Saturday, October 22, 2016

gh woes

Hurricane Matthew interupted (thanks to knocking out power) our gh prescription. Basically, Ayden Jane needed an increase in dosage which required a new Prior Authorization. We couldn't start the process without power and we had never had issues before so I was not too concerned. With the late start we then found out that the insurance was deciding to make it a challenge for the first time ever!

They did not want to agree with Dr. Miller's dose increase because Ayden Jane has not had an office visit with her in over a year. You see, our wonderful Dr. Clark who used to prescribe her gh and saw her every 6 months moved out of state. So, we decided Dr. Miller would be her only endo. We saw Dr. Clark in January before she moved in June and it was over the summer that Ayden Jane had the strange spurt of growth in so many ways... including a dropping IGF-1 (finally)

We did see Dr. Miller last October, but it was at the 5k. Honestly, she spent more time with her that weekend than she would in her office so I think it should totally count! And we decided to cancel her actual appointment because of that very reason.

It's all pretty confusing to me though, because whether or not Dr. M sees Ayden Jane with her eyes, her blood work is clear that she needs the increase. So.... grrrrrrr.

For now, Dr. M had pfizer send an intermim supply while she works on it.

So, what were the results in Ayden Jane of the lack of gh? Not fun at all. After just a few days. Low energy. Panicky. Rigid. Poor sleep.... It was obvious to her teacher at school as well. For me, it's just a little scary to realize how dependent Ayden Jane is on things like gh. Truly makes the difference between being a typical kid or not.

Thursday, October 6, 2016

Prepping for Matthew

Yes, I have been extremely slack with the blog lately. I will try to improve that but for now, the topic is Hurricane Matthew.

We are not evacuating and are not is a zone that HAS to go. I'd say about half the people in our neighborhood are still here so we are in good company. For us, Matt prep has consisted of cleaning out the garage and gutters - yes, it's needed to have been done all summer and it took a hurricane to make it happen.

I also cut the grass. Not because I was afraid long grass and a hurricane somehow make for dangerous conditions, but because it was really long and with another dose of water and time to dry out it might actually be a jungle I would need serious equipment to get through. All the outdoor furniture is no longer out doors. Okay, for us that means the plastic adirondack chairs are put away. It wasn't too strenuous.

I did prep food for Ayden Jane. I know we may lose power for a while and I need to have options that do not need to be refrigerated or heated. I went with cashew bread, cottage cheese muffins, hard boiled eggs (okay, I will have a cooler) and coconut bars. I also bought some beef jerky, nuts and even Kind bars. She should be set.

Ayden Jane decided she needed to play outside ALL day long because, gasp, it's going to rain for 2 straight days!! She has no idea what she will do with herself. Crazy kid. It was great though. Her friend from the other side of the neighborhood came down and the bounced and rode bikes and gary took them to the playground. She was filthy by days end so I know she had a great day.

School work has been put up as we have had mini vaccation from school since it was cancelled Wednesday through Friday. (and I has a suspicion it will be out Monday as well).

We may never get back on the soccer field with all the water monsoon season turned hurricane has brought us, but she is happy when she gets to play. It has been a super sweet group of girls and she has really enjoyed it. I can't say her soccer is impressive, but the friendships and social skills are doing well.

She is still swimming on the swim team and I have to say I am pretty impressed. She is getting much better in the pool. Her arms are coming well out of the water when she swims and she is proud of herself for swimming 100's.

Hopefully we will still have power most of tomorrow and I can fill you in on Ayden Jane's 9th birthday!

Wednesday, September 28, 2016


 I was going through some things and I found the picture on the right from April. It struck me how different Ayden Jane looks now so made this. These pictures are only four months apart, but what a change in four months!!

Friday, September 2, 2016

Third Grade Update

We are nearly done with the third week of third grade. It is going so fast! Glad to say Ayden Jane is doing GREAT. She has been working hard in math and is mastering the concept of multiplying. Lots of arrays, groups, number sentences, skip counting... She totally gets it. I do see trouble on the horizon as she does not memorize her facts well and needs to figure them out pretty much every time but I choose to enjoy the moment.

Her writing is and will likely always be her biggest challenge, but I can say it is already improving this year. That bar is set higher so she is digging deep and making it happen. I often still think she can't write it small enough, or she can't fit that in the box, or drawing out that multiplication array is too tough. Then she does it. On the flipside, she has gotten very comfortable dictating to me on homework when it's overwhelming. I love that because I pretty much hate spending time on homework.

Her teacher only has good things to say so hooray.

She did come home with a tradgedy today. Her star was moved because she was talking when she was not supposed to be... She lost some "dollars". It was hard for her to accept, but in a way it was a good thing for her to get through and realize the world did not end. As a side note, I don't really like the 'behavior' systems that take away points or money or whatever. So much easier to handle not earning things than it is to have to hand over things.

The hardest part of the star moving is that Ayden Jane feels it was a sort of injustice. She does not deny that she was talking when she was not supposed to be, but feels that other kids were talking when they were not supposed to be at other times and did not get in trouble. Oh dear child. It is a fact of life that sometimes people get away with doing wrong.... Life is not fair.... I can't fix that nor can you. We talked about how she can spend her time worrying about how other people are behaving and whether or not they are being "caught" ect, but that it is a waste of time and effort. The best thing she can do is take care of her own choices and be the best Ayden Jane she can. Such big kid lessons.

She cried it out. The interesting thing is she sort of knew he just needed to be miserable about it. I thought it might be the topic for the remainder of the night. I wondered if she would balk at going to school this morning. I asked if she wanted me to fix it. She said she knew I couldn't fix it and after about 10 minutes or so mourning she bounced back, never mentioned it again and went on with her night. I so hope she has a great day today and learns that sometimes we all mess up, get caught, pay the price and move on...

Tuesday, August 30, 2016

What Does Histamine (H3R) Have to do with it?

When October arrives our Ayden Jane turns 9!! Can you believe it? What a wild rollercoaster ride she has taken us on over the years. She entered the world a frail, weak little mystery; too weak to eat, or move, or even respond. I never could have imagined at that time as I sat by her side waiting for her to “wake up”, hidden inside that beautiful, motionless little baby was a determination and strong will like I have never seen.

She has lived a life of hard work, therapies, dietary restrictions, doctor’s appointments, blood draws, shots and much more, but she does it all with no complaints and a smile (most of the time).  She worked to learn to crawl, walk, run, read, write (still working hard on that one), play sports, learn social skills … the list goes on. 

She still has gaps in her ability to operate in a fast paced world. Everything takes more effort for her than for other kids and just getting through the day can be exhausting. Even sleep is a bit off for kids with PWS. Every part of their day and night is affected.

Even as I write this, waiting for supper to come out of the oven, Ayden Jane is napping. Naps had all but disappeared over the summer but with the return of school, she is tired. It takes so much out of her to keep up all day long. If only things could come easier! 

So what if they discovered a receptor in the brain that effected things that lined up almost precisely with the host of symptoms of Prader Willi Syndrome.

I introduce you to the Histamine 3 receptor and the drug that has already been developed and approved for Narcolepsy in Europe – Pitolisant. The link below describes the science for those who enjoy that.

In Ayden Jane's short lifetime new ideas have made a big change in what is possible for people with Prader Willi Syndrome. I am hopeful that Pitolisant will level the playing field for Ayden Jane and allow her to not just 'keep up' with her peers but do so without giving every ounce of effort she has to give. I am hopeful it will allow Ayden Jane to enjoy the ride more often and even to excel in some areas. I am hopeful this will allow me to see what Ayden Jane would be like without Prader Willi Syndrome.

Wednesday, August 24, 2016

Third Grade Day 6

As far as I know, Ayden Jane is doing well in 3rd grade. The first week started on a Wednesday so she had just 3 days. She handled it well and didn't seem too worn out. Of course, then she went into the weekend by going to the sleepover on Friday night...

This week is her first full week, and by full I mean FULL. She went into it a little tired still from the weekend. Then Monday she had soccer practice. She is still new to that so it's a lot. Then Tuesday she had friend over and played late.... By the time she had her homework done and was showered it was after 9.

Today is Wednesday and wow did it catch up to her. She really just needed a nap, but would not cave on that. In the end, she did all she could do and was in full meltdown and asleep by 6:30.

It is going to be a challenging balance this year. School, homework, after school activities and good old hang out fun. Ayden Jane wants to do everything ... but really cannot function when she gets too tired. It's tricky. She will push herself to the brink - and then dump on me.

Meanwhile, I am feeling like the slack mom. I screwed up some communication and Ayden Jane will miss a couple doses of gh just as she is trying so hard to adjust to the new busy schedule. ugh!! It should get here tomorrow. Just makes me so sad/frustrated that I didn't keep track of the details so that this happened. I know it sounds like no big deal. Okay, I know it really is no big deal, but somehow it just hits really hard. Pretty much all of Ayden Jane's meds, supplements, diet, PT, OT, exercise .... is my responsibility and I know each little piece adds up.

Here's hoping that she has a great nights sleep and tomorrow goes better.

Saturday, August 20, 2016

Third Grade

First day of third grade!! It was an interesting start to the school year. I took a last minute job to fill in for a friend who is unable to start the school year so I was not even able to take Ayden Jane to school on her first day.

Gary took this quick picture of our girl all ready to take on third grade. It sounds like all is well so far and she is doing great.

She also had her first slumber party at her friends house this weekend. They went over to a pool and played. The parents were ordering pizza so I told them I would bring some of Ayden Jane's pizza over for her to eat about the same time. When I came in, Ayden Jane saw me and asked, "mom, what are you doing here? You don't have to pick me up you know. I'm spending the night."

She wanted to handle this one all on her own and did not want mom around to cramp her style.

I did stay a bit to chat with MY friends but not too long.

Later I stopped by to give the gh shot to her. I tried to get her to slip off the sofa where the girls were just starting a movie so we could do it quick in the other room. Ayden Jane was not interested in that. She wanted me to come to her and just get it over with. So, on a sofa with 4 girls she pulled out her thigh and I gave her the shot. One of the girls eyes were so wide I thought they might pop right out of her head! As I left the room, there was lots of what was that?! Ayden Jane just said, "it's my shot I do at night. It's fine. It doesn't hurt." That was the end of it.

We treat Ayden Jane's PWS as no big deal. The supplements, the oils, the shot... it's just a part of life. She has totally adopted that same attitude but she has no idea that all she does is slightly shocking to others. Not that it would slow her down anyway....