Thursday, July 5, 2018

I Know What I Can Eat

Posts have slowed... obviously. It's partially because we are busy. Partially because life is sort of on cruise control. Mostly, however, it's because Ayden Jane is older. The things I would write about seem more personal somehow. I want the blog to reflect life with Prader Willi Sydrome for Ayden Jane. At the same time, she is just a kid who has struggles like any other kid and who wants their struggles broadcast.

That said, I haven't abandoned trying to write her story. I am just working on how to do so respectfully.

The struggle she had today is clearly a PWS struggle. Well, I guess it is really a 'medical diet' struggle. Ayden Jane works hard to understand her diet. She is learning well and proud of herself. Today she was at a camp and the director was concerned about giving Ayden Jane whipped cream on her blueberries. Ayden Jane knows she can have whipped cream but the director texted me anyway. (which I think was totally the correct choice. Better safe than sorry) Ayden Jane, however, is very bothered when situations like this come up and she knows, that she knows, she can have something and people doubt her. It may be more that she thinks they don't trust she's smart enough? Maybe that they think she is not smart? Maybe she partly worry's they think she is lying?  Whatever the reason, it REALLY bothers her. She held it in until she got home and then I got an earful of how mad it makes her that they don't believe her. That she knows what she can have and that she isn't going to eat anything she can't have because it would just make her feel awful and she wouldn't have any fun....

Having Prader Willi Syndrome means having a lot of rules about food. It means having to think with your head and not trust you body. It means having a great deal of self-discipline. At age 10 Ayden Jane is determined to use her head, follow the rules, and have more self discipline than most adults can muster. Great job AJ.

Sunday, May 27, 2018


Last weekend Ayden Jane and I traveled to Houston to meet up with a few of the families that are also taking Pitolisant. Well, the point to the weekend was to meet with Drs. at Texas Children's Hospital to discuss pediatric narcolepsy and Pitolisant and PWS and better characterizing sleep in PWS.

Ayden Jane and I ended up flying in too late to actually meet with any of the doctors as the agenda switched a bit after our tickets were purchased but Ayden Jane had a wonderful time hanging out with her friends.

It was interesting to see how relaxing it was for her to be around kids who operate in her realm, so to speak. They have similar food rules, the have a little slower processing so Ayden Jane is never left behind. They all accept each other for exactly who they are. They totally get it that each one is being the best person they can be and that sometimes it's just hard.

I had a great time watching the kids run around, play hide-n-seek and just enjoy being together. No pressure. Ayden Jane was firing well for most of the weekend. She did crash a bit while we were at the airport. She really wanted to hang out with J for a little while before our plane took off but AJ was really just not much fun. After we said goodbye I finally got smart and found her some caffeine and poof! She was right back on track and just in the nick of time! We got on the plane and then it was delayed... yep, we had to sit and wait a while and if she hadn't rebounded so well it was going to be a long, long wait. She did beautifully. Pulled out a sticker book and dressed the movie stars in outfits and was GREAT.

We returned late Sunday night and I was tired as well but we both got up Monday morning and went about our days.

I need to spend the summer getting her lots of opportunity to hang out with kids. Being a youngest by so many years and having such awesome big siblings, she spends a lot of time with "adults". We pulled her from school and she has been spending even less time with kids her age not to mention that she started acting a bit crazy from all the stress before we pulled her.

I'm out in a couple weeks. Noah comes home. Summer camps and beach/pool days begin. Hoping it is a wonderful summer! 

Sunday, May 13, 2018

Overdue Update

I can't believe it's been a month! Okay, I can believe it because time has been flying. When I left off we were considering bumping Pitolisant. I chatted with Dr. Miller and for a few reasons we opted to wait. Mostly, I was grasping at straws to figure some things out that honestly, had nothing to do with Pitolisant.

Ayden Jane did not have a banner year in school. Not sure exactly where it went off the rails but it became more and more obvious that there was no way we were going to get things back on track. It was beginning to really hurt Ayden Jane's health, well being and ability to learn. By this point, Ayden Jane was not sleeping well, waking with night mares, snapping defensively at most comments, her posture was tense and hunched over, her voice unsure and the tics... oh how the tics were developing. She was becoming anxious about food and her weight was rising. Basically, PWS was creeping in as the anxiety escalated. I spoke with Dr. Miller about all of it and she said to pull her from school. She would fill out whatever form I needed but that Ayden Jane needed to be removed from that setting.

I was honestly unsure how it was all going to work but I could not handle watching the tics that shook her body take over. After about 3 days the anxiety seemed to wane. A week in there was so much more laughter. So much better sleep. No more night mares and the tics were down to mild movements. The food anxiety disappeared and eventually the defensive snapping at us relaxed. I was getting my sweet, confident, funny Ayden Jane back. I began to realized just how bad things had been as I saw the relief.

So how has the schooling piece gone? Great. Ayden Jane works so very hard and Gary is home with her. Their routine is to go to dunkin' donuts and get a coffee and work there for a couple hours. (Ironic, I know) Ayden Jane is focused and gets a lot of work done. Her love of learning has returned and she is like a sponge. The other great thing is that she is truly excited to go back to school next year. She is relieved to finish the year this way, but does miss seeing other kids and the activity of school.

Last week she went to her school to take state standardized testing and did great. No problems, no anxiety ... almost gleeful to go. Here's to 4 more weeks of medical home bound, and then SUMMER.

Tuesday, April 17, 2018

Camp Out and Spring Break

The week before spring break, Ayden Jane's school did a 4th grade camp out. The kids spent arrived at the state park early on Wednesday, spent the night and stayed Thursday until time for school to end. They had lots of activities and learning and fun. Ayden Jane really enjoyed Wednesday - I was not there for most of it - and we slept in a tent Wednesday night. That was hard on her. She did not sleep much and Thursday we ended up going home a bit early because she was DONE. Just tired and not having fun so no reason to push through. 

I received good reports on how she did through the day on Wednesday and enjoyed chatting with a teacher that helped with her class. I was told that Ayden Jane was well behaved and made great connections. The teacher even said she was smart and she liked how Ayden Jane's brain works. So fun when there are people that "get" her and appreciate how she thinks.

The camp out rolled us pretty much right into a road trip for spring break. It was a whirl wind trip with stops in Asheville for hiking and visiting water falls, Chicago to see many friends and to visit with Noah. On the return trip we swung by Indianapolis for a very quick visit with more friends who are part of our PWS family. 

Overall, Ayden Jane did well. The hiking was tough, but she did enjoy most of it. Spending the night in multiple homes, a hotel, lots of car travel. Not typically a great plan for kids with PWS but she rolled with it pretty well. She did get tired which causes over emotional and snacky. That's never fun. 

On this side of the trip, we are settling back into a routine. She is definitely 'mushy' and her core need lots of work from the winter and travel. Her weight is not good. Her sleep is still not back to where it should be. So, what do I do? Make a PLAN.

First up, we are going back to horses. It's just so great for her and we have had a long break. Next, the pool and ocean are beginning to warm up so there will be lots of swimming and boogie boarding in our near future. I am working on a running plan with motivators along the way. And finally, I am going to bump up her Pitolisant on Friday. It typically comes with a bit of weight gain, but at this point we may as well just add that to what we are already working on. Hopefully, the bump with help her get her sleep schedule back on track and maybe give her a bit more daytime stamina. 

We shall see!

Wednesday, March 28, 2018

Good Computer, Bad Wifi

At times, I need to sum up Ayden Jane's brain for people. The other day this analogy came to me. It is as if Ayden Jane's brain is an excellent computer. She is smart and can connect things well. She loves to learn and has some great skills. She is capable of learning everything she needs to learn to do very well in school.

So why does she have days/times where it seems she is very smart and days/times when she seems to struggle academically or to even put together a sentence?

I think the best description is that she has a good computer (brain), but the wifi is not always great.

-Sometimes it is all in there but waiting for her to respond, or "load the page", is like watching that little circle on your computer spin as say loading.

-Sometimes that loading sign means she has taken in more information than she can process and she needs time for all of it to file in the right slots.

-Sometimes that loading sign means she wants to concentrate on the words you are saying but her brain can't turn off the uncomfortable feeling of her shoes, or how hot she feels, or the background noise you don't even notice, or the new decorations on the wall, or.... you get the picture.

-Sometimes that loading sign means she is running low on food for her brain.

-Sometimes that loading sign means she is just plain too tired to summon the extra effort it takes to process the information you are sending her way.

I have to admit, I love the days were information process quickly and she can learn complicated things with ease. Oh how I love it when anything comes with ease for her.

I need to remind myself sometimes, when I am frustrated with that spinning circle, that it has to be even more frustrating for her. 

Tuesday, March 20, 2018


I haven't said much about Pitolisant in particular lately. Mostly because the other things that have been going on sort of overshadowed it.

Here is where we are with it. Ayden Jane started a bit over a year ago. We went up fairly quickly, okay very quickly. Approximately every two weeks brought about a dose increase. The increments were in 4.5 milligram amounts until we got to 18. By that time we were close to the end of the school year and decided to let things settle in over the summer. Summer's are easy on pretty much all fronts. Of course, other than the keeping her busy front. She wants non-stop action in the summer. (for details on how all that went you can look back to Feb/March of 2017)

After summer I decided to let Ayden Jane settle into grade 4 on the same dose. Not thinking it was all she needed, but wanting her teacher to get to know her as she was on 18 and help me be able to note improvements if I increased the dose. Well, it started off okay, and I increased and then this year sort of crashed and burned on a number of levels. I have not been able to get good info from school. She got sick in December. 

I saw Dr. Miller at the end of January and she was still not well. Dr. Miller gave me the go ahead to bump her dose again. This relieved the need to cut her pills into fourths and allows me to give her a pill and a half, equaling 27 mg. My frustration is that I feel like her brain is better. I think she is processing well and can learn more easily. I believe she is communicating better in general and know she has been way more fun to live with. It's the feel, think and believe stuff. I really want an objective opinion that does not spend as much time with her to be able to help put some parameters on the gains she has made.

Enter her amazing teacher from last year. The one who walked us through those challenging first months and pushed Ayden Jane to new heights last year. Even more importantly, the teacher that Ayden Jane connected with, is able to communicate well with, trusts and loves. The wonderful Mrs. M has agreed to spend a bit of time with Ayden Jane with a two fold purpose. 

1) Help Ayden Jane improve/regain her confidence in her academic abilities.

2) Get a feel for how Ayden Jane is processing, comprehending and learning. 

I can hardly wait!!


Thursday, March 15, 2018


As the days go by, we are enjoying a pretty calm time. Ayden Jane is getting up and out the door on time for school most days. That was not the case much of this year. I am not sure what the change is... In the past she was simply not stressed about whether or not she arrived on time. Nothing we said or did seemed to motivate her. We all know that no steps in the morning routine could be skipped. And, of course, push too hard and you just create meltdown central... Some days she would nearly make it out the door and then decide she would not make it through the day if she did not shower! Seems as though the past few weeks have been smooth sailing though!

Ayden Jane also seems to have a better grip on school in general as well. She comes home and knows exactly what she wants to work on. She does not get a sort of standard homework. She brings home anything not done or asks for me to go over with her anything she did not understand. I have no idea how she is doing from the schools perspective, but she seems much calmer and less overwhelmed by it all.

We have been working on a lot of Khan Academy in the evenings. I am still a bit unclear on whether these assignments are completely required, or if they are supplemental. I'm guessing both to an extent. Ayden Jane really likes when we do these together because she does not understand the video. The idea behind Khan Academy is that there are video instructions for each skill so you can watch the instruction, It sounds strange, but she is not good at obtaining information from video. Even as a small child, she did not watch TV or video. I remember being thrilled at about age 4 that she would watch an entire episode of Curious George and give me a break!

I have enjoyed the Khan Academy with her though. She truly does not want to get answers right just to get answers correct. She gets so excited about understanding what she is doing. I could give her correct answers and she would not enter them unless I showed her how. I hope she retains that love of learning and wanting to understand. If we can keep Khan over summer I will be happy to Khan and read the summer away and be on top of things for grade 5.

We need a win next year. This one has been rough.