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


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.