayden Jane just said, "it's my shot I do at night. It's fine. It doesn't hurt." That was the end of it.

We treat Ayden Jane's PWS as no big deal. The supplements, the oils, the shot... it's just a part of life. She has totally adopted that same attitude but she has no idea that all she does is slightly shocking to others. Not that it would slow her down anyway....

Saturday, August 13, 2016


I did not think it was possible for things to get even more busy! But I think they have.

So, Ayden Jane has had a full week of the increased gh. Her weight has stabilized and I think she is already growing! I am really excited to say that she is still not needing to eat much in the way of snacks. Life, food wise, is just easy right now and I am really enjoying it. I'm sure it won't last.... sigh, but I am smiling and appreciating every "I'm not hungry" and "sure, a blueberry bite is plenty" I hear.

It really struck me at the pool this evening. She has had lots of great summer activity and was enjoying another evening swimming with her friends. Listening to her get along so great. Having compassion to take an extra turn being it. Standing up for herself when necessary. Negotiating a truce between other siblings. Just happy and healthy and confident. 

Hooray for summer!!!

Thursday, August 4, 2016

Oddity Continues

Today was day 4 of maintaining Ayden Jane's weight at a steady place. Good news. We shall see if we maintain it tomorrow as well.

Ayden Jane ate breakfast of a cheese stick, watermelon and a blueberry bite at her wide awake hour of 6 AM. Then she and Gary ran a bunch of errands and they stopped at Dunkin Donuts. Ayden Jane got a bit of sausage and egg... but just a bit.

By the time they came home Ayden Jane was itching to get into action, so even though it was only 11:30 she went ahead and had lunch which was 2 pieces of Cauliflower crust pizza. Sticking to the strange behavior she didn't even ask for anything with it or eat any fruit with lunch. Typically she plans her day around what fruit she gets at each meal because it is her favorite.

She went out into the neighborhood to find some friends to play with for a while and then around 3:30 the neighbors invited her to the beach. They typically eat supper at 6 so I figured they would be gone a couple hours tops. I gave Ayden Jane a Hint water and a chia bar, and offered for her to take a cheese stick and/or nuts. She turned down the extras and told me she'd take the bar if she needed to... Seriously?

She got home at 7:30!! That means she played from 11:30 to 7:30 with a 100 cal. chia bar.

The other odd thing is she told me she was so thirsty she drank her entire Hint water plus a water bottle from the neighbors. I have never before heard the words "I was so thirsty" come out of her mouth.

We will see what her weight says in the morning. Hoping I managed to get a enough in her to maintain her weight or Dr. Miller will not be happy with me... Maybe the extra gh will build a little muscle :)

Tuesday, August 2, 2016

Labs and a Lack of Hunger

We got Ayden Jane's labs back. Most things are fine but a couple things stood out. First of all was her bilirubin was high and her ALP low. Dr. Miller has decided that since all the other liver enzymes are great, she is going to assume it is some form of error or anomaly for now. We will retest in a month.

Secondly, her IGF-1 level shows she definitely needs an increase in gh. It is her first increase in 3-4 years! She was doing great with levels spot on until the 'winter of infection'. Then her IGF1 level spun out of control. We even had to reduce from the 0.8 down to 0.6 for a while and slowly work back up.

We have felt that Ayden Jane was a little off for a while now and lack of gh makes sense for those things. She is a bit low on energy which is definitely commonly an effect of low gh. Napping a bit during the day and needing breaks when playing.

What is not common is that her hunger has pretty much disappeared. She has significantly cut down on breakfast and sometimes skips it all together. She has not needed snacks and even cut back on the meals she is eating. I have let it roll most of the time but she has dropped about 4 pounds in a month and it has not changed.

Now, you'd think I would be doing the happy dance because she is not hungry and losing weight. What more could a PWS momma ask for?? Well, the speed at which she is dropping makes me pretty certain she is losing muscle as well as fat. Not good.

Typically, doctors do not really want to raise gh if a kid is having trouble maintaining weight or is already losing weight. I mean, common sense says it would be hard on a body to power a growth spurt and lose weight at the same time.

Dr. Miller and I talked through it and I have convinced her that this is actually Ayden Jane typical. She used to get scrawny through the shoulders and have trouble maintaining weight when she was little and needed a gh bump. I just haven't seen it in forever!

She agreed to going up to 0.9 but I will be responsible for tracking her eating and weight to make sure she is eating and her weight maintains. So, if we can keep her weight at 83-84 pounds and she grows a bit along with rebuilding some lost muscle... Wow will we be in great shape. My guess is we may need to go up to 1.0 but we can decide that in month when we recheck labs. 

Saturday, July 30, 2016

Interesting Summer

Obviously, if you are reading this blog you know a bit about Prader Willi Syndrome. The 'hallmark' of the disorder is supposed to be hunger ... insatiable hunger. This hunger is supposed to start at varying ages but certainly should be a part of Ayden Jane's life at this point.

We have seen the PWS hunger a few times. It generally comes when she is ill. I still don't quite understand why that is the case, but it seems Ayden Jane is not alone in her response to illness. I also see her misinterpret tired for hunger. Even those of us without PWS do this to an extent. You know, the late night munchies when what your body actually needs is to go to bed? Well, just magnify that by a ton.

Ayden Jane has learned to listen closely to her body and interpret the cues well. She refers to hunger as her brain being hungry or her belly being hungry. Her brain needs a snack sometimes between meals. She says she is not really hungry, but her brain starts to crash so she needs food. Then at meal times her belly is hungry. She is doing great with it all and has learned to recognize the brain crashing feeling before it's crashed too much and realized she only needs a small snack to be right back on track - like a blueberry bite or keto chocolate.

The interesting part this summer is that Ayden Jane is a little off right now. She is just struggling to power her typical activity, is having trouble tolerating the heat, and getting 'stuck' more than usual. It's not extreme or anything, just enough that I feel like I am banging my head against a wall a few times a day. Most people probably just notice that she is a bit slower getting her thoughts out. We are working on it and Dr. Miller has labs that will hopefully give us some clues.

I am really curious, though, because usually when she is struggling to power her little body and brain, it sets off hunger cues. This time, however, she is not hungry or concerned about food. She has cut her breakfast back to half of what she typically eats. She sometimes has a morning snack and sometimes not. She is in no hurry for lunch... It is so strange. I have had to remind her to eat. Over the past 2 months she has even lost 4.5 pounds. I know that does not sound like a lot, but it's pretty significant for a kid with PWS.

It will be interesting to see just what those labs tell Dr. Miller.

Friday, July 29, 2016


It's been a quieter week this week and I have to say it is nice. Another huge flurry is coming soon just as the school year is right around the corner. We are catching our breath a bit and Ayden Jane has remembered how to entertain herself when she does not have a pack of friends around. I enjoy listening to her talk to her babies and pretend all sorts of things. She has also managed to get some extra rest which is good because I think things are really going to get crazy soon!

I have been waiting to get a summary of what Dr. Miller thinks about her recent labs, and of course, that can't come soon enough to make me happy. A couple things were off a little and likely they mean nothing but.... a mom always wonders.

It has been HOT. Not so much that the thermometer has said sky high numbers, but the humidity of late summer in South Carolina hot. It has made activity tough. We have all but stopped running, we do manage a bit of walking with the dogs but even that has been cut short. The pool is so warm that it is even more sleep provoking than anything.

Ayden Jane had art camp in the mornings this week so any chance we had to get out and moving was pretty well blown in the morning. Evenings just don't cool down quickly enough.

I was a little concerned that her weight would begin to climb as we chilled out in the air conditioning but she is still holding steady. I can hardly even write that sentence. I really do not like have to think about the weight of my 8 year old and it is such a tricky balance to help her watch it without getting worried. I am not looking forward to the coming years where it will be a bigger deal among friends...

I will say that she is doing so great as it comes to food. She is just not that hungry lately and has cut breakfast way back. She is sometimes has a morning snack but I actually had to talk her into one the other day before art camp. I really did not think she had enough in her to make it through. One day this week she had lunch at 12:30 and then turned down supper at 5:30. She did not eat again until breakfast the next morning. All her choice...

Her swim team had an ice cream party that I did not know they were going to have so I had nothing for Ayden Jane. I panicked a little because I thought she would get upset but nope. She just said she didn't care about the ice cream, she just wanted her medal and ribbons for the season. I don't know what she would have done if I had not been there but I like to think she would have said no thank you.