Friday, June 23, 2017

Repetitive

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

Summer

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. 

Wednesday, May 31, 2017

Awards

I have been down the awards road with my other kids. I was happy for them with all the awards they earned, but I also knew that many of the academic ones were not earned with incredibly hard work. They were sort of awarded for being smart and doing what they were supposed to do. Congrats was nice and obviously, they could have chosen to squander their brains and behave poorly. So awards were good to acknowledge they were "good kids".

As I go to awards events now with Ayden Jane, it is different. I mean, I see lots of kids getting awards just like my big kids did. It's great that they use their gifts and make great choices. I hear the same sort of awards I have heard in the past.

I think all of this hit Gary today as he was watching Ayden Jane's final award celebration at Elementary School. I was at work, so did not attend and he sent me this text: I want to stand up and tell all these people that Ayden Jane deserves an award!!!! They have no idea whatsoever. I guess that's okay, but she's a brave little girl.

I think it sums up the struggle. On one hand I want to tell people what Ayden Jane's diagnosis says she won't be able to do. I want people to hear how much she is supposed to be struggling. I want people to have an understanding of how much work this kid puts in and how much crap she puts up with from PWS. On the other hand, I want people to see her as a typical kid and not treat her differently. I mean, that's the goal. Make PWS invisible. Trouble is, if they don't know what she fights everyday, they can't know how amazing she is.

So, I believe Ayden Jane would earn the BRAVERY award if there was one. I mean, she forges ahead fearlessly. She is happy to take on the role of being one of the first to try things and knows that others are learning from our mistakes and rewards. She boldly goes where PWS could not before.

I think she would also win the WAKE UP AND DEFY THE ODDS EVERY SINGLE DAY award. I think sometimes it's the every singe day piece that amazes me. No matter whether the day before was a hard fought, miserable,wonderful, exhausting, painful or joyful day. Whether it was a triumphant victory or miserable defeat. No matter, she starts fresh every morning with a positive attitude, ready to give every ounce of effort toward whatever she is doing that day.

That's the stuff awards are made. 

Monday, May 29, 2017

Settling In - New Normal?

I'm not entirely sure what happened to the month of May. We have been taking it a day or week at time and managing to make it to all the end of year events and squeezing in as much time on the beach and in the pool as possible. 

Ayden Jane has been at 18 mg for a month now. I feel like she is evening out. She is learning to control her new super powers and use them for good. She has days where she NEEDS a lot of big muscle movement and deep input. Craves the ocean, wants to rough house, can't sit still, talks loud and feels deeply. There is so much laughter. So much engagement with her friends and siblings (which is occasionally overwhelming) 

I measured her at 57 inches and 91 pounds. She has really grown! I dropped the rest of the ACL and so far so good. We are using very little lactoferrin, less iron, and she has no energy crashes even when we had supper an hour and half late! I asked her if she was okay or needed a snack and she said, "I'll be fine Mom. I don't crash with Pitolisant. I can eat whenever."

And of course she can't wait for summer!!!! 

The last week of school is upon us. It has been an amazing year. The difference in Ayden Jane from last November to this spring is like comparing two totally different children! She goes strong all day long, no naps, no lost hours due to needing to regroup. Awake until 8-8:30 every night and up about 6:30 in the morning. No constant night waking, which means no night waking for the Momma either :)

The arguing still exists but is much better and does not surface unless she is tired or trying to do too much. I don't mean PWS tired, I mean, typical, huge days of beach and pool and friends and water parks tired. 

It is interesting to watch her work through how to do life now that her brain works faster. She is going to need to learn to give people space, to slow down the conversation, dial back the intensity, to let things go. She has spent nine years fighting with every ounce of her being to keep up, to be a part of the conversation. She learned how to manage life that was always a tad faster than she was made to go by working so hard, concentrating hard to understand, working hard physically to keep up, relying on effort and the things she did well to get by. That same intensity and effort now can get a bit obnoxious. What should we do now? That's not how you ____. Constantly and inch a way from whoever she is playing with. Often a bit "in your face". I am hoping her good friends will have patience with her but also that they will be straight with her. and nicely help her get up to speed on these things  

Let the fun begin!

Wednesday, May 17, 2017

Disorganized and Chill

Ayden Jane seems "all jumbled" these days. We have lost glasses, papers at school, forgotten homework ... all things that would have typically sent her into a tizzy of panic. Instead, she is just not worried about any of it. It seems we have swung from overly worried about most all details to sort of 'whatever'. I am hoping this is an adjustment period which will eventually swing to a healthy concern.

On my part, I stopped the acetyl carnitine (ACL) after the last increase in Pitolisant. She was operating so fast that neither she, nor the rest of us, could hardly stand it. She was talking super fast, but still not as fast as her brain was going. The result was a lot of half sentences and gaps in what she was saying. She basically needed a translator. So, part of me wonders if now that she is fully adjusted to the Pitolisant, does she need a touch of the ACL? Questions with no clear answer.... It's the challenge of this entire thing.

I will say she was pretty entertaining last night at the soccer fields. She had to survey 50 people for a math project at school. She was a little shy at first so we did the first one together with a friend of mine. Then she went to a few moms and with me just letting them know it was a school assignment, they were glad to help. Ayden Jane was hooked. She asked her question to 50 different people and recorded their responses. No translator needed and she was so excited.

The entire interaction shows the improvement in processing speed. She was able to explain what she was doing and adapt to questions people She could just plain interact back and forth at the speed of typical conversation.

Now, if we can just get the data displayed in 3 different types of graphs... legibly... we will be in good shape.

The other crazy thing is Ayden Jane can throw and catch!! Seriously, she used to try to throw but it was ugly. The ball never really went anywhere, let alone with any speed or accuracy. We threw a ball back and forth on the trampoline the other night and she could absolutely hit a target and throw with decent velocity. Even catching was vastly improved!

Sunday, May 7, 2017

Headstrong

Headstrong is a word I would have used to describe Ayden Jane pretty much her entire life. Sort of just bulldozing her way through "challenges". So, basically, underneath all the PWS is a truly strong-willed child in every sense of the word. This basic nature has served her tremendously well.

So what if she suddenly gained confidence. Processed information faster. Was able to respond quicker. Came to her own conclusions and reasons at the speed of light. Basically, that's what we have going on. Her wonderful teacher, Mrs. M, referred to her as "even more headstrong". Hmmmm.

You see, there are wonderful things about being 'headstrong', and learning how to take on the world tactfully and with a touch of humility are things that are taught from a very young age to those wonderful, strong-willed children. Only, in Ayden Jane's case, she wasn't really capable of learning the subtleties of tact and realizing the effects of her behavior on others. Now she is.

It seems there is an adventure ahead of us. Ayden Jane is learning and processing at an amazing rate but definitely needs to catch up how to do so with respect for those older and wiser and with patience. Don't get me wrong. She is not suddenly an oppositional defiant child who is wreaking havoc. She just has an edge about her that comes across as disrespectful.

Luckily, none of my big kids will put up with her crossing lines of respect and they all get where it is coming from. They will join forces with me and teach Ayden Jane to slow down and listen. They will make her understand that sometimes the answer is no, even when you want it to be yes and can make an excellent argument.

Wish us luck!

Saturday, May 6, 2017

Who is This Kid?

I love the new Ayden Jane, but I am also a little lost because I don't really know how to read her. Or maybe I am simply waiting for something to blow up on me and can't trust it all yet. 

Her blood sugars are more stable than ever, well, as I view from the outside. I mean, she does not 'crash' anymore.

Her mental stamina is through the roof. She took the state testing this past week and the first day included writing an essay. The kid started at 8:30 and did not finish until after 2!! Her teacher said she honestly worked the entire time. She came home happy as a lark and bouncy - just like my typical kids would.

So where's my issue? 

I guess, previously, I could read AJ. I mean, I could look at her and tell you if she needed more gh. Her behavior told me if ACL was too high or b12 too low. I could manipulate her diet to manage blood sugar. Now I have NO idea what I am looking for. Where are my "boundaries" or ranges of .... well, everything? How do I know what's going on in there?

Soooo, I guess I am living in the land of is it all too good to be true? 

I'm curious if she still has a black hole for carntine. Wonder about the gh bump... and pretty much everything else. I mean, it's like the kid has a new metabolism. She is more typical as it comes to food. She has always wavered between forgetting to eat and maintaining weight to gaining a bit too much. I always managed it from the outside by talking her through it.

I'd say the past month really, I have had very little to do with her food. I don't remind her if she skips a snack or a meal comes quite late she does not miss a beat. She has days where she is more hungry and eats extra, but her weight is remarkably stable. We will head out the door with no plan for food and she will tell me not to worry about it. She will be fine. Seriously?

This week has been brutal as far as schedule ect. Long days testing at school. Late nights at the HS with Mckenna in playoffs and chorus concerts. I am barely functioning and she rocking it. Yes, there are lots of questions about peoples expressions and social interactions (apparently she can now 'read' faces and body language but is still learning how she should respond). Yes she has some sensory needs which shows me the neurology is continuing to connect. She no longer gets stuck on topics or questions... and can "take a subtle hint."

I guess, my mind goes to - if there is this big a shift in .... metabolism? something... then how can all that I am giving her not need to be adjusted? I have been managing and tweaking this stuff for 9 years and suddenly have no idea what I'm doing.  I mean, I wouldn't be pumping all this stuff in my other kids. Is there anything I give her that can be over done? I'm thinking labs are in our future, even it its just for Momma's sake. 

Wednesday, May 3, 2017

Whew

That's a big sigh of relief. Testing days 1 and 2 are over! The first day Ayden Jane worked on her test from 8:30 in the morning until after 2! Obviously, I don't know anything about how she did, but I know she tried really hard. She showed her true tortoise self. Not at all phased by those around her, just plodding along doing her thing.

After the test I took her to the beach. Wow did she need to hit the waves! Fresh air and sunshine. Salt water and good waves. It was perfect. She even found a couple of boys who were boogie boarding and she joined in. The ocean is the best reset button I have and I knew she had to get up the next morning and start the entire thing again!

Day 2 she said she finished shortly after lunch. She told me she was done, but was not done going over all her answers before lunch so she had to have silent lunch. She didn't mind too much but she was very glad to report she was finished in time for recess!

Today, she had swim team after the test and then we had a playoff game of Mckenna's to attend. She has been so funny this season. She is totally into the games. She knows all the players names, talks about how great they did, and cheers during the game. (well, as she runs by while playing hide and seek or whatever) She is so proud of Mckenna and is loving the season. Tonight they won a hard fought playoff game and Ayden Jane was complaining about the bad calls during the game and reliving highlights. She not always right in her recount but her enthusiasm is unmatched.

She is so glad Mckenna's team won tonight so there is another game on Friday!

Monday, May 1, 2017

Standardized Testing - Sheesh

Time is flying by. Ayden Jane is loving Pitolisant and her fast brain. She is still getting used to her new super powers so will only continue to improve her skills but she is learning at an amazing rate.

Tomorrow is the SC Ready test. It is South Carolina's standardized testing. Being in 3rd grade it is Ayden Jane's first exposure to such a testing situation. She is a nervous wreck. I think hearing about it and all the hype surrounding it, but never actually having had the experience is hard. It's hard for all of them!

She has been working on something called a text dependent analysis (TDA) for the past few weeks. It is hard but she absolutely gets it. Now it's just time to pray she does not freeze up. Typically she reads the story and likes to talk about it. Then she can organize her thoughts enough to get it all put together and written down. Tomorrow there will be no talking through and if she gets 'stuck' it's going to be ugly.

As she worked through things last night she asked me if she forgot to put in comma's or something if they would still grade her paper. Sounds crazy doesn't it. Here is how it works. Teacher reminds the students (remember, 3rd grade) that they need to use their neat handwriting. She tells them if whoever is scoring the paper cannot read their handwriting they will simply not score it. Yep... Ayden Jane took the leap that if she misses anything, as in anything less than perfection and her paper will be thrown away. Then she asks me if it is okay if she gets a couple wrong. Ummm yes, dear. Just do you best and show what you know. There will be some questions you do not know. Just take your time and be sure to get the ones you know right.

Her teacher is going to give the kids 'breakfast' before the test. It's a great idea for 99 percent of the class... guess who it is just adding more excitement/stress to.

All in all I'd say Ayden Jane is half nervous and half excited. Yep. Excited. I mean... it's an opportunity to work hard and accomplish a goal. That's right up her alley. Hopefully she will still be exited the 2nd and 3rd days!

Sunday, April 23, 2017

Important Event In My Life

I was looking at the news letter Ayden Jane's teacher sends to us at the beginning of the week so we know what she is studying or doing that week. One of the things mentioned was a diary entry of sorts about an important event in her life.

I asked Ayden Jane if she had any important events. She said not really. Maybe going to a big soccer game of Mckenna's? I told her I could think of a lot of important events in her life but most of them she would not remember. Things like getting the ng tube out and eating all on her own so she could come home from the hospital. We talked through a few ideas. 

Mckenna looked at me as Ayden Jane was talking. We were both very intrigued by how unphased Ayden Jane was about things that we understand to be HUGE. I mean... dare I say Pitolisant? There is a cluelessness about her in regard to what she has accomplished. 

I teased her and suggested maybe the day I found and made a chocolate cake for her birthday was important. She told me, "mom, that's not important. That's just food." Now there are some unique words to come out of a kid with PWS.

In the end, I really have no idea what she will pick. Likely, it will be something unrelated to PWS. Maybe, somewhere in that brain of hers, she doesn't want her important thing to have to involve PWS. I mean, she doesn't really want to be that kid with a rare genetic disorder. She just wants to be Ayden Jane - the nice kid who wants the best for everyone. 

Obviously, I will want to read it when she is done!! I am really curious about what goes on inside that noggin of hers. Maybe getting her to write things like this is a way to find a window into her thoughts. I also need to learn to talk less and ask better questions. Patiently listen and not redirect or correct details or fill in pauses. 

Increase to 18

Ayden Jane had been asking to increase the Pitolisant (Wakix) dose to 18 mg. I'm not sure if she felt she needed the increase or if she was just being her goal oriented self. Either way, I wanted to wait until the trip was coming to a close and we were home with nothing major to do.

So, on Thursday, our last day of travels I gave Ayden Jane the increased dose. All was well on Thursday. Friday, especially in the morning she was tired. She was spacey. She would say she wanted to do _____, but then would change her mind because she was too hot, or too tired, or just didn't want to. She napped in the morning. Then we went to the beach after lunch.

She did not complain of headache but it was clear a lot was going on in there. It looks on the outside like she is super spacey. She is slow and moves like she is tired but she's not sleepy. When I took her to the beach and after swimming a bit she just wanted to sit on the beach. Sit and watch people and do nothing. At the same time completely peaceful and totally NOT content. It's hard to explain. If you push her to do anything she gets very argumentative and says her head hurts. When I asked her about it she just said her brain was thinking a lot but she didn't know what it was thinking about.

Saturday came and I was hoping for a big improvement. She started asking to take the dogs for a walk. She could not get it together and finally decided she did not want to take a walk and went to her room. She fell asleep... again. When she woke from that nap, she looked more ready for activity, finally. It's hard to explain.

We went back out to the beach and this time she played hard, sat in the sand a bit but was chatty and drawing things.... Then swam some more. Finally, we went over to the pool and she found a couple friends to play with. They are sweet giggly girls and Ayden Jane still a step slow, but they were patient and Ayden Jane had fun.

Finally, this morning (day 4) she is bouncy and quick and playing jokes on me. No nap, no slow down. We walked the dogs and will go for a dip in the pool later but I think she is really ready for school. Yay. It's going to be a rude awakening tomorrow morning. Well, for me and Mckenna for sure. It will be a long day for Ayden Jane as she will have school, swim team and Mckenna's soccer game to attend tomorrow night.

Saturday, April 22, 2017

Faith of a Child

At the end of our long adventure to TX, Ayden Jane was excited to stop and see her friend E. I thought we would be able to stop by Wednesday evening for a bit and then head home but as I redid the math ... It wasn't happening. We had to decide whether to go home and abandon our last stop or commit to spending the night in yet another hotel so we see our friends the next day.

I wasn't too worried about finding a hotel. I figured it was a Wednesday and there was not anything major going on in the area. Well, those facts were true, but that did not mean there were rooms. The first place I stopped was booked. The guy at the desk was very nice and sent us to another hotel which I would not have found if he had not told me. It looked just as busy and there was a sign that the desk person would be back soon... I went out and made some calls but everything was booked. Well, that's not true, there were some places I drove by that likely had a room but I didn't think they were good places for Ayden Jane and I to stay.

I wasn't worried in that there were plenty of other exits with hotels... I was just tired and wanted to be done. Ayden Jane told me "don't worry Mom, God will take care of us. You know, just like he put that gas station right where we needed it when you were almost out of gas. It's really cool how God takes care of even the little things."

So, we went back in and the lady at the desk was finishing up with someone who had been waiting with us earlier. I asked the question, any rooms left for tonight? She said there were. She chatted with us for a minute as well. She came up with a discount. Then another. Before the evening was out Ayden Jane's story, her faith, was shared a little and hopefully the kind, night shift, desk person was blessed.

It was great for me. I was thinking a lot on the drive. Probably too much. Some doubts were creeping in about a few things (not necessarily Ayden Jane related things) In the end though, a cute little 9 year old reminded me - not to worry. God is in control of even the little details. Doesn't mean He will make things easy all the time, but He does have a plan and I need to trust that.

Saturday, April 15, 2017

Traveling

An entire week has gone by. Wow. It has been an unbelievably busy past couple of weeks. Work busy for both Gary and I. School busy for Ayden Jane and Mckenna. Kayla - well, sometimes you can barely tell she lives at home so is gone and doing so much.

Spring Break has finally arrived we and managed to pull out of Pawleys on Thursday evening after a full day of school and a quick trip to the beach and pool to help with the wiggles. I think I packed my stuff and Ayden Jane's medicines, supplements and food in about 20 minutes total!

We traveled all night long, which although exhausting, is a very efficient way to go. Ayden Jane did awesome. Kayla made a pnut butter sandwich for her with cashew bread before we left so when dinner time rolled around Ayden Jane could have that a some snacks from the cooler. The rest of us planned to wait and stop for a late supper after we had a few hours of travel under out belts.

All went according to plan and after our 'late supper' and bedtime snack for Ayden Jane she watched a bit of a movie and went to sleep. We were hoping she would fall asleep in South Carolina and wake up nearly to Texas.

Somewhere around 1:30 or so AM we stopped for gas (and coffee). Ayden Jane woke up and declared she could not go back to sleep. Now, before Pitolisant, no matter how sure she was she couldn't sleep, just put her in the car and she was out like a light. ha. No longer. She watched some more movie and read a little and finally did go back to sleep. She was great though. No whining or complaining. Just awake.

Morning came and she was wide awake and chatty. We talked (okay, she talked and I listened) for about an hour. She was just so excited to be on the trip and to see her grandpa, aunts and uncles and cousins. The excitement was escalated because she hadn't seen them since she was 3 and honestly couldn't even figure out who she was going to see.  About 6:30 her belly time, we stopped for breakfast.

The rest of the way she was still great. She always wants to know how much longer, but that is nothing GPS can't handle. Traveling with a PWS kiddo, typically brings out the anxiety in them. So many unknowns. Typically, Ayden Jane travels well with me, but I do have to answer a lot of questions about where, when, how, who... well, everything. I have always just accepted that and made sure to let her know the plan, and let her be a part of any changes to the plan. This is a whole new level of go with the flow. I mean, there are 5 of us and a HUGE family is a part of the plan. We all know how flexible you need to be to handle that! I don't know that she could have enjoyed this trip nearly as much before Pitolisant. 

Saturday, April 8, 2017

Big Dreams

It's no surprise that we have always allowed ourselves to dream big for Ayden Jane. Maybe in a way, that first endocrinologist who told me - my problem was I needed to learn to accept that Ayden Jane was a special needs child and stop trying so hard - did me a favor. I mean, Ayden Jane gets her "just tell me I can't" attitude from genetics other than the PWS region.

So we went on our merry way helping Ayden Jane achieve as close to her peers as possible. She learned to read... with a little extra effort. She kept up in school, with the support of awesome teachers who recognized she could learn but sometimes took an alternate route to solving math problems. Ayden Jane would talk of going to college like her siblings and I imagined she would, but likely community college where she could still be home with support. She talked of becoming a veterinarian (like most girls her age) and I could totally see her as a vet assistant.

You get the picture. All wonderful success that could lead to her being able to be happy, productive and independent eventually. Or at least, independent enough that she would not be a heavy burden to her siblings one day. You know, needing one of them to check in with her and help her out at times, but not the 24/7 care that we were told would be necessary. That was a dream nearly unthinkable for kids/families with PWS even 5-10 years ago.

Those were the dreams we HAD for Ayden Jane.

I catch myself now, with Pitolisant, dreaming all new dreams for her. Although the dreams of the past were wonderful, I now catch myself dreaming, expecting that she will go away to college if she chooses. That she will be anything she wants. The limits I put on my dreams for her - not that she ever had any limits for herself - have been lifted.

Why the change? She operates so much closer to the speed of life now. The pause, the step behind she always was, is gone. It will take time to learn how to use her faster brain and there is still catch up to happen, but even others are noticing. We have said goodbye and good riddens to "the stand and stare." She is engaged and a part of all that is going on around her and not just an outside observer gleaning all she can but unable to do so fast enough to truly be a part.

Wednesday, April 5, 2017

A Message From Ayden Jane

Mom asked me why i like Pitolisant and if there is anything bad about it.

I think it is great. It helps me do better in school. I can understand things better, figure stuff out, remember more, and can work a longer time. School is easier now.

I can tell that I can stay awake longer in school and keep my concentration and I don't fall asleep as early at night as I used to do. I have enough time to get all my homework done before bed and don't have to do it in the morning before school. Homework is easy now. I can play longer because I have time and I also have more energy.

No problems with Pitolisant other than the first day or two after I start an new dose. I was tired but slept really well. Sleep right away changes. At first sometimes there is a headache with the tired and I don't want to do anything but after that it is all good.

I feel happy and laugh a lot now.


Tuesday, April 4, 2017

Sensory Seeking

Over this past weekend, I noticed Ayden Jane having some 'sensory needs'. This language seems normal to me now but may sound strange if you haven't lived a life of occupational therapy. Basically, from the time Ayden Jane was little and we started things like B12 and carnitine and fed her brain with good fats her little neurological system has been growing. That growth is often not smooth however, and needs a little help to sort of sink in and/or organize all the information it is getting.

This sort of thing can show up the form of anxiety, emotional instability, rough housing, itching, complaining of clothes not being comfortable, not wanting to wear shoes, things being too loud or too bright, using entirely too many words to say something simple ... When Ayden Jane was really little we did a brushing protocol and joint compressions. I remember the first time Mrs Jen did joint compressions and Ayden Jane relaxed and was quickly peaceful. She explained it then as sort of helping the messages coming in connect to the brain and become filed correctly. Like there can be a sort of back up of input that can't figure out where to go and instead makes her feel overwhelmed and disorganized. The system of nerves just not moving information effectively and causing a traffic jam.

Pitolisant has caused some of these old familiar things to resurface. I think it is a good thing, as it shows there is a lot of neurology firing these days, but it does feel uncomfortable at times for Ayden Jane. This past weekend we dealt with it by sending her with a friend to Savannah's playground, taking a long walk, bouncing on the trampoline .... That was all Saturday. Sunday we hit the ocean. Nothing seems to calm her internal storm like being pounded by the waves.

Monday, when Ayden Jane woke up she was calm, and focused and happy. She had her reading MAP test and knocked it out of the park. By Monday night, however, the storm was again brewing. I think it was fed by the excitement of how well she did, the desire to repeat the success on Tuesday with part 2 and a huge field trip the following day. So much to be excited about. So much to process.

Tuesday morning came and there were a couple of shirt changes and a small pile left behind of shirts that were no longer acceptable. Shoes had to be put on a couple times because they 'weren't right'. She got upset over something little that typically would not be a concern.The palm scratching, using so many words to tell me things I did not even need to know.

She had part 2 of her standardized testing but before it even began she went to her "problem solver" at school because there was no way she could stay in her current shirt for MAP testing and needed to call home. My awesome friend who teaches there proceeded to take her to the school store and find her a new, nice, large, soft t-shirt which was acceptable. Whew. She did not do poorly on her testing but did not blow it away like yesterday. Well, other than it took her half the time it took her last time! 

Anyway, tonight she bounced on the trampoline, wrestled with Gary and is heading off to the High School soccer game to play with the pack of younger siblings. Wishing her teacher luck on their trip to the Charleston Aquarium and touring downtown tomorrow! They will be gone 10 hours! Anyway, there is definitely an awakening that is to some extent uncomfortable.

Sunday, April 2, 2017

Great Reports

With the weekend I get a chance to collect some 'reports' from school for the past week, spend some time with Ayden Jane and get a feel for where we are in this process.

The changes for this week included reducing Acetyl Carnitine (ACL) and adjusting to an increase in gh. Pitolisant was staying the same at 13.5 mg. The little by little tweaking of things can be very stressful. Basically, I take in the information I get from those who see her all week and combine it with what I see. I take a deep breath, pray over each choice and then I check with Dr. Miller to make sure I'm not doing anything crazy.

I let a change go at least a week and assess where we are. It sounds simple enough but it is my kid I'm messing with so the stakes feel really high. I am thrilled to have teachers watching over her at school that know her well, love her and pray for her regularly. It makes it so much easier to turn her over every morning as I pray for the best. This week the reports were fantastic. Here are some highlights.

Please remember that I am simply sharing our experience. I wish I had the power to make this the experience for all kids with PWS but I cannot.

Anxiousness- Compared to previous weeks, her anxiousness has definitely decreased. The only time she got anxious was when she had to stop her S.S. Chpt. 7 Test on Thursday (after an hour). Once I explained she would complete it on Friday all was good!

Focus- Doesn't seem to be as scattered. AJ has struggled somewhat to switch gears when we transition from one subject area or task to another. In the past week, she has been able to transition better and appears to be attending to the lessons better.

Speed of Work Completion- a little faster depending on what the task is requiring her to do. For example in Geometry she has had to cut & paste shapes when sorting by attributes. This has been a lengthy process, but one she perseveres through.
Daily Language Review Quiz definitely went faster this past week. She continues to work even when most students would throw their hands up & throw the towel in.

Processing & Problem Solving- definitely taking in the concepts she's being taught at a faster rate & amazing to see how deeply she understands the concepts. For example in Geometry, the students were asked to draw specific shapes based off of there attributes. There were a total of 8 tasks. ONLY 4 tasks could be drawn the remaining 4 were impossible, but the children had to write why the shapes could not be drawn. AJ was clear in all 4 written responses explaining exactly why this was impossible. *I am attaching the task & will be glad to send home AJ's work so you can see her explanations.

Socially- I've noticed AJ engaging more with her peers. 
Classroom- she will approach students to have a conversation😊
Lunch- she is talking and laughing more than I've ever seen her 😊

Whew. Thankful for weeks like this where God shows up and lets me know to just keep going and follow Him. We are not done yet, but plan to stay right where we are for now. 

Monday, March 27, 2017

Busy Weekend

So last I wrote was 5 days ago. It has been a busy few days.

Friday was quite the test for Pitolisant. Ayden Jane had a full day of school, swim team practice, then we ran home to pack up and head to Columbia. I figured she would take a good nap in the car before the 8:30 soccer game of Mckenna's we were heading to. I figured wrong. She played her ipad, read her box car kids book and generally entertained herself the entire trip.

Now I thought I was in for a rough night at the game. I mean, Friday night - game starting at 8:30? How could it end well. Again I was totally wrong. Ayden Jane played her little heart out and ran around all the way 'til 10:00 pm when the game ended! It is such a new world we live in.

So Saturday brought 2 more soccer games and the trip home. She did well. A little tired and cranky, but so was I. Mckenna played awesome and the second half of the second game, Ayden Jane was tired of running around and it was getting hot. She came and sat with me and totally got into the game. It was great. So basically, 3 soccer games in less than 20 hours and all was well.

Sunday I thought Ayden Jane would be tired but she woke up full steam ahead. We went to church, then hit the beach for a while. She had a wonderful time riding on and playing in the waves. There is something so calming to her about being knocked around in the ocean. Next up was Awana - a kids program at church - and lastly we took the dogs for a walk.

She was up and ready for school with no problem this morning. It's awesome.

Nitty gritty wise, this is where we are. Ayden Jane is currently on a dose of 13.5 mg of Pitolisant. I am planning to maintain this dose for another week at least. At the same time, we started her increase in gh that labs showed back in early February she is due for. The other thing I am working on is reducing the amount of Acetyl Carnitine (ACL) I am giving her.

ACL is brain activating. It was very helpful in the past with keeping AJ alert in school. It is a nutritional supplement and not a drug, but is one that dosing can be tricky. For Ayden Jane, too little and I could not keep her carnitine levels decent and Ayden Jane had trouble focusing on things. The tiniest bit too much and AJ was anxious and ornery and none of us had any fun. I was wiling to put up with a little panicky kiddo if it meant she could focus, but there was a line.

With all that seems to be going on in Ayden Jane I am cutting back the ACL in half (after having checked with Dr. M, of course) Whew. Lots of tweaks and adjustments. Ayden Jane is handling it all like a pro. 

Wednesday, March 22, 2017

The Pattern Continues

Gary picked up Ayden Jane from school and she was tired. Not grumpy or irrational or shutting down tired. My PWS friends will know exactly what I am talking about. She was just, kid who had 2 super busy days back to back tired. She knew she was tired because she told Gary she was too tired to go to swim team. She had a snack and watched a cartoon or two while playing with some old friends, her mini pop beads. It was something she used to love to do when she was little. Sort of a calming thing she did when her little sensory system was overwhelmed. I have not seen her do that in years.

After a bit she began to get a little ornery which is common when she is tired. What was new was that she stopped arguing and just slipped off and went to her room to take a nap. I say that is new because all too often in the past, she will pick and push buttons ... eventually our patience wears thin and we fuss at her. The ensuing events end up with her going to bed crying and not having a clue how it all happened. 

She slept for a pretty long time and then woke up, happy as a clam. She had some supper and then happily completed her homework. There is an ease about her. Gary and I were talking and it as if we see glimpses of what Ayden Jane would be like without PWS. Let's just say she would be a handful in a whole other way!!

I think things are evening out but it will continue to be a process. She feels so good at times she over does it and feels it the next day. She is taking in so much she needs a little help organizing and integrating it all. I am hoping the pattern of super powers / worn out / super powers.... will even out  over time and Ayden Jane. Hopefully in a a way that is super powers at all times!

Tuesday, March 21, 2017

What a Difference a Day Makes

Yesterday compared to today was a big change. If it keeps going at this rate it's going to get wild around here.

Yesterday, Mrs. M said Ayden Jane was a little sluggish. I saw the same at home. Not full on tired, not falling asleep, just processing a little slow and struggling a little. I don't know how else to describe it. She did a lot, school, swim team, shopped, homework, walked dogs... She wanted to do all of it but at the same time you could see the effort in it all. There was some anxiety, which always comes with tired.

Today? I do not know how school went but since I have been with her - starting at 4 ish - she has been on FIRE. She did an awesome job picking out new glasses. She was cracking up the ladies there helping her. She was decisive about what she liked and did not like. She was delightful.

After glasses we joined up with Gary and Mckenna and grabbed a bite to eat. She was so animated. So much laughter. So much joy. All of that and she was on her way to supper so you would think she would be slowing down.

After a burger and veggies we headed home to knock out homework, walk the dogs, wash the dogs and take a shower. There is NO WAY all of those things could possibly have been done in one night before Pitolisant. Not only did she accomplish the list of things she wanted to, she did it all with ease. It is currently 9:00 and she is still firing quickly.  As we were nearly home from our walk Ayden Jane stopped to tie her shoe. Being the good mom I am, I just kept walking and figured she'd catch up and knew her way home anyway. I got down the road a bit and I heard footsteps. They were the footsteps of Ayden Jane running. I didn't even turn around because I loved the sound. It was different. Her steps were quicker and lighter and even and.... sounded like a kid who ran easily. After she caught up she said, "mom, Pitolisant makes me way faster. Hey, can we start running this week? I want to do that bouncy race coming up! I bet I can run the whole way with no problem.... "

I am honestly a little nervous about tomorrow. I think that sometimes, on P, she has felt so great one day that she over does it and the next day she is tired. Or maybe she is sort of over energized that she doesn't sleep well? I don't know, it's just a pattern that has seemed to surface. I am hoping that it will all even out over time. I have a feeling my quick increase is partly to blame. I think Pitolisant needed to really settle in and she needed more time to adjust at each dose. I guess that is part of what we are doing. Looking for benefits and side effects and knowledge for dosing.

What I do know for sure is that today was great. The hope that days like today will string together and become the new normal is amazing. Of course, only time will tell... patience, patience. Prayers of thanksgiving tonight for a great day and a glimpse of what may be to come. Prayers for little Ayden Jane as she walks through this all and rides the ups and downs of all sorts of things going on inside.

Sunday, March 19, 2017

Ahh the Weekend

Weekends are great, of course, but there is even more reason for me to love a weekend these days. I get spend time around Ayden Jane. I see first hand how she is doing. I try to pick out the little nuances of what Pitolisant may or may not be doing for her.

Yesterday we started our day with a dog walk and errands. Dog walks serve a dual purpose as they are obviously exercise, but even more than that they are wide open, one on one, talk time. We usually walk about 45 minutes or so and Ayden Jane talks the entire time. Some days it's boring, making up stuff to talk about rambling. Lots of days its her time to really work through the mysteries of life. She just seems to talk through things better when she is on the move and other distracts like electronic and family fade away.

We headed out and I had in my mind the challenges she has been having expressing all that is going on in that little head of hers. One thing I learned quickly is that she is not short on words! She rattled on and was enjoyable. She is actually really getting into competition. She is following Mckenna's soccer team and tells me who plays where and how they are the best at that position. Some is pretty spot on and some is entirely made up. She is definitely the teams biggest fan though, and has obviously really watched the past couple of games. I have had basketball on a fair amount and she loves it too. She has reasons for the different teams she picks in different games. No, I'm not saying it has anything to do with Pitolisant, it's just fun to chat with her and have her really understand the game.

After the walk we made pizza for her and did a bit of shopping. Finally, we took a bike ride to the library to exchange books. I notice her energy is much more even. Supper time has been pushed back a bit to more like 6:00, which is good because bed time has been pushed back a fair amount. (she used to go to bed shortly after 6)

It is a little comical because she gets bored sometimes with the added energy and hours in the day to fill. She has not had much practice with time to kill and energy to just do something for fun to entertain herself. She had been pushing to get things done in her awake/alert moments because she did not have enough time.

All in all, she is doing great and I am super excited to see how this week goes. I am curious what mrs. m will see at school. I am curious to see how homework goes. I am curious to see how swim team goes. I am curious to see if any weight or body composition changes begin. I am curious to see if the improvements in temperature regulation continue. I am curious to see if she passes her multiplication timed tests haha. Guess we will see...

Saturday, March 18, 2017

How Did I Get Here

I want to share this evening some things that are on my heart. Sometimes I stop and wonder how I got here. I mean, how, or even why, did God bless us with sweet Ayden Jane. At 42 I certainly was not looking to begin such an adventure. I was not looking for an entire new purpose in life. I was not looking to learn crazy amounts of science. I was not looking to be a part of an amazing group of brave families looking to forge new paths for a rare genetic disorder. I was not so smart, or brave or even rooted in my faith to the extend I would need to become.

I thought I was a pretty good parent. I thought I was a pretty good teacher. I thought we had a pretty great life with our 3 awesome kids. Okay, we did have a great life with 3 awesome kids. (and still do)

Ayden Jane entered our fold with a bang. Somewhere on this blog are recounts of the first months and the shock that ensued. That's not really what's on my mind.

I don't know why Ayden Jane is doing so awesome. Maybe that surprises people, thinking that it's all the time and effort and reading I have put in. That sounds all pretty, but reality is, there are lots of mom's that have poured as much and more into their children, and their children are just not where their heart aches for them to be. I am not enough to explain why Ayden Jane is all that she is.

I guess trying pitolisant has my mind racing. I know all the things we have done over the years and been one of the first to do. I know we turned away from what the very doctors who were supposed to know best told us to do, and instead, pushed into new territory.  I know the incredible minds that have brought information to us and am so grateful that God has given me a brain big enough to understand half of it. (maybe more than half but definitely not all of it!) I know I have met people with unbelievable passion to help, and use their gifts to do so. Their commitment and passion is amazing.

I know Ayden Jane has effected, shaped, molded, changed my other kids forever - I hope positively in the end, although it was hard on them. I will always wonder what they would be like if there had not been an Ayden Jane.

I know I am not the same mom or teacher or wife or person I was before all of this. I'm not saying I was better or worse, just different.

I used to wonder what Ayden Jane would be like if she didn't have Prader-Willi Syndrome. Would she have been athletic, or musical, or artistic? Would she rather play in the dirt or read books? Would she be quiet and shy or outgoing? The best thing this Mothers' Day is that I don't wonder anymore. With the addition of Pitolisant, I feel like I am seeing just who Ayden Jane is without PWS. Sure, there are parts that will always remind us that she has PWS, but none of those parts will stop her. As Dr Miller has made me promise, we will save her a seat front and center at Ayden Jane's college graduation.

I know that many, many times I was in way over my head which led me to prayer, which led me to trust a still small voice inside my heart. God has guided our paths and woven an amazing story. I still have no idea where He is leading all of this. I don't know if Pitolisant is the 'final' answer to all that Ayden Jane needs. I don't know how her story will end, but I do know I will faithfully continue to leave her fully in His hands. 

Thursday, March 16, 2017

Temperature Regulation

For those of you who have followed Ayden Jane over the years, you have come to know that the child has some strange temperature regulation issues. She will run around outside with no coat and no shoes when everyone else is totally bundled. It's a known thing and I'm sure most people think she does not even own a coat (or that her parents are slack)

I distinctly remember when I fought with her and made her wear a coat in the past. We went on a dog walk. It was after dark and I layered up because it was cold. Ayden Jane did not want to wear a coat but I convinced her. We made it down the road a ways and she asked to take it off because she was hot. I didn't believe her at first but sure enough her hands were even warm. I touched her back and she was so warm she was sweating. I listened better after that.

Of course, there were times I was pretty sure she was cold but just wanted to prove her point. All in all though, I would be sure she was cold and then feel her arms. Warm. If she wore long sleeves or long pants to school she would overheat in the classroom.

I think the odd part to me is that she does not particularly overheat in summer. It seems to be more related to clothing than actual temperature.

So, this evening when it was upper 40s, Ayden Jane surprised us all. She just said it's kind of cold out so I'm going to wear pants and a coat. Oh my. Once the shock wore off I just agreed that it was a good idea and didn't make a fuss of it. She was great at the game and ran around with a couple other younger siblings and never once took off her coat or even looked flushed or over heated. When we got home she came in and was playing a game on the ipad. She sat down on the sofa and didn't even take the coast off. Added layers in a warm house....

So, why the change? Well guess what one of the area's regulated, at least in part, by H3r.  Yep. It has to be pitolisant.

Wednesday, March 15, 2017

Smoothing Out

Last post was 2 days ago and things were not pretty. Glad to be able to say that today was a great day.

There was improvement yesterday, but Ayden Jane was still a little on edge. Sort of like, she was okay but we had to tread lightly to keep things that way. She did okay at school but had a small meltdown that she was able to pull back together. There was not a lot of laughter. We could not tease her as she would get upset. She was just not fun.

I talked through things with Gary. We discussed the rising dose, how it seemed to correlate with what we were seeing, that it would likely work it's way out but ... how long? In the end, he was the voice of reason and reminded me it was not a race. That just because I had a target goal in my head did not even make the target correct. We decided to go back to 13.5 mg and see how things settled in. I knew it was the right choice but for some reason my crazy brain only wants to push forward.

So how did it go? She had a great day at school. She did awesome at swim team. She was funny and quick minded. She laughed. She laughed a lot.

We will stick with this dose for a while - or until I get impatient, or until Ayden Jane asks to go up, or until I learn something that says otherwise.

All I know for sure is that today was a good day. Once we get to string together a bunch of good days, maybe I can begin to piece together what sorts of things Pitolisant is helping. Hopefully, then I can piece together how or why these things are improving and how they are connected with Pitolisant.

The question of dosing is the hardest. Not that I am scared we are hurting her at any particular dose. We have that piece covered. Just that there are not many who have tried the drug and even fewer who have PWS and are trying the drug. I'm talking single digits. So optimising dose to gain the benefits we are looking for but with no negatives. There just aren't guidelines yet... much of it is determined by what we see in Ayden Jane and what she reports to us.

It's all and adventure.

Monday, March 13, 2017

Adjustments

Today was not a good day. Ayden Jane woke up this morning saying she had a headache. I let her stay home and she hung out with Noah and Kayla. When I got home, the looks from Kayla and Noah told me that she not been easy to deal with all day.

A little PWS background is that they often struggle with emotional stability. This can mean a lot of things but in Ayden Jane, it means she can go from happy, smooth sailing to a melted down puddle of mush in about 3 second. Typically, she is this way when she is over tired or I am messing with her supplements. Apparently, she gets this way when adjusting to doses of Pitolisant as well. Good to know.

Basically, she was tolerating, in fact asking, to go up on the Pitolisant each week. I think it was all going well and she had no side effects other than that first couple days of a rough morning. Now pneumonia is gone, the steroids and antibiotics have worn off and she should be getting back to her old self. But there is something going on.

So, she woke up with a headache and couldn't go to school. The other odd thing was this evening when she was cold - inside the house! The child that is never cold. Well, never feels cold. The child that will turn blue and refuse a coat. She chose to put on a coat!! Her skin was chilly to the touch. I know that sounds like a strange thing to say but in the past, she looked cold but her skin was always warm to the touch. It was strange, but actually normal.

She is also having a hard time explaining what she is thinking. I believe she is just thinking faster than her mouth can keep up right now. It has happened in the past. Sort of like she has a complex thought and has made connections but she can't find the words to explain those connections. It is irritating for us and frustrating for her. In the end, I tend to get impatient and she tends to cry. Yeah... it's a lot of fun.

Anyway, I am guessing that our quick rise in dosing was not a great idea. 

Saturday, March 11, 2017

Party Weekend

Ayden Jane is following up her insanely busy week with a birthday party on Saturday and one on Sunday. This girl loves a party.

Today we bumped her Wakix up to 18mg. In theory she should be wide awake and going full steam. In reality, she was tired and went to bed early. This is wear life makes the "control" part of the experiment difficult. I mean, just yesterday was her last day of medication for the pneumonia. She (we) went super hard this week with school, after school activities, make-up work, 2 high school soccer games, out to supper, Noah home on spring break.... I know I'm tired!! I took myself a nice little nap.

I will use a "situation" as an example today of a challenge Ayden Jane has, particularly when she is tired. I told her she had a follow up at the pediatrician on Monday. She asked some questions about why and proceeded to get very upset. What she was saying and her reaction did not match. I gave her a minute to calm down and then pushed the issue. Finally, she said, she didn't want to miss any more school because she is just catching up. I told her we could change the appointment and do it after school and poof. She was back to excited to walk the dogs and get on with our day!

It is so aggravating and I am not sure where the glitch is. It is like she does not want to say the 'real' underlying issue so she makes up something else as a reason for ... oh I don't even know. This sort of talking around issues without giving you the real meat gets frustrating for everyone. I need to write a few down.

I can say for certain that it is something that happens much more often when she is tired. Then again, she would find a reason to argue with a brick wall when she is tired too.

As for the birthday party. I dropped her off. I have no idea what she ate but I trusted her. She was at a party of a boy in her class and most of her class was there. I was happy that it was a between lunch and supper party with popcorn and fruit for snacks. She brought some dark chocolate for cake time. When I got there she was finishing up some fruit and headed out to play on the zipline in the back yard. It was nice because it was one of those moments where you could not pick her out as any different from her peers.

I am looking forward to Pitolisant making those sorts of moments the norm. 

Thursday, March 9, 2017

Sorting Out What's What

Sorting out cause for effect. It is not so straight forward. Here is what I know.

Past: Ayden Jane would get sick and recovery was a long slow process.
Now: Ayden Jane recovered amazingly well. Back in action the very evening she was released from the hospital.

Past: (well, I'd say November to the start of Pitolisant) Every day was a rush to get everything Ayden Jane needed packed in before 6 pm because any moment there after she may shut down. That shut down coming in all forms, the "stand and stare", the argue until Mom's head explodes, give in where ever you are planted and go to sleep, tell me "my brain is no longer working" and go to bed.

Now: Brain is working strong all day long with no after school recovery period and bed time more like 8-8:30. Ayden Jane went to Mckenna's soccer game last night and even ran up and down the sidelines as a ball girl for the second half. We did not get home until almost 9:00 and sure, AJ was tired, but it was typical kid tired.

Past: Ayden Jane had to have supper by 5 as her energy was crashing. She could not handle sleep tank and food tank empty. So either we all had to eat by 5 or just have more than one supper.

Now: We went to dinner at about 6 pm which translates into food around 6:30 and chatting until 7:30. I thought about it on the way and we could not have done that prior to pitolisant. Not on a school night. It's a simple thing, but it was the only night we could all do dinner together while Noah is home on spring break. She was able to keep up with the conversation, play games with Noah and enjoy the whole evening.

So, helping her recover from illness, increased wakefulness and pushing bed time back by 2 hours? I count them as positive effects of Pitolisant. 

Negative effects? She is "feeling her oats" at times and gets quite sassy. Well, she has always had the need to argue, but now she is quicker minded and argues more effectively. It is aggravating. She is even taking on new challenges like her teacher! Luckily Mrs. M will let her know where that line is and not allow her to learn any new habits.

Monday, March 6, 2017

Back on Track (hopefully)

Ayden Jane made it through the entire school day today. Typically, that is not anything all that exciting, but just a week ago we were sitting in the ER while she gulped for air. What a difference a week makes.

Starting on Saturday morning, Ayden Jane upped her dose of Pitolisant to 13.5mg (3 pills) per day. When we started this whole thing with Pitolisant, I was expecting to be able to observe each little increase over time. To sort out what benefits she was receiving and watch for any side effects. Ayden Jane getting sick has made much of that impossible, but I can give my opinion on what pitolisant is doing in spite of the illness.

First of all, in the past when Ayden Jane has gotten sick she has been quite slow to recover. It would take her weeks to stop napping and return to her normal energy level. Not this time. This time I would say her recovery is pretty remarkable. I mean, she made it through the full day of school, started the Girls on the Run program and did all her homework staying up until after 8:00.  So just recovery from illness, my gut says is improved by Pitolisant.

Noah is here for his spring break. I know that has nothing to do with Pitolisant, but he has noticed a significant improvement in her energy. He was amazed that she did not go to bed before 8. It reminds me that she was pretty much crashing by 6-6:30 every night. It made a big impact on our whole family. It effected dinner time, having to squeeze everything in before 6 because once the tiredness overcame her there was just nothing to do but put her to bed. We had to give up soccer because practice was 5:30 to 7 and she would fall asleep in the car on the way there and then could not pull it together and even participate. Now we have gained 2 productive hours in a day!


Sunday, March 5, 2017

What a Week

It is now Sunday. I think this may have been one of the longest weeks EVER. The highlights are Ayden Jane spending 3 days in the hospital and me being sick and exhausted.

I think we are both well on the mend. Mckenna is finally feeling better, although she has on very swollen ankle from soccer. The emotional part of this week for Mckenna was a friend dying in a car accident. Mckenna was not super close to her, but they were in Chemistry class together and joked around in the training room as they were both athletes. Mckenna spoke of her a few times in the past weeks and said how great she was and what an awesome attitude she had.

It's a little haunting, hearing Mckenna say things like, "but Mom, she was doing everything right. She was a good person and was just heading home from track practice. She wasn't out there doing stupid stuff, she didn't deserve this." She is right. Her friend didn't deserve this. Her friends family, the boy driving, the other kids in the school... none of them deserve to have lost this precious life. That's a lot for teenagers to process.

So glad to have this week behind us and know that each week, each day ahead is an opportunity to live the life God wants for us. Ayden Jane is such an example of knowing God, trusting Him and using every bit of ability within her to give God her best.


Monday, February 27, 2017

PWS and Illness

Ayden Jane has been super healthy for ... well, honestly, years. We were going on 2 years infection free. Wow did AJ break that streak in a big way.

Last Wednesday she started with a cold. It seemed simple enough, she didn't feel well and Gary got a call from the school nurse. By Thursday, full on fever had arrived along with congestion. The fever ran it's course and was gone by Friday evening. I took her into the pediatrician Saturday morning just so he could have a listen to be safe. He gave her the all clear sign and sent us on our way.

Then this morning, Monday, the momma ears knew there was more to this. I called and made an appointment back at the pediatrician, he took one look at her and sent us to the ER. I spent the next several hours watching her struggle, a fever begin and climb rapidly over 102 and listening to her gasp. Finally, meds on board she came around.

It's amazing to see how fast she went down. I wouldn't believe it if I had not witnessed it. Good news is she will likely bounce right back just as quickly because this kid is tough. I am excited to be on the mend and get back to seeing how Pitolisant brings out the best in her.

Thursday, February 23, 2017

Week One Done

As we end week one on Pitolisant/Wakix I am really excited about some results. I don't have too many to report because week one got side tracked with illness. Ayden Jane is entirely pitiful and it seems one of the nasty things that has been going around for months has finally caught up with her.

I will wrap up how things went up until yesterday. The 4-5:30 wakings seemed to have gone away. She settled into waking at around 6 or so, when I would be sure to slip her the Wakix. Then she would fall back asleep for a little while. Finally, she woke at 6 or so and stayed up with no afternoon lag either! The evening was still a bit short as by 7 she was deciding to go to bed, but she did so without that slow brain, eyelids half open look.

The report from school on Tuesday (the day before she got sick) was great. Her teacher, Mrs. M, noted improvements transitioning, more focus during math which is a time of day she typically struggles with and a general calmness she had not seen all year. I wish we had been able to put all that to a longer test as one day could be, just a good day.

After school, she had a bit of chill time but it was more a typical kid after school. Not a temporary shut down like happens often. She popped up to do her homework earlier, finished it quickly and then decided to do the entire week's worth of language, just to get it done.

So... I am torn. I truly feel like we are on the right track with Pitolisant, I want to get really excited about school. I want to hope homework evenings will be a breeze from here on out. I want to believe that she will sleep through and maybe even sleep in a bit once in a while.

On the other hand, I can't get too excited yet. It's too soon!!!

So for now, we will go to 9.0 but I don't think I will have anything to report that is a result of Pitolisant until after this awful cold goes away. 

Monday, February 20, 2017

Day 5

I feel like Pitolisant 4.5 has settled in. She is still up earlier than she likely should be, but I figure it's still a low dose so we are going in the right direction. Time and increased dose should improve that.

There's not much to report other than she is now sleeping pretty much all the way through the night, getting up around 6 and staying up. Her energy is more even, but still not where it should be for a 9 year old kiddo.

Basically, I want to go up in dose because I am excited by the direction of the improvements, but I know it is best for Ayden Jane to be patient and take it slow.

We shall see how school goes tomorrow.

Sunday, February 19, 2017

Day 4

Morning is the first test. Ayden Jane woke up a bit early and went back to bed.... Not as bad as it gets sometimes but not sleeping in like she had the past couple days. On the flipside, she also did not fall back to sleep and was easy and full of energy all morning.

My only drawback to Pitolisant so far is that Ayden Jane is more hungry. Now that could panic some parents but I'm really okay with it at this point. It's not that she is super hungry, it's more that she asked for a snack when she typically wouldn't. She wanted supper very early. I think there is some sort of shifting going on in there and she is struggling to figure out her bodies clues.

I asked her tonight how she liked Pitolisant so far. If she thought it was good, or bad, or didn't really make a difference. She said: I like it. It's good. So I asked her why. Her answer: I sleep better and have more energy. Not run around energy but I don't feel like I might fall asleep. I am taking that as pretty significant. I feel sort of bad because I had no idea she was sleeping so poorly or that she went through her day trying not to fall asleep!

She had her first blip tonight as she melted down at church club but I'm not entirely sure that had anything to do with the Pitolisant. More to do with driven kid who was not properly prepared. The church leaders love her though and were patient. Hoping she will knock their socks off in a few weeks.

Saturday, February 18, 2017

Day 2 and 3

Day 2
I got up at my usual time this morning and noticed that Ayden Jane's door was still closed. I realized she had not been up at all during the night. That is pretty big news as she was on a 4, 4;30, 5... spurt lately. I went about my morning routine and then I headed out the door ... before she was even awake!

Gary filled me in a bit on what I missed. AJ got up at 6:50 but ended up back in bed in full meltdown by 8:00. I am not even sure what the final catalyst was but mostly Ayden Jane needed to go back to bed with today's dose in her body.

Apparently, there can be a morning lull in the drug, particularly in the first week, that is quickly corrected once the new days dose is given. Sounds like it was pretty rough going there for a little while. We will have a three day weekend to be ready for school next week.

The rest of the day went smoothly, although she never did make it to school. I think that had more to do with it being the 3rd day of having a substitute teacher and it being more fun to be at home. When I got home she was in a NUTTY mood. Wanting me to wrestle and chase .. like the old days of needing deep input to organize her brain. When I was not enough fun, she went to her friends house in the neighborhood to bounce on the trampoline and play. Thankful for great neighbors!

At dinner we noticed 2 things. First, she drank and entire unsweet tea in about 30 seconds. Then went back for more. She typically drinks maybe half of a drink. Then, she inhaled her food. Also really strange because she is usually a very slow eater.

Day 3
Ayden Jane woke up at 6:30 and I gave her Wakix immediately.  She headed out to the sofa to watch TV and fell back asleep. I'm not really sure when she fell back asleep because, well, I might have also fallen back to sleep on the sofa. I do know she woke up at 9!! Really odd.

After a bit to eat we took the dogs for a walk and planned our day. I asked as we walked how she felt with Pitolisant? She said she feels happy and slept really well. I'll take it. We decided on a trip to Savannah's playground and a bit of shopping for our day.

No sleeping in the car. No hundred mile per hour talking to keep from sleeping in the car. No interview questions stuck on one topic. She read for a while, and then just sat calmly and looked out the window. I know that sounds crazy to note, but it is something she has never done. Her brain has always been working so hard in overdrive that if she stopped for a second, she was asleep. Today, she sat. Peacefully. Calmly. She sang to the radio. She occasionally asked me a question and then went back to just being quiet.

Oh, and the water thing turned up again today. She actually asked for a bottle of water. When I gave it to her she started drinking and then kept drinking. She asked me how I knew when I was thirsty. I tried to describe it and she just said that it is different for her, but maybe it is changing. She said she didn't know she was thirsty but she knew she had a snack and that wasn't fixing it so she wanted to try water. After she FINISHED the entire bottle she decided that was what her body was telling her.

So far, I am a fan. I am also amazed by the things that must be shifting inside of her somehow. I mean, a lot of the difference in her could just be a couple good nights sleep, but then there are the things like water? As long as Ayden Jane says she feels good we will forge ahead.

Thursday, February 16, 2017

Day 1

So. Much. Energy! Well, I know that Ayden Jane typically has huge responses to changes in her energy stream but even with that knowledge I was not prepared for today. And maybe her excitement over Pitolisant played a part as well?

We gave Ayden Jane her first dose at 6:15 this morning. She headed off to school ready to enjoy her day. Reports from school were great. Mrs. H checked on her here and there since her regular classroom teacher was absent today. She knows her well and saw some subtle differences in expression which was pretty cool. 

We unexpectedly picked Ayden Jane up early because her amazing PT, Mrs. Jen had a cancellation and we have been wanting her to take a peek at her scoliosis and help us plan a bit. AJ had a snack and then went out to play while she waited. She had lots of energy. which is not super odd, but it was right after school which is when she typically has a sort of afternoon lull. I didn't think too much of it since Mrs. Jen was there and we talked scoliosis. - (putting a few little things in place to hopefully turn things around)

After Jen was done with her, Ayden Jane started playing happily around the house. I took Mckenna to an appointment and returned with a rotisserie chicken and some fruit for supper. Mckenna had to eat right away so she could go to her game. It was nearing 5 which is when AJ often will decide she wants supper, especially if she sees me fixing some for somebody else. She looked at it and went back to playing. Mckenna ate, I ate, Ayden Jane played. At 5:30 I dropped MJ off at soccer and went back to work. Gary and she finally ate around 6:15 before Mckenna's game but so strange how she was just not worried about it.

I met up with them again at the game and Ayden Jane went wide open for nearly 2 hours. She did not seem a step behind, as usual but maybe I was just hoping. I guess time will tell.

Wednesday, February 15, 2017

Here We Go



Whew!! It made it. So tomorrow we will begin our grand adventure. The 'likely' target for dosing in 18 mg per day. To start, we will work with 4.5 mg tablets, taking one per day for a week or so then going to 2 per day and so on. (obviously, 4 per day is 18 mg) Once she is at a dose of 18 she can switch to the 18 mg pill.

So... what exactly am I hoping to see from Pitolisant? First of all, more wakeful and productive hours in her day. Currently, Ayden Jane has spurts where she does great but then she gets a bit knocked of schedule and ends up falling asleep in the afternoons and cannot recover the rest of the evening. Or, she just crashes, announcing her brain is done working and goes to bed by 6-6:30. Either way it's just not enough hours in the day for a 9 year old to do all the things she has to do!

Additionally, I am hoping that it will improve her processing speed. Basically, when all things are at a perfect balance, we get to see just how smart Ayden Jane is and enjoy her quick wit and caring personality. Trouble is, this balance is so very, very tenuous. The hope is that with Pitolisant, she will be able to operate in her sweet spot all day every day!! Wouldn't that be fantastic!

We have gathered some baseline data from school, and cognitive testing and even OT. At the end of the school year we will compare January data to May and then we should have good data to back up whether or not there have been significant improvement.

So, like I said, tomorrow is day 1. Her teacher will not be there which makes me a little nervous because she is awesome and knows Ayden Jane so well that it's like being there myself. Truthfully though, I am guessing that day 1 at a low dose will be slightly anti-climactic. If there are positives it should show up in the evening since that is when AJ struggles the most anyway. And tomorrow will be a very long day for her with school, after school club and a soccer scrimmage of Mckenna's where she will run around until fairly late. (8:30)

Wednesday, February 8, 2017

Getting Exciting

We applied for Pitolisant just a couple weeks ago. Hard to believe I received an email from UPS today that Ayden Jane's Wakix has made it through the border and is in the hands of UPS. I don't have a delivery day yet but I am guessing either Friday or Monday. Yay!!

I am hoping it will come on Friday because it would be great to start her on the weekend when I am with her. I am so lucky to have an awesome situation at school that I have no need to worry about Ayden Jane trying the drug out there. I just really want to be 'see for myself'. Of course, we are starting the dose low so I don't expect anything shocking right off the bat but I am so very, very, curious!!

Today Ayden Jane's OT did an evaluation to get a baseline before Wakix. Next up the school psychologist is going to do a few processing speed and executive function tests on Friday. Slipping them all in just in time! The hope is that Wakix will make a significant impact in these areas and we will be able to capture that impact in numbers. 

Stay tuned!

Friday, February 3, 2017

All In

We have spent the past couple weeks slipping down to Florida to see Dr. Miller, heading off to Greenville to check on her scoliosis and getting labs drawn. It is tiring, and not all the news was good but Ayden Jane is a trooper.

When we saw Dr. Miller she was happy with how Ayden Jane is doing. No surprise. She did get to see AJ while she was a little off. The crazy eye was doing it's thing. Always a sign she is a step off. She was quiet and that delay in conversation was working. Travel with no exercise in bad weather and lots of waiting around is never a good thing.

The biggest issue I wanted to discuss with Dr. Miller is how tired Ayden Jane has been getting lately. It is painful to watch her day go by so quickly. Her day has been cut short by an ever advancing bed time. She is just absolutely done by 6:30 most nights, well, unless she takes a nap after school. There are just not enough hours in her day. She is at an age where this is common in PWS and referred to as daytime sleepiness and is connected to a sleep/wake cycle that does not really work properly. There are a few medications that have helped many families but the side effects make me nervous. So, we decided to apply for a new medication that is only available in Europe. This link explains what we are doing. Pitolisant/Wakix

Hopefully I will lots of exciting things to post in a few weeks when Ayden Jane starts it.

Next we went to Shriner's to check on her scoliosis. Dr. Miller had seen a curve beginning again and that was confirmed by x-ray. Currently her curve is 12 degrees. That is an increase from our last visit and concerning as she is getting ready to head through a large growth spurt. We will be hitting the Physical Therapy hard again. I am thankful swim team will be back in full swing next week and I am hoping the schedule will work out that Ayden Jane can participate in Girls on the Run this spring.

Her tone is not great at the moment but that is often the case in February... Time to start getting active again. I am hopeful with Pitolisant Ayden Jane will gain added quality hours in her day which will give her the ability to get back to regular activity and beyond.

Saturday, January 14, 2017

Winter Challenges

Keeping Ayden Jane in the sweet spot so that she is can do well in school, keep her weight well managed and just be happy over all, (not to mention easy to live with) gets to be a real challenge in the winter months. We are nearing the end of January when she is typically the heaviest, most challenging, lowest tone....

The exercise piece seems to be so crazy important for Ayden Jane. I am not sure what the extended exercise does in some areas but I am sure that it positively effects blood sugar, ketone production and metabolism. It goes like this - when she gets the long walks, the good hard swim team practice, hours at the beach and pool, long sunny days on the trampoline with friends, regular running from training to run a race... you get the picture. When these happen, Ayden Jane is happy, not hungry, thinks quickly, sleeps soundly, learns fast and is just a joy to be around.

When this sort of exercise ceases to happen, her brain gets stuck. She gets a little more hungry. She argues more. She gets a bit anxious. She needs things repeated. It takes forever to do schoolwork. The pace of life slows even though the effort increases on her part. It is frustrating for ALL involved.

In some ways, I think it is good that during this time is when we are seeing Dr. Miller. It reminds me of all the concerns we still have for Ayden Jane. On AJ's part, she notices the difference in her brain running smoothly and not. She gets frustrated by it. She says things about how she does not like having to take her pills ect. EVERY DAY. I think she feels the very real and consistent, daily burden of dealing with a chronic medical issues.

I look forward to the day that she does not feel that burden so heavily.

Monday, January 2, 2017

2016 Recap / 2017 Preview

One thing I really enjoy about the blog is I can look back over the year we just completed as I look toward the year to come. 2016 had some fantastic accomplishes and some very hard times.

The hard times are marked by my Dad's passing, Gary having major wrist surgery and being out of work, Noah with a total of 3 surgeries before years end, Mckenna ending up in the hospital for a week and Kayla patiently/not so patiently trying to figure out the next phase in her life. We are thankful that most of these things have settled, and hearts and bodies are all the mend.

There have also been many good things. First plane rides, first trips to roller coasters, visiting Boston and Chicago and Indianapolis. Cheering for Noah in person, as a family, for his last home soccer game.

For Ayden Jane, the triumphs add up. Her first honor roll at the end of second grade. Reading taking off, Swim team going well, Traveling to Boston with Kayla, Trips to see PWS friends across the country. Climbing to the top of the rock wall. Standarized test percentile ranks climbing. Making good friendships. Hunger nearly disappearing. Learning to monitor and maintain her own weight. Eyes improving. Conquering the monkey bars.

Looking forward, I can barely see through to the end of January. Coming up this month we are remodeling our kitchen. Annual review time at work with 41 IEP's to write and meetings to hold. Gary going back to work (hopefully confirmed tomorrow). High School soccer back in action for Mckenna. Swim team and (hopefully) girls on the run for Ayden Jane by the end of the month. A trip to see Dr. Miller and to Shriners's for a scoliosis check. Such a busy month it will be!!

Hopefully the rest of the year won't be quite so incredibly busy. I am thankful to have jobs and active kids. I am so thankful to get a much needed kitchen update. I am thankful for caring doctors even if travel is required to get to them.

There is much to hope for on the PWS research/treatment front as well... Not going into that right now, but I am truly excited and believe that one day, the last pauses and challenges brought to AJ by PWS will be put behind her.

Wow, here is to the start of a new year and tackling it head on!!