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...

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.

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.

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. 

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.

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. 

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.

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.

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.

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.

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)