Top Ten Part 2- Food, Hunger and Satiety

My sweet, beautiful little baby sat quietly in her car seat as the doctor described the HUNGER that would surely come with Prader-Willi Syndrome. His advice was to restrict her calories and lock up the food in our house. Not to allow others to eat around her and be prepared for her to think she was starving but not allow her additional food. Yeah.... that sounded horrific to me as well.

The combination of hunger and slow metabolism was described as the main characteristic of PWS. Where are we now? Ayden Jane not only accepts that she has to eat differently, she can make the choices on her own. She can order her own meals at restaurants and fix her own meals at home as needed. There are no locks on our fridge or pantry and Ayden Jane is left unsupervised regularly. These are things that were unheard of by her original doctor.

The most amazing piece with Ayden Jane, however, is that she can 'read' her hunger and full signals. I do not think they are they same as a typical person, nor are the quite as reliable, but she has learned to use them to help guide her. She will stop eating and tell us she is full. This I NEVER get tired of. We can trust her when she says she is hungry, or still hungry and requests a bit more at meal time. It is not frequent and she always talks through it. Mom, I'm still a little hungry. Should I wait a few minutes and then decide if I need more food? Or, I am still hungry, would more vegetables be the best choice? I think I had plenty of meat....

Most kids with PWS will also talk, think, dream about food. I mean, hard to focus on school or anything else food on the brain. Also, they worry about food. What food will be there, what will I be able to have, how much will I be allowed.... Since Ayden Jane is not constantly hungry she does not have food always on her brain.

I can't say how Ayden Jane has reached this point but I can say she has eaten low carb, moderate protein and high fat since the start. We have also talked about food and how her body uses food extensively. She understands the science behind how she eats and how PWS effects her nutritional needs. I am also very aware that different kids with PWS just vary in this symptom like all the others. 

PWS Awareness Month Top Ten - Part 1

May is Prader-Willi Awareness month. I struggle a lot with this. On one hand, PWS is a crazy exhausting, syndrome to manage which seems more like wrestling a constant moving target with no way to know what "joy" is coming our way next; and on the other hand, Ayden Jane is pulling of quirky typical kid to most people.  I get frustrated at the chronic nature of the genetic syndrome and wish Ayden Jane could spend her days with her biggest worry being if the waves are good.

When Ayden Jane was diagnosed 10 years ago we were given the sage advice of the day. Otherwise known as the list of all the things Ayden Jane would never be able to do and the ways she would struggle/suffer.

I think this year for PWS Awareness Month I will make a sort of top 10 list of things that were predicted and nearly guaranteed and where we are with them now.

Number 1 - Scoliosis

I don't say number one as in this is the number one challenge, I am just putting it out there first because the wonderful horse barn that has loved on Ayden Jane since she was an 18 pound 18 month old riding a sweet little horse all the way until now!

Scoliosis is very common in PWS. Absolutely not a guarantee, but definitely something that has to be monitored and addressed as needed. For Ayden Jane a 24 degree curve seemed to pop out of no where when she was approaching 5 years old. We were given a few months to see what we could do with PT etc and the appointment was made to consider serial casting. Lots of PT and swimming and hippo-therapy and running and.... her curve gradually reduced until it was just minimally monitored.

Fast forward to this March. Poof. Curve jumped up again and we started night bracing and we are ramping up the running etc. It is extremely uncommon for a curve to be reduced at this age and the goal is simply to keep it from increasing and avoid surgery. Of course, Ayden Jane says don't worry she will have it back under 20 by the time we head back for our next check in September. That's how she rolls!!

Scoliosis

A few days ago we headed to Shriner's for a back check. We have been doing this for years and up until this trip Ayden Jane has mostly received good news. Her streak of strong and straight came to an end.

She did her thing with standing in the cool x-ray machine they have. It scans from the top down and the image comes up on the screen where I can see it. My heart dropped. What was once a slight curve was now a very obvious curve. I did my best not to panic Ayden Jane, saying it looked bigger but we will see what the Dr. says.

Doc came in and as I looked at the x-ray and heard the numbers. In the past 4 months her curve has gone from 11 to 26 degrees. The doctor continued to outline options and future. Night bracing. Daytime bracing. Surgery... I was trying to focus. All the while Ayden Jane was whispering in my ear. We will work hard. We can fix it. We did it before.... I put my hand on her leg and quietly told her we need to listen to the doctor right now. "Okay mom, but have FAITH. We will have everyone pray and Jesus will tell us what to do. He always does. He is faithful to take care of us. I'll just surprise the doctors again."

Now I'm not going to pretend that as she was picking out patterns and being measured for the brace that the reality of what's coming didn't bring forth a few tears. It was a roller coaster of a couple days, wavering from very worried she will be able to sleep in the brace to determined to straighten her spine so fast she won't even need it.

In the end, she is ready to fight. New running shoes were purchased and day one of running already accomplished. Contact was made to the amazing Mrs. Jen, her PT of old that helped her conquer a 23 degree curve at age 4/5. Lots of people praying that we will know just what to do and that Ayden Jane will have the strength and determination to do it. We return to pick up the night brace in 4 weeks.

In the end this song is what she is running to and the life she lives.

Confidence

Schedules and Flexibility

Ayden Jane is having a fantastic year. I may say that at the beginning of every post because I will never get tired of typing that! I got such a kick out of her the other night as processed through a change coming up.

Her schedule so far this year was to have exploratory from 8-9 and then settle in to LA until recess time, somewhere around 11:30, give or take a few minutes depending on the day. So it is along academic block, but Ayden Jane has done fine with that. Recess and lunch puts them back into the classroom and hard at work from 12:30 - 2:30 for dismissal.

The change is simply to flip math and LA so that she will have math in the morning and LA after lunch. (and yes, social studies and science are in there as well, I'm just not exactly sure when) When Ayden Jane heard of the change her initial reaction was to get a bit upset. "But I'm doing so good! Why do they have to change things?" Ha, valid.

In the end, she asked me to be sure to include some math foods at breakfast (no clue what a math food is) and keep in mind she has LA after lunch so pack accordingly.  Sure kid. I'm on it. Hopefully she will have a great day Monday and will be 100 percent on board by days end. 

Growing Up

Yep, I'm back. The full time work is done for now and I am hoping to get a bit of writing back on track.

Ayden Jane is really growing up. I'm not talking about how tall she is getting, even though that is happening too. I'm talking about her conversation. About dealing with social things at school. About caring for others. Okay, parts of her are growing up. There is plenty of her that still loves princesses, and fairies, and playing with toys, and imagination. I am thrilled those parts are content to stay young a little longer.

Growing up is challenging for any kid. Most kids have fears that other kids will laugh at them. Most kids worry that they will get picked on. Some kids worry about tests and grades. A few kids glide through pretty much unscathed but I think if we are honest, that is very few.

Now, how about heading into the wild world of growing up when you are a bit, "quirky, different, unique, weird, interesting, complicated, ...." just pick a word. These are the words that make fascinating and successful adults, but targets in school.

Over the years Ayden Jane has had her share of being made fun of. If that makes you gasp, breathe and think about it. SOME kids are just not nice. Some kids do not understand that all people are valuable. Some kids do not like others who are not the same as they are. She's had a bit of a resurgence of being on the receiving end of unkind words. We had a long chat about the stuff that is said and goes on in school. In the end though, here is what she had to say about it all:

1 - don't worry Mom. I know what he/they say is not true. God made me awesome.

2 - he/they are just mean. I think maybe they had a rough life but I'm not letting them take that out on me any more.

3 - if he/they were ever really, really, mean, C would punch them in the face. (I did NOT encourage this one. don't worry)

So there ya go. Confident, compassionate, protected. I'll take it. I mean, to hear negative things about yourself from your peers at age 11 and truly know and believe that those things are not true? To not lash out but to have at least some compassion for them?

Working on knowing when to ignore and when to speak out. - things like don't touch my desk or stay away from me. Although not nice, those you can just ignore. Calling you names, using bad language, threatening you, telling others to say bad things about you. - speak out.

Basically, Ayden Jane is a great kid. She is smart and perceptive and just when you think she is clueless, you find out she wiser than you could imagine. Some day she will be able to express all the cool stuff that rolls around in there. In the mean time, I enjoy the glimpses. 

It's Been a While

I am surprised how long it's been since I have posted. Well, sort of. In November I went back to work for a few months. Then December just full on flew out of control!

So to recap highlights, Ayden Jane managed straight A's for the first time. She was so proud of her all A's. She worked hard for it and totally earned it! As a reminder, she is in all regular mainstream classes and did not receive one bit of special education support the first quarter (more on that later)

Thanksgiving came and we had a nice family meal with all the girls. Ayden Jane does really well at these sorts of times and honestly, I just didn't worry too much about portions being a bit bigger. She was excited to bring over some keto candies that we made as a special desert and she was happy.

On to Decemeber. It was so very, very, very, beyond busy. With the rain and flooding of the fall, most activities were pushed into December and all the typical December excitement and Christmas things were mushed together with them. It was overwhelming for me and Ayden Jane loves to do EVERYTHING so we just over did it. On top of that we passed a nasty, long lasting cold around much of the family. So, much of December is a blur.

I think we FINALLY are putting together the last pieces of handling illness in Ayden Jane. She caught the nasty, snotty, cruddy, endless cold. She handled it pretty well the first few days and then, she didn't. It was like she hit a wall and in a matter of hours she went from okay to looking like a truck ran over her. Her skin color was bluish on her arms and the whitest white on her legs. She felt cold to the touch. Her energy disappeared and a headache came on strong. At that point, we went to the doc and he gave us a script for hydocortisone at Dr. Miller's recommendation. It was definitely the way to go! She turned around pretty fast and was back to fighting the cold well. After another week of fighting the cold we hit up the antibiotics since Ayden Jane has such a horrid ability to fight infection. In the end, Ayden Jane managed to kick the cold with no major complications. Woohoo.

It did, however trigger the strangest tics. She often fidgets her fingers quickly and sometimes makes a few odd facial movements when eating, but nothing life altering. Kayla nicknamed it chipmunking and with a reminder she stops Then this crazy uncontrollable ticiing came on. She could not stop it even when we pointed it out. It became nearly constant. After some thought, consulting smart folks, lots of reading, I decided to try treating it sort of like PANDAS even though I believe it was triggered by a virus rather than infection. Non of this is actually a PWS thing, it's a sucky immune system thing. Anyway, treatment is basically naproxen for inflammation and PharmaNAC because there is some evidence that it increases the effect of the naproxen. (and it can't hurt)

Sure enough,, after one dose of both Ayden Jane was more calm/movements more quiet. She told me after about 30 minutes when I went to check on her that she thought it was really helping because she could make it stop when she noticed she was doing it. That was yesterday and this morning it was still a bit better. I dosed her again and I really think it's working. So hoping she will be back to herself in time for school in 2 days.

She's one tough kid. One thing after another....

Carnitine

So before we ever went to see Dr. Miller I started to notice Ayden Jane was losing weight and looking scrawny across her shoulders. In the past this was a sign her gh was low. She was also starting to get tired earlier in the evening.

I talked through it with Ayden Jane, trying to figure out what she was feeling and she honestly amazed me. We talked about things that could be causing her reduced energy feeling. She described it as a physical tired, not a brain tired. She felt that it was not Pitolisant related because it felt different. She was excited to get lab work done to see if maybe it was gh. I was a little concerned about how labs would go in terms of gh because she was a little sick the week before and that always messes up the gh level. Sure enough, her IGF- 1 was a fair amount higher than it was 6 months ago and raising gh was off the table even though her dose has not been increased in a year and a half.

So now what? After a chat with Dr. Miller she told me I had room to play with Ayden Jane's carnitine supplements so play I did. I made some pretty risky moves and we all paid for it. Oops. Here's the scoop. Ayden Jane requires significant amounts of carnitine and always has. Over years of experience I have learned that Carnitine Fumarate provides physical energy and a dash of Acetyl carnitine seems to really improve brain energy and speed and over all 'enhance' the carnitine usage. I stopped the ACL with each Pitolisant dose increase and then slid it back in, with the exception of this last increase. ACL is apparently a natural remedy for narcolepsy so it all makes some scientific sense. (sorry, I am being tough to follow)

I decided to add back in both CF and ACL  The result was Ayden Jane's little brain was in over drive. Her poor teacher had her hands full with Ayden Jane partnered with a boy she was sweet and kind to the week before and had ZERO patience with the early part of this week. It was not pretty and none of us liked Ayden Jane on this dose. ha. I decided to ditch the ACL for now and keep the added CF and see how this rolled. It was so much better. Sweet, focused, happy Ayden Jane came back to us.

I also noted with the increased carntine that she is not hungry. As in, leaving food on the plate and saying she isn't hungry and doesn't want any more. Time will show if she increases the muscle mass back in and if I see the return of her physical endurance at swim team. Hoping for the best!

And, because I always mess way too much, I will likely try a small amount of ACL eventually.