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.