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.

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.

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. 

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.

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.

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.

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.