Monday, December 26, 2016

NAC

The last couple of days of school and the first part of break I gave Ayden Jane NAC. Once upon a time I gave her pharmaNac which is a pill that fizzes in water like alkaselzer. It was a good thing for her and helped in a few areas including reducing the pws pause and a sort of studder she had when younger. The formula of the Nac was changed by the company and it upset AJ's tummy so we stopped. That was last spring...

After chatting with Dr. Miller we decided to give a capsule form of Nac a try. Others have used it with success - although most use it to help with skin picking. It sort of fell in the category of safe and maybe it would help her get over the afternoon lull a bit more smoothly.

What did we notice? I think there was a slight improvement in the fluidity of her words and the quickness of her verbal interactions. You know, a reduction in th pws pause in processing speed. That was the positive side. On the negative side Ayden Jane STUNK. I mean, shower in the morning stinky by lunchtime. I'm not talking a mild odor, I mean no one would want to sit near her sort of smelly. 

Additionally, she was hungry. By 10 in the morning she was already asking if it was lunchtime. In fact, every hour or so she was wanting to eat something. That can be typical with PWS but it is not typical for Ayden Jane. She gained a couple pounds during the week she was on it.

I stopped and within 48 the horrid smell and the hunger were both gone. It is so strange. I asked Dr. Miller if that was a side effect of the NAC and she had never heard of it happening. Well, I guess that's just my AJ. At least she never leaves us guessing as to whether something is effecting her in a positive or negative way.

Saturday, December 17, 2016

Third Grade

I know, I have been terrible about keeping things going on the blog... Going back to work full time has definitely taken a toll on my time for things like this that I enjoy.

Ayden Jane is still doing well in third grade. She does struggle some days in the afternoon with maintaining the energy demands of a full school day. Her teacher is awesome, however, and finds ways around it. If there are things Ayden Jane just cannot get done while she is at school, she sends them home on the weekend. There is no time during the week to add on to her current homework and extracurricular activities.

The plan was for soccer 2 nights a week and swim team 2 nights a week. The timing did not always work out so most of the time she made it to soccer just once. She also attends and after school club one day a week.

This coming spring we are looking to trade in the soccer for girls on the run. Ayden Jane is not entirely sold on the concept yet but I am hopeful. Basically, girls on the run is close to home and I think she would be very successful in it. Soccer is a bit of a drive and, I hate to tell her, not her forte.

Point of concern is that as we were walking on the beach with the dogs today, I noticed her crazy leg again. She ran with Tucker and that darn right foot was not aligned like we worked SO HARD on. Of course, the PWS momma in me wants to panic that her foot is a sign that her hips are internally rotating which is a sign that her back is crooked! We have a scoliosis check with Shriner's in February and hopefully we can get a quick check in with the amazing Mrs. Jen over Christmas break.

On the positive side, Ayden Jane is finally a totally independent reader! She sat and read 175 pages of a book in one sitting last night. Until then we would read chapter books, but she always wanted to read them together. More intersted in the time with one of us than in the actual reading.

Big things coming up for Ayden Jane over the next couple of months. Trip to FL to see Dr. Miller, labs and a trip to Shriner's. 

Monday, November 28, 2016

Monkey bars


video

Years of hard work paying off. Ayden Jane has been working on being able to do the monkey bars for years. Seriously. She would have occasional success and get 4 or 5 bars... This is a long set of bars, 13 all the way across.  She has been working on them each day at school and apparently mastered them! She was so proud to show me.

Monday, November 14, 2016

PWS Pause

Ayden Jane is doing so amazingly well .... most of the time.

It goes like this. When she has had enough sleep, enough exercise, the right food, proper supplements, the right dose of meds, completely healthy, not too much to process ... she is quick witted, kind hearted, funny, deep thinking and an overall ray of sunshine.

An example of her wit would be last night. I told her I was going running but I stopped by the computer to shoot off a quick email. She called, "mom, I thought you were going running." I said, "I am." She giggled and said, "that doesn't look like running to me!" Then Kayla came in the door and I invited her to go run with me. AJ didn't even look up from the page she was coloring and said, "Well we all know that's not happening."

I suppose I should not encourage the sass and it may come back to bite me one day.

Most of the time, Ayden Jane operates in near top form and although it is a lot to balance, we have become quite good at maintaining the balance needed. At times, though, she just can't follow what you are saying or there are just too many things going on for her to process quickly enough. She has begun to recognize this for what it is. In the past, she would just argue with you and claim that her lack of understanding was your fault. Or just stop and stare, as if she is waiting for her brain to catch up to real time but it can't.

A few days ago I was trying to explain something to her when she was a bit tired. After I repeated the same thing for a third time and was getting impatient with her she tells me, "I hear the words coming out of your mouth but my brain isn't processing them." Wow. What a great description. I have also heard, "Can I say it one more time, please? I just can't get it off my brain."

These PWS pauses/glitches in her neurology are annoying. They make some days a challenge. If I put a positive spin on them, they also show how amazing Ayden Jane is thinking most of the time. They keep me from taking the other 90 percent of her day for granted.

Monday, November 7, 2016

Adjustments

I have gone back to work full time which has been a big adjustment around here. Well, a big adjustment for most of us. I have been leaving for work before Ayden Jane is even up in the mornings often times. I don't get to pick her up from school. It's strange.

In all honestly, Ayden Jane gives Gary a really hard time. It's funny, in a way. Probably not to Gary, but still. Bascially, I have been primary care giver for as long as Ayden Jane can remember. Gary has worked a ton of hours and mostly has played with Ayden Jane and spent great time with her in a sort of... give me a break role. Ayden Jane simply just does not yet trust that he has any idea what he is doing. I mean, want to play a board game? Dad's the go to. Want to swim out in the ocean to the deep, deep? Dad is happy to take charge of that. Need someone to be spoiled rotton? AJ knows Dad is always there.

It has been a few weeks and it seems they are begining to find their own rhythm. Hopefully he is no longer hearing, "that's not how mom does is" too many times each morning getting ready for school.

Aside from the adjustment, Ayden Jane has had a few pretty hysterical comments lately. I figure I really need to start writing them down so here are two Ayden Jane 'quotes'.

Ayden Jane took the CogAT testing. It is a test to determine eligiblity for the gifted and talented program. Honestly, it makes no difference to me if she even takes the test, but I just let her roll with it and give it a go. You know, sort of like practice. After it was done she was talking about it one evening.

"I finished one section and the directions said to go back and check your answers so I erased them all but then ran out of time to do them again." True PWS brain moment. So very literal.

Saturday we were riding over to grandma's house to help with a few things. Ayden Jane started talking about Grandpa (who died last summer) She asked, "How did they know Grandpa was dead?" I answered, "well, he wasn't breathing so they had to go to the hospital." Then Ayden Jane asked me again, but answered her own question with, "Did they know he was dead because all the light went out of him and only darkness was left in his body?" She just gets things on a different level than the rest of us.

Friday, October 28, 2016

Senior Day at Wheaton



Such a great time watcbing Noah play in person one last time. It was the only game I was able to attend in his two years at Wheaton. (So thankful for the live feed on computer). Loved getting to meet some of his friends and their parents. Loved hearing him talk the entire game long as he directed and encouraged his team. Was able to see his first goal of this season (and possibly only) So great having the entire family there to cheer him on even in the cold, misty Wheaton weather. Ayden Jane watched the game intently and asked questions and cheered for her brother. So cute. Noah summed it up well, "it was a great senior day. We won 4-0, I scored and my whole family was here." 

We have watched Noah play soccer since he was a little, 4 year old boy. He played with such joy and watching him last night, he was having fun! That's how it should be. He was enjoying the gift of athleticism God blessed him with. He was enjoying the comraderie that goes with a tight knit team. He was thoroughly enjoying the game he has played his entire life, but with a peace that the end is near. It's been a great run and he is ready to transition to life without soccer. Hard to believe.


We also spent some time just hanging out and enjoying fall. Something we don't really have here in Pawleys. There are times as a parent you wonder if you are doing anything right. Okay, there are a lot of times as a parent when you wonder if you are doing anything right. Then days come along when you get to just watch your kids relax together and enjoy each other. You put aside the worry and just enjoy the amazing people they are turning into before your eyes. Each has there own challenges to conqueor and missions to accomplish and I have no idea where life will take them. I can say for certain, however, they are good people who know how to laugh, love, serve and be kind to others. Love them all.

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

Growing

 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. 

http://www.chionfoundation.org/single-post/2016/08/14/Why-Pitolisant

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....

Saturday, August 13, 2016

Whew

I did not think it was possible for things to get even more busy! But I think they have.

So, Ayden Jane has had a full week of the increased gh. Her weight has stabilized and I think she is already growing! I am really excited to say that she is still not needing to eat much in the way of snacks. Life, food wise, is just easy right now and I am really enjoying it. I'm sure it won't last.... sigh, but I am smiling and appreciating every "I'm not hungry" and "sure, a blueberry bite is plenty" I hear.


It really struck me at the pool this evening. She has had lots of great summer activity and was enjoying another evening swimming with her friends. Listening to her get along so great. Having compassion to take an extra turn being it. Standing up for herself when necessary. Negotiating a truce between other siblings. Just happy and healthy and confident. 

Hooray for summer!!!

Thursday, August 4, 2016

Oddity Continues

Today was day 4 of maintaining Ayden Jane's weight at a steady place. Good news. We shall see if we maintain it tomorrow as well.

Ayden Jane ate breakfast of a cheese stick, watermelon and a blueberry bite at her wide awake hour of 6 AM. Then she and Gary ran a bunch of errands and they stopped at Dunkin Donuts. Ayden Jane got a bit of sausage and egg... but just a bit.

By the time they came home Ayden Jane was itching to get into action, so even though it was only 11:30 she went ahead and had lunch which was 2 pieces of Cauliflower crust pizza. Sticking to the strange behavior she didn't even ask for anything with it or eat any fruit with lunch. Typically she plans her day around what fruit she gets at each meal because it is her favorite.

She went out into the neighborhood to find some friends to play with for a while and then around 3:30 the neighbors invited her to the beach. They typically eat supper at 6 so I figured they would be gone a couple hours tops. I gave Ayden Jane a Hint water and a chia bar, and offered for her to take a cheese stick and/or nuts. She turned down the extras and told me she'd take the bar if she needed to... Seriously?

She got home at 7:30!! That means she played from 11:30 to 7:30 with a 100 cal. chia bar.

The other odd thing is she told me she was so thirsty she drank her entire Hint water plus a water bottle from the neighbors. I have never before heard the words "I was so thirsty" come out of her mouth.

We will see what her weight says in the morning. Hoping I managed to get a enough in her to maintain her weight or Dr. Miller will not be happy with me... Maybe the extra gh will build a little muscle :)

Tuesday, August 2, 2016

Labs and a Lack of Hunger

We got Ayden Jane's labs back. Most things are fine but a couple things stood out. First of all was her bilirubin was high and her ALP low. Dr. Miller has decided that since all the other liver enzymes are great, she is going to assume it is some form of error or anomaly for now. We will retest in a month.

Secondly, her IGF-1 level shows she definitely needs an increase in gh. It is her first increase in 3-4 years! She was doing great with levels spot on until the 'winter of infection'. Then her IGF1 level spun out of control. We even had to reduce from the 0.8 down to 0.6 for a while and slowly work back up.

We have felt that Ayden Jane was a little off for a while now and lack of gh makes sense for those things. She is a bit low on energy which is definitely commonly an effect of low gh. Napping a bit during the day and needing breaks when playing.

What is not common is that her hunger has pretty much disappeared. She has significantly cut down on breakfast and sometimes skips it all together. She has not needed snacks and even cut back on the meals she is eating. I have let it roll most of the time but she has dropped about 4 pounds in a month and it has not changed.

Now, you'd think I would be doing the happy dance because she is not hungry and losing weight. What more could a PWS momma ask for?? Well, the speed at which she is dropping makes me pretty certain she is losing muscle as well as fat. Not good.

Typically, doctors do not really want to raise gh if a kid is having trouble maintaining weight or is already losing weight. I mean, common sense says it would be hard on a body to power a growth spurt and lose weight at the same time.

Dr. Miller and I talked through it and I have convinced her that this is actually Ayden Jane typical. She used to get scrawny through the shoulders and have trouble maintaining weight when she was little and needed a gh bump. I just haven't seen it in forever!

She agreed to going up to 0.9 but I will be responsible for tracking her eating and weight to make sure she is eating and her weight maintains. So, if we can keep her weight at 83-84 pounds and she grows a bit along with rebuilding some lost muscle... Wow will we be in great shape. My guess is we may need to go up to 1.0 but we can decide that in month when we recheck labs. 

Saturday, July 30, 2016

Interesting Summer

Obviously, if you are reading this blog you know a bit about Prader Willi Syndrome. The 'hallmark' of the disorder is supposed to be hunger ... insatiable hunger. This hunger is supposed to start at varying ages but certainly should be a part of Ayden Jane's life at this point.

We have seen the PWS hunger a few times. It generally comes when she is ill. I still don't quite understand why that is the case, but it seems Ayden Jane is not alone in her response to illness. I also see her misinterpret tired for hunger. Even those of us without PWS do this to an extent. You know, the late night munchies when what your body actually needs is to go to bed? Well, just magnify that by a ton.

Ayden Jane has learned to listen closely to her body and interpret the cues well. She refers to hunger as her brain being hungry or her belly being hungry. Her brain needs a snack sometimes between meals. She says she is not really hungry, but her brain starts to crash so she needs food. Then at meal times her belly is hungry. She is doing great with it all and has learned to recognize the brain crashing feeling before it's crashed too much and realized she only needs a small snack to be right back on track - like a blueberry bite or keto chocolate.

The interesting part this summer is that Ayden Jane is a little off right now. She is just struggling to power her typical activity, is having trouble tolerating the heat, and getting 'stuck' more than usual. It's not extreme or anything, just enough that I feel like I am banging my head against a wall a few times a day. Most people probably just notice that she is a bit slower getting her thoughts out. We are working on it and Dr. Miller has labs that will hopefully give us some clues.

I am really curious, though, because usually when she is struggling to power her little body and brain, it sets off hunger cues. This time, however, she is not hungry or concerned about food. She has cut her breakfast back to half of what she typically eats. She sometimes has a morning snack and sometimes not. She is in no hurry for lunch... It is so strange. I have had to remind her to eat. Over the past 2 months she has even lost 4.5 pounds. I know that does not sound like a lot, but it's pretty significant for a kid with PWS.

It will be interesting to see just what those labs tell Dr. Miller.

Friday, July 29, 2016

Summer

It's been a quieter week this week and I have to say it is nice. Another huge flurry is coming soon just as the school year is right around the corner. We are catching our breath a bit and Ayden Jane has remembered how to entertain herself when she does not have a pack of friends around. I enjoy listening to her talk to her babies and pretend all sorts of things. She has also managed to get some extra rest which is good because I think things are really going to get crazy soon!

I have been waiting to get a summary of what Dr. Miller thinks about her recent labs, and of course, that can't come soon enough to make me happy. A couple things were off a little and likely they mean nothing but.... a mom always wonders.

It has been HOT. Not so much that the thermometer has said sky high numbers, but the humidity of late summer in South Carolina hot. It has made activity tough. We have all but stopped running, we do manage a bit of walking with the dogs but even that has been cut short. The pool is so warm that it is even more sleep provoking than anything.

Ayden Jane had art camp in the mornings this week so any chance we had to get out and moving was pretty well blown in the morning. Evenings just don't cool down quickly enough.

I was a little concerned that her weight would begin to climb as we chilled out in the air conditioning but she is still holding steady. I can hardly even write that sentence. I really do not like have to think about the weight of my 8 year old and it is such a tricky balance to help her watch it without getting worried. I am not looking forward to the coming years where it will be a bigger deal among friends...

I will say that she is doing so great as it comes to food. She is just not that hungry lately and has cut breakfast way back. She is sometimes has a morning snack but I actually had to talk her into one the other day before art camp. I really did not think she had enough in her to make it through. One day this week she had lunch at 12:30 and then turned down supper at 5:30. She did not eat again until breakfast the next morning. All her choice...

Her swim team had an ice cream party that I did not know they were going to have so I had nothing for Ayden Jane. I panicked a little because I thought she would get upset but nope. She just said she didn't care about the ice cream, she just wanted her medal and ribbons for the season. I don't know what she would have done if I had not been there but I like to think she would have said no thank you.

Saturday, July 23, 2016

Lots of Randomness

Today was the last swim meet of the summer. Ayden Jane enjoyed it all I think, but honestly, we have been so busy she didn't make many practices. And of course there was the whole iron thing...

She swam in 2 meets before today and her best times were recorded on the heat sheet. I have not kept track in the least so I relied on it for my reference point. Ayden Jane dropped her time by an average of 5 seconds in each event. I wish I could say it is because she worked hard and was improving her stroke ect...  I suspect, however, that it was the iron.

She played on the playground between races and at one point the kid even did several monkey bars!! The hard ones. The ones that aren't actually bars but are hanging rings. She has NEVER come close to that. Iron anyone?

Still fun to be had this summer as she has art camp next week in the mornings and a couple friends who want to play in the afternoons. She also needs to be brushed back up on school work as 3rd grade is coming, like it or not!

Looking toward fall, I'm struggling with deciding what activities will be good. Ayden Jane wants to do EVERYTHING, of course. I can't handle being her personal cab driver and she will need time for school work. Have I mentioned I hate homework?

So far it looks like the travel soccer team she wanted to play on will not have enough girls. There is an extremely strong group in her age and they were trying to get a second team that she would fit well on. Since that is not looking good, she will likely want to play rec soccer again, which is fine. Then there is swim team. She has her eye set on a year round team that is a bit more intense than the one she is on - mostly because her 'best friend' is on that team. Not sure yet if I can handle that commitment.

Next, she wants to continue golf. Only problem is, we heard from the golf coach that Ayden Jane has not been coachable. Not really surprised as she would argue with a rock but... not wasting the money if she is not going to take instruction. Lastly is the girls on the run program. I would love for her to do that but she is not super excited about the whole running thing right now....

It's hard because I know the struggle with running was the iron, however, if I tell her then she will get very upset and it will take forever for her to trust me again. I'm hoping that instead we will just get a good few runs in and she will be back to loving it.

Saturday, July 16, 2016

So Slack

I have just not made time this summer to keep up with the blog. I'm not sure how this summer got so busy!! Ayden Jane has gone to 2 weeks of soccer camp (more or less), flown to Boston, gone to space camp, done vacation bible school, swam on swim team, goes to golf camp next week and then art camp the week after!! She hears about something and she wants to try it.

Between, or before, or after camps we have spent plenty of time in the pool and a fair amount of time on the beach. Need more beach time... I should be able to make that happen :)

Summer has gone well but I did learn something. Apparently, iron is really important for Ayden Jane. Here's how I know. Summer hit and mornings became more casual. I did not do as great a job with supplements and iron is one that I skipped more frequently. Still not a problem. Then I was just about out before I sent AJ to Boston with Kayla but wasn't worried. It was just a few days. When they got back I gave Ayden Jane a few days here and there the next week what I had left and then just never got around to ordering more. I mean, it's not like she ever tested deficient.

Over the past two weeks Ayden Jane had become very high maintenance. She was tired, but I just figured the crazy schedule was catching up to her. She was argumentative - I mean more so than usual.  To an extent she was just mean and over emotional. Also her running was not going well. Her stamina had crashed and she was really upset about it.

I was still clueless.

It was time for labs so Dr. Miller emailed me the lab request.When I looked over it I saw iron on it. I suddenly remembered I never ordered the iron. Two more days of behavior, tiredness and a lack of fun. I got her labs drawn and then the iron came in.  Honestly, I didn't think the stuff we were dealing with would be iron related but sometimes you just feel like you have to try something... anything.

In 24 hours she was a totally different kid! Happy, easy going, full of energy, and reasonable. She has stayed that way for a couple days now.

When will I learn?? Don't mess with this kids supplements.


Tuesday, July 5, 2016

Boston

This is very late in coming. I guess I get a pass because this past week has just been a blur all the way around with the passing of my Dad.

Kayla had planned a trip to Boston to visit the famous Allie. (her college roommate whom we consider an additional daughter). At the last minute - as in during the day Kayla was to leave - Kayla and I decided to send Ayden Jane with her. Kayla is brave!!!


It was a Thursday night flight, not departing until 10 something. Then it was delayed... Ayden Jane was determined to stay awake to get on the plane. She made it and off they flew around midnight. It was Ayden Jane's first flight and she did great.

While in Boston the girls spoiled her. They went to a lake and kayaked. They took her to a water park. They saw finding Dory.  They walked Allie's dogs. Those were just the highlights.

I hear there was a pretty good meltdown on Saturday night. but I'd say AJ gets a pass considering she was up so late traveling and had such a huge couple days.

As for food? She was awesome. The girls had no problem with her and the choices she made. They ate out frequently and ask Ayden Jane says, "just made good choices." The day after they arrived home I had Ayden Jane 'check her number' and she had actually lost 2 pounds while they were gone. Sounds great to me. I mean, who loses weight on vacation? Ayden Jane was so proud of herself for doing a great job with food and exercise and all without Mom! (she even wanted me to email dr. miller and tell her)

To top it all off. she arrived back at MB airport Monday morning at 7:20 and the first thing she said was, "mom, what time is soccer camp today?" She had to get up around 3:30 in Boston so I figured she would skip soccer Monday. Apparently she had other ideas. It was one of those ideas she got in her head and there was no stopping her. I'm pretty sure she was completely useless at soccer, but she made herself physically stay awake and be there. She's nuts.

Thursday, June 30, 2016

My Dad


My dad passed away last Tuesday after a long battle with Alzheimer's and today we held services to celebrate his life. Yep, that's him, my mom and me when I was right about Ayden Jane's age. That's pretty much how I remember him from my childhood. He was the strong, silent type who loved and cared for his family, served his country, and laid a strong foundation for many things. The sort of man that this world needs more of: hard working, honest man with integrity and a desire to put others first. He had an iron will that I see in all his grand children, but especially in his kindred spirit, Ayden Jane.


Dad loved all his grandkids. He became much more talkative after he retired and most of what he had to say involved stories of his grandchildren. He was proud of them. He enjoyed them. I think his smile was bigger when he was with them.


 Ayden Jane came on the scene not too long before the Alzheimer's began to accelerate. The Marine, loving father, adoring grandfather and ultimate protector/provider saw the fighter in this little one. He delighted in her, I'm sure prayed for her and somehow bonded her to himself in a special way.

As the years passed, Ayden Jane grew stronger and fought off PWS as grandpa weaker and struggled more. She began to care for, worry about and pray for him. She enjoyed the last church service he was able to attend.

At the memorial service today, there was opportunity for family and friends to come and share. I told Ayden Jane that opportunity would be coming but did not speak with her about what she might say. She told me she has something to share but didn't want to tell me what. So when it was her turn, Noah helped her with the microphone ect. (and I held my breath because, well, who knows what Ayden Jane might say)  She proceeded to tell everyone that she enjoyed spending time with her wonderful, wonderful grandpa and said a few other things about how he remembered all of us, I couldn't quite hear. Then she proceeded to recite John 3:16 - For God so loved the world, He gave his one and only son that whoever believes in Him might not perish but have everlasting life.

Yep, that sums up what Grandpa would want everyone to know.

Wednesday, June 15, 2016

Oh Summer

Today's activities were brought to you by summer. At 8:30 Ayden Jane and I went running. It was and easy run, but she rocked the pace. She is doing GREAT. The neighbor kids have joined her on her mission and even though they can't really keep up, all are having fun and Ayden Jane is very motivated by having them join in the fun.

After a bit of cleaning and summer assignments, we ventured off to the library. That sounds all mellow and calm, but when Ayden Jane and I go to the library, we bike there. It is a bit over 4 miles. Her friends wanted to come to so I grabbed a backpack filled it with 3 water bottles, pumped up a few tires, sent Ayden Jane back home for shoes (that's a recurring summer theme...) and off we went. 

It was truly an adventure. Ayden Jane can really go and she was our leader. I know, that sounds a bit frightening. She honestly did a great job though. She has learned how to slow down as she approaches driveways ect and of course, stops at true intersections. One of her friends was right on her heals and the other was lagging... a lot. So I took third slot, and kept my eye on the leaders and tried to be the link with the lagging one. I will be honest, there were a few tense moments as the leaders got a bit far ahead ect... But all in all they did great.

We got home in time for a late lunch and just before the thunderstorm came crashing down. The kids played inside until it was time for Ayden Jane to go to swim team. That's right. She followed up her morning run, bike ride to the library with swim team practice. Oh how she loves summer. The evening included playing with Noah, walking and washing the dogs and a rousing game of jenga with Dad. The grand finale was reading one of her hard earned library books before bed. 

Whew. Asleep around 9:30 but we all know she will be back up at 6:00 ready for action! But will I?

Sunday, June 12, 2016

Soccer Camp

Ayden Jane has completed her first official full week of summer.

In summers past, Noah has coached soccer camp and a few of AJ's friends attended. Ayden Jane tried it once most summers but abandoned ship in about an hour.

This summer she started talking about soccer camp weeks before school was out. She was determined she was going for the whole week and was very excited. Day one came and I was not quite as optimistic, but figured it was worth a shot. Guess what, she stayed the whole day and could not wait to get back to it on Tuesday! On top of that, there was plenty of beach and pool time after soccer camp hours so the kid was going really, really hard.

I'm not saying the she was a super soccer player, or that she even played hard the whole time. She did, however, not get overwhelmed and had a great time. She was excited to go every morning.

Week two of summer will start up our running plan. Since we could not find a race to train for that timed out as we needed, I told Ayden Jane that if she completes the running plan then we could go do something fun of her choice. We will still run with Dr. Miller in November but this is to try to use the summer months to build strong legs and a some endurance.

Swim team also kicks in this week with 2 practices and the first meet of the summer on Thursday night. In some ways her swimming is really coming along, but in other ways (including speed) it's not really improved much. We shall see...

Sunday, June 5, 2016

June

I have no idea how it is June already. The end of the school year always seems to ramp up the level of busy and stress - good stress - but none the less stress. It's just a flurry of excitement and celebration.

We do have a lot to celebrate as far as school. Ayden Jane's last nine weeks was her best and she had all grades in the 90's. She was so proud of her honor roll certificate and the accomplishment. I can honestly say that this year, we took the hard line as far as grades for AJ. She got exactly what she earned, and she earned them without the need for academic support in the special needs classroom. She started the year still a bit behind the other students in a few areas, but finished the year right on par. Writing is a great example. She struggled with even the formation of the letters and spacing of writing at the beginning of the year. It was very hard to read and very hard for Ayden Jane to do. By the end of second grade her handwriting looked like a different child's handwriting. It comes fast as she is writing and spacing is much more natural to her.

I did not realize during the time, just how stressful the end of year is for her. I mean she loves it and all the May Fest and end of year parties and thank yous and homework free nights... but it is had her wound pretty tight.

Ayden Jane loves school so when she asked if she had to go the last 2 half days I said no. She had already completed her work, had her end of year party, awards ceremony... She was done. For the kid who has perfect attendance most every quarter, to ask to stay home really says something.

The last day we went to the beach and she played in the ocean for hours. Wore herself totally out, had a mini melt down and slept. And slept.

On Saturday, Noah took her to Ripleys Aquarium as a sort of end of year treat, although mostly because he just hasn't had a chance to hang out with her since he has been home. Mckenna went as well and the all had a great time. It was so freeing for me because they drove off and I knew I wouldn't get a call, she wouldn't come home with something else to do, I didn't need to worry about food, I wouldn't have to watch the time so I could pick her up... Noah had her.

So here is to the first official days of summer break. Lots of beach/ocean time, pool time, playing and sunshine. Ahhhhh.... just feel the relief.

Monday, May 30, 2016

Fun Times

It has been a while because life is BUSY. End of school year is busy. Mckenna's birthday. Memorial Day weekend with Allie here. The sun beginning to shine and summer fun underway.

Ayden Jane had a wonderful weekend. Saturday she had a birthday party to attend and then was thrilled to play with Kayla and Allie for the afternoon. 

Sunday we had church and then she went to see Jungle Book with some friends. She loves being independent and seems to have done a great job. She went with her friends grandparents who were in town and were willing to take on the challenge of Ayden Jane.

To top off a great weekend, she was invited to go to the water park today! One of her favorite places. I mean, combine water and speed and she is one happy kid. The family that invited her along was going to eat in the snack bar so I packed Ayden Jane's lunch box. My favorite words of the day from her friends Dad was, "oh, they will let us bring her food in. She has dietary restrictions so they will just have to deal with it."  It was music to my ears.

I did prep Ayden Jane both days. She knew she was allowed to have popcorn at the movie but there would be no candy.... Her friends would have candy and popcorn but Ayden Jane needed to be fine with just popcorn. She totally was, but having that conversation in front of the adults in charge of her makes it easy on everyone. I also sent a Hint water to drink.

For water park day I packed a lunch that had several easy to eat things. Cheese stick, blueberry keto bite, coconut bar along with some cottage cheese and frozen blueberries and her milk with MCT oil. I wanted her to be well fueled. That is our best defense. I also used the same tactic and told Ayden Jane with her friends parents there that if they chose to get dippin' dots or another treat she needed to be okay with that and think about how great it is to be at the water park. She answered, "no problem. I promise."

So they left at about 10:30 and brought her home about 5:30. Ayden Jane said they didn't even have to wait in line so I can't even imagine how many stairs the kid climbed today!

Sunday, May 15, 2016

Staying Positive

Whether by nature or learned, I tend to look at the positive. That comes in handy a lot when parenting Ayden Jane. I know, on one hand, what the diagnosis of PWS should mean for Ayden Jane. She should simply not be able to operate in a 'typical' world without help. 

By diagnosis, Ayden Jane should need special education support in school, but she is making great gains without it. She should need support handling situations where there is a lot of food around, but she walks right past the cookies and cakes without a care. She should not be able to participate in sports with typical kids... Special olympics should be more her speed. You get the picture. A million little ways every day....

So, the positive voice in me cheers with each passing day that she brings home a paper from school that she did well on. Cheers with each new skill she picks up in the swimming pool or on the soccer field. Cheers each mission she sets herself on - like running races and training hard. Cheers new friendships and happy days. I live in this positive land of joy and pride and incredible thankfulness to God 90 some percent of the time.

Then there are days when it just hurts my heart that she has to work so hard and there are things that she wants to do, she will never be able to do as well as she'd like.

I struggle with the gift that is Ayden Jane. I know that God made her. I know that God has a purpose for her life. I know that He opened many doors and showed me how to help her body and mind to grow and develop to their best potential. 

Her life is drenched in God's love and mercy. She knows Him in amazing ways. There is no complaint in her about it all, only thankfulness that we have found ways to power her. Concern for her friends with PWS. A desire to change the lives of others. All in a tiny eight year old package.

I pray for God's direction for her life and for His grace as I stumble my way through helping her. May my hopes for her not get in the way of God's plans.

Saturday, April 30, 2016

Latest Running Adventure

So, Ayden Jane has started to talk about running again. She has mostly good memories of last years training and mostly awful memories about how bad she felt running the actual 5k. Running your first long race while recovering from pneumonia is not fun. She ran the first mile okay and then went down hill fast. She was determined to finish, but oiy....

Today we went out a bought new running shoes and then she wanted to pick a training program. She was convinced that the one she did last time was too easy now that she was an experienced runner... such humility.

I was glad to look for something different because the last one was time based and she was so focused on time that her pace was really slow. We googled and a lot of distance based plans seemed to start at a distance that was intimidating for Ayden Jane. I finally came across a plan for kids that seems like a good place to start. Ayden Jane bought in and got super excited. That's really all I need anyway! So here it is:

Run Four Days per Week.

Week 1: Day One: 1/2 mile, Day Two: 1/2 mile, Day Three: 1/2 mil, Day Four: 3/4 mile

Week 2: Day One- 1/2 mile, Day Two: 3/4 mile, Day Three: 1/2 mile, Day Four: 3/4 mile

Week 3: Day One- 3/4 mile, Day Two: 1 mile, Day Three: 3/4 mile, Day Four: 1 mile

Week 4: Day One- 1 mile, Day Two: 1.5 mile, Day Three: 1 mile, Day Four: 1.5 mile

Week 5: Day One- 1.5 mile, Day Two: 2 mile, Day Three: 1.5 mile, Day Four: 2 mile

Week 6: Day One- 1.5 mile, Day Two: 2 miles, Day Three: 1.5 miles, Day Four: 2.25 miles
.
I know it's shocking to hear that Ayden Jane had me out the door for the first half mile run today. But I was actually quite impressed. We have not 'gone running' since fall and I had no idea how she would do. I guess I shouldn't be surprised that she rocked it. Even after we had taken the dogs for a 2 mile walk earlier. she ran the half mile at a 12 minute mile pace. I think that is a pretty great place to start!

Now, this does not mean that we will only take 6 weeks to get back up to 2 miles. The beauty of it is we can repeat any given week as many times as necessary. And, if we don't get all 4 runs in during a particular week, life will go on...   

Friday, April 29, 2016

Organized and Focused

Organized and focused. That's the best way that I can describe Ayden Jane's brain now.

Okay, so maybe she doesn't present as entirely organised and focused, but the improvement is significant and has lasted long enough that I think it is a lasting improvement. Hooray!

I'll try to explain what I mean by a more organized brain. One sign is she suddenly has the ability to recall/retell things that happened during her day at school. It has been fun. She has told me what happened in class, is able to retell conversations and even give me clear descriptions of examples they did during class time. One of the impressive academic examples was in math. I was checking her math paper. It was a sheet where she needed to measure things around the house. One blank was for measuring in ft and one was for measuring in inches. When she measured the table height she wrote 2 ft in the one blank and 30 inches in the other. I wanted to correct her because when I looked at it, all I could think of was that 2 ft is not equal to 30 inches. Ayden Jane was able to explain to me that she was to round each unit to the nearest unit. So, when she measured the table height in feet, it was closer to 2 ft than 3 ft. She recalled and explained an example from class that day. I was stinkin' impressed! Absolutely a new skill.

I guess I call this a more organized brain because it's like she takes things in and files them so that she can retrieve them and apply them quickly later. There had been a gap in her ability to do this sort of thing. She would absolutely take it all in, but it always seemed like it was a bit of a mess in there. Like she knew it was in there somewhere but couldn't quite get the pieces to line up. Sort of like information is supposed to be filed neatly in the file cabinet but it came in too fast so it began to pile up on the desk. She took it all in, but then could not find it when she needed to. On top of that, she found that the messier her desk got the more stressed she got.

It's a wonderfully strange shift. 

Monday, April 25, 2016

Independence

Ayden Jane has always been an independent soul. She is determined and would rather work at something for an eternity than accept help. It can be infuriating, exhausting, exasperating. I am sure it is also a big part of the reason she is doing so great.

Lately, the independence has surged yet again. She has taken full control/responsibility for getting herself together for school and out the door in time to be early for school. Now parts of this are not new. She has been dressing herself, brushing her teeth ... Well, let me just give a run down of our morning routine.

Ayden Jane gets up early. Often before my alarm rings at 6:20. She heads out to the kitchen and grabs a fruit and a piece of cheese. Then she turns on the TV and that's how breakfast begins. As she works on the first part of breakfast, I mix up her avocado and get her supplements together. She finishes all of that by about 7:00. Then off to her room to get dressed while I finish packing lunch and snack. In the past, by the time she got dressed and teeth brushed, I washed her glasses, double checked her back pack, filled her water bottle, found her fitbit and got socks and shoes ready to put on. She would then come and sit down while I 'finished her up'.

Lately, she has taken to getting her own socks and shoes and putting them on picking her snack and getting it in the right compartment of her backpack, filling her water bottle, getting her fitbit herself and washing her own glasses. She is double checking me with her backpack and is just totally on top of things. It's pretty awesome.

At the same time, she has taken to doing her homework entirely independently. Not saying she never asks a question but she knows she needs to do it. She manages her time to be sure it gets done. She gets it out of her backpack, completes it, makes sure her name is on and gets after me for things like permission slips. She is totally on top of it.

I know these things are not earth shattering but they are a difference. They are a kid who is growing up. They show a kid who is not just expressing independence, but is also becoming responsible. I think the recent ability to process things and keep up with things has helped her put the missing piece together. She just doesn't need me or anyone else to do all the organizing any more.

Go Ayden Jane. (but don't grow up too fast)

Friday, April 22, 2016

A Little Too Fast

Ayden Jane is settling in on the b complex. I'd say the best way to describe it all is that her brain is fast. It is fast enough that she has gotten into a bit of a verbal confrontation with another student. Nothing terrible, just that when a comment is made by another student, she is now quick enough to 'come back at them'. She does not know how to do so gracefully yet, particularly with the teenage sarcasm she has grown up with being a youngest. Hopefully this will not become an issue since, up until now, she has been really easy going with peers. Small comments made about any of her quirks have just rolled off or gone right over her head. Not saying that kids have been mean to her in any way, just that kids can be very honest with what they are thinking and are not known for tact. 

Secondly, Ayden Jane's brain is so fast she is now positive she knows more than I do. I mean, she was pretty sure before but now, it's a fact, just ask her. Ugh! Again, it's an adjustment. I'm trying to be patient ....

Thirdly, Ayden Jane's brain is so fast that she now makes mistakes and gets things wrong because she doesn't want to write things down or work things out because, "It's so easy." So, instead of taking a faster, clearer brain and apply it to the steps in a problem, she wants to skip the steps because she is sure that the answer that popped in her head is right. 

So, yes. Her brain is faster but she does not have a handle on how to use it properly! I go back and forth between wanting to strangle her and her new found spark. And feeling bad for her because it must be really be hard to suddenly have a different brain to adjust to.

Thankfully her teacher is patient with the adjustment. 

She is showing some signs that the rewiring is an adjustment. She is seeking sensory input like preferring to spin during recess over play with her friends. Wanting to wrestle at home. Constantly on the go.... But we have seen all this before:) Last night Gary took her out to the ocean and she had a good long boogie boarding session. The ocean has always been great for that sensory piece.

I am still not exactly sure what about the complex is benefitting her. I think that the folate is good, but needs to be supported by the other b's. I also have a feeling there is something to the b6, which she has not recently been getting. I don't have it in me to piece each one out so we are sticking with the complex and choosing to just be happy.

Saturday, April 16, 2016

MTHFR - The Saga Continues

So far what I know is that Ayden Jane rocked on the b-complex. She was on it for a week and going strong. Fun new super powers seemed to abound. Yes, she was getting a bit tired from time to time but the sort of tired that comes from going extra strong and feeling extra good.

Enter the fall. I decided to add an evening dose. Only, I thought that it should be in the form of straight folate. I believe now, that the folate alone was not a good choice. I mean, I can't say for certain ... but it was UGLY.

The chain of events goes like this. Tuesday a week ago the complex came in the mail and I gave it to Ayden Jane in the afternoon. By Wednesday afternoon the great reports started and the super powers began to show. A week later on Tuesday evening I gave Ayden Jane the extra dose of straight folate. Wednesday. eh. Thursday, not great. Friday, complete unraveling. What did it look like? Arguing, agitated, crying, not able to reason, miserable for all...

Needless to say I skipped the dose Friday night and she went to bed EARLY. She slept off a large portion of the issue and I dedicated Saturday to calm, non stimulating activities. AJ stayed in her PJ's until 11. That has never happened. Then we rode bikes to the library and checked out books. (That's about 4-5 miles one way). After a snack we took a long dog walk and talked. There was lots of giggling today which was a total contrast to yesterday. Finally, after supper Ayden Jane just played with her toys and enjoyed a quiet evening. I didn't even turn the TV on.

Now I need to decide where to go from here. I think (and remember, I'm making this up as I go so I could absolutely be wrong) that the straight folate alone, even at a low dose like 400 mcg is just somehow overwhelming to her system. I have begun to attempt to plow through all the info I can find on the subject and I'm amazed at the interactions and balancing act of all of this. It may be that I just stumbled onto a good starting place for Ayden Jane and it would take a lot of work to figure out what the perfect balance of all the b's is. I'm not sure I have strength for that.

Instead, I plan to keep the daily morning B complex that I have started with and possibly add a second complex every other day or so. The idea of 'pulsing' was mentioned in something I read and it somehow made sense. Anyway, so far the b complex does not seem to keep her up at night like it could have the potential to do. I had started it at night and moved it to morning to prep for the added dose so at least I know that much.

Honestly, I felt so horrible last night. I mean, I had dosed her into a miserable frenzy and then sent her off to school to cope with it all. To top it off, I was slow to pick up on what was going on and fussed at her for her poor behavior - which I had caused. One of those parenting lows. I will be glad when this is all sorted out and I am confident that Ayden Jane is running at optimal effectiveness and then I am taking a break from mad scientist/dr mom.

Tuesday, April 12, 2016

In The Lead / Cutting Edge / Leading the Pack

In a lot of ways life tends to be a race or a competition. Not saying that it should be, it just sort of ends up that way. We often strive to be 'In the Lead' or 'Leading the Pack'. Who wouldn't want to be on the cutting edge. It sounds like the perfect place to be.

I am thankful for those who lead the pack. In Prader Willi Syndrome, someone led the way by pushing for and administering gh to their child. Over time, people were willing to push the envelope and start gh even younger. There were no guarantees even if it was all based on the best possible science. Those pioneers proved to be correct and the administration of gh is now standard of care for PWS.

Every clinical trial that comes down the pike requires people to take a step in faith as well as science. Science says if we give a person ___ then it will improve ___. But. Somebody has to be the first to try it and learn as they go how to best dose and avoid side effects and, hopefully, prove it is safe.

Like it or not, Ayden Jane tends to be toward the front of the pack. I tend to seek out and try new things as long as the science makes sense and I can be assured of the safety. She was the youngest and one of the first to go high fat/low carb. We figured it out as we went. We didn't even know that she was in ketosis for years, just liked how her body and brain responded and let Ayden Jane show us the way. So much science to explain it all has caught up and thankfully, we were pretty spot on as it turns out in the end.

We began using B12 as it was just an idea. I remember asking my pediatrician about it and him telling me he could assure me it would not hurt her but he did not expect it to make much of a difference. Oh what a difference it made!

I know there were other things along the way, especially before Ayden Jane was two and we found Dr. Miller, but this MTHFR gene and folate thing leave me feeling the same stress as those decisions in her early years. Read, reread, think, ask questions ... Decide on a plan and a supplement - don't forget to research the source and quality of the one you settle on. Now take your best shot at dosing. Then watch the ticking time bomb. Take great notes along the way for the next person addressing the same issue. Try to interpret what you see and adjust timing, dosing .... all over a fairly long time since each change needs a fair chance of at least a few days or week.

Rinse, repeat... It's feels like being on high alert. Sometimes I really wish there was a manual that would just tell me how to keep Ayden Jane operating at optimum levels. Until then, I guess we will continue writing her manual as we go. Maybe it will help the rest of the pack one day.

Monday, April 11, 2016

Public Service Announcement

This is going to be a bit challenging to write without sounding whiny or being offensive, but here goes.

This week was report card time (again). I have four kids and the first three were always straight A students in grade school. I can honestly say I did not post about their accomplishments, (then again, I don't think facebook was a thing) pay them for good grades or other sorts of things. They whipped through homework, never studied for anything and were always rewarded with good grades for their academic abilities. Notice, they were rewarded with good grades for their academic abilities, not for their hard work. Honestly, school was not hard for any of them. I felt it was their job to do their best in school and for that, I would tote them to the extra curricular's they loved ect.

As school became harder we did a few small things to recognize hard work. Whether it was a particular subject or struggling to manage time or stay organized.

Insert Ayden Jane. I see the constant photos and accolades of parents to who are celebrating their students "hard work" and resulting honor roll. Things are in a whole new light now. Even though I was more of the grouchy parent who just expected that the kids did their best in school, they were always on the list for a reward of some sort. Many of the awards they won had little to do with hard work and a lot to do with being smart kids with all the advantages of a supportive home and a fully working neurological system.

I mean, in all honesty, I think there is a lot more reason to celebrate Ayden Jane's accomplishments of B and C in her academic classes in second grade than there ever was for my other kids straight A's. Well, if you are truly rewarding hard work. I could ask for more accommodations and even modifications to the curriculum in order to improve her grades. I could have her sent to a special needs classroom and given alternate instruction. I could do my best to demand an aide for her.... But all of these things would simply be so that she could get A's and B's. I don't know that she would learn any more than she is now and I am sure she would not be working as hard.

So for the families who's kids truly work hard to receive those good grades than congratulate away. I would, however, say be sure the message to your kids is clear. You are proud of them for working hard, learning to use their talents and being the wonderful little people God made them to be. Sort of like, don't let them get caught up in feeling like you love them and are proud of them because of the grades they achieve. All kids are so much more than grades and eventually, they will come across something that does not come easy and the grade may not be an A.

We all know you will still love them to the moon, just make sure they know.

Saturday, April 9, 2016

MTHFR - Dr Mom

I started the new b complex last Tuesday evening when it came. There have been noticeable improvements every day since. Today, it was on the soccer field. Ayden Jane loves to play soccer, but her response time is often too slow for the game. Sort of like her brain and her feet are really far apart and it takes too long for the message to get to them.

There were times when she was playing that she could shorten this connection and get to the ball, but she had to concentrate really hard. Like you see her determined and trying so very hard. Today, her brain and her feet were just connected. It didn't even seem like it was hard. Don't get me wrong, she wasn't suddenly ready for the Olympics or anything, but everyone noticed the difference.

The other curious thing is the lessened need for snacks. She had lunch at 11:30 between games and she did not come in from playing with the neighbors until after 7:00. I had asked her earlier and she said she just wasn't really hungry. So, after lunch she played soccer game, went swimming, trampolined, ran up and down the street with the neighbor kids, rode the pogo stick... Finally, when she came in she announced she was hungry and tired.

She had a chicken leg, a cheese stick and some fruit. Jumped up from the table and said, "thanks Mom, I'm going to bed."  That was it!

Level energy all day, happy, relaxed, flexible, processing quickly....  I LIKE IT.

Of course, where to go from here is my next challenge...

Thursday, April 7, 2016

MTHFR continued

Ayden Jane tested positive for a heterozygous mutation in two places. I have read a fair amount on what all this means, both very scientific and more anecdotal. In the end, I am still a bit foggy.

What I do know is that when the MTHFR gene mutation occurs it means the bodies ability to convert folic acid into a bioavailble form is down regulated. Typically, a person can work around this without too much struggle but, of course, those with PWS already seem to have something going on with the B12. So, if you need folate to properly use b12, but you aren't good at converting folic acid then you are not getting the full bang for your buck from the b12.

Now history shows how significantly Ayden Jane is effected by b12 and there have been lots of parents who report dramatic effects from it. So, I was scared to mess with this. Seriously, it could be wonderful, or it could make her crazy. Or, it could make her crazy on the way to being wonderful.

I looked at lots of options and settled on the Pure Encapsulations B complex. here. I went with a balanced approach because it just made sense to start there. This complex has 400 mcg of Metafolin. My plan is to start with this complex and then add a bit more of the straight folate very gradually over time.

If you have managed to hang in there this long, now it's going to get good!

Day 1 response: I received a text message from Ayden Jane's swimming coach. She has never texted me before so this blew my mind. Here is our exhange:

 "I wanted to tell you how impressed I was with Ayden Jane today. She listened so well, barely had to ask me twice with instructions, was focused and had no trouble keeping up! Really proud of her hard work."

I responded and thanked her for noticing. That we were tweaking something in the nutritional supplement arena and hopefully we are on to something. Her response:

"You are! I thought you may be trying something. She was a different child and I could tell she loved it and was so proud of herself that she could listen and then do it. It was like her brain was awake!!"

Day 2 response: Substitute in school so not sure how that went. Once home, she had a snack and then hit the ground running. We went to the beach and I offered her supper before the pool. She said she wasn't hungry and opted for pool first. After pool her friends knocked on the door so she chose to put it off again. By this time it was about 7:15. She came in after playing and too a shower. Then chose to walk the dogs because, "I still have wiggles." Finally, by 9:10 she was tired and ready for bed, but still had not eaten ANYTHING since 3:30. She just said she was fine and didn't need anything to eat. 

Now I am guessing that this will not last. That she is sort of on a high from being flooded by stuff she has been managing without. It will take time to adapt to a new and improved typical.

Tuesday, April 5, 2016

Spring Break Lessons

First thing I learned on spring break, hopping from place to place, eating on the go... Ayden Jane is a wonderful traveler. I mean, I don't think 8 year olds are typically known for being wonderful on long car rides but Ayden Jane was great. She played, occasionally asked how much longer, waited really well for whatever the next stop was. Between the ipad, a few babies, a magic tree house book and some coloring books she kept herself as happy as a clam.

As for food, I packed a cooler with a few key items for snacks. We had the coconut bars I make and the warrior chia bars I buy. Both are things she eats regularly. We also had some nuts in nice little prepackaged 100 cal packs which are easy to slip in pockets when heading into places. Honestly, she hardly used them though. I also brought a loaf of bread I make and a small jar of peanut butter so we had an easy meal wherever we landed. Drink wise I put in some coconut water and one of her cups so we could do her half coconut water/half water she likes and keep her hydrated.

Along the way we ate at more fast food spots than I care to admit. Among them, Chik fil a, Wendys, Taco Bell, McDonald's and Moe's. Ayden Jane is great at just choosing decent options at each and being happy with that. Early on I was worried about weight gain during out trip. I mean we were still trying to shed the last of the weight gained when I went away. I am thrilled to report that when she stepped on the scale back at home she was at a great spot. In fact, if anything she lost a half pound or so!

We did have one rough day with one MAJOR fit. I think it was a bit of my fault though. She had gone up to bed and as she climbed in she told me she wanted a coconut bar because her "tummy growled". She uses this term occasionally and it must be uncomfortable. I told her I did not think she needed one and she should try to go to sleep. Well, the tantrum that ensued was a doozy. I realized by her lack of control and ability to reason she likely needed the snack, but now was pretty well backed into a corner. I couldn't set the precedent that if she tantrums she gets food.

I rode it out until she was reasonable again and we decided a better way to deal with this if it happens. She agreed to trying to go to sleep for 5 minutes and that if she can't, she can come tell me she tried but thinks she really needs a snack. I left her in bed, telling her that if she could not go to sleep to come ask me with good manners for a snack. I didn't hear from her until morning.

We had one other tough spot when she overflowed a toilet (the handle sticks) and had to freak out over something that was not her fault. You know, something I caused so she did so about clothes.... She recovered very quickly and we went on with our day. In the end, I'd say 2 meltdowns on a week long trip is pretty decent for an 8 year old, PWS or not.

Monday, April 4, 2016

Spring Break - Part 3

We left South Carolina intending to hit Carowinds on our way home. Ayden Jane is just barely tall enough to ride ALL the rides there but had never actually even seen giant roller coasters. We had told her all about it and had originally planned to hit it on the way up, but with Mckenna suffering a nasty high ankle sprain in a soccer game we decided to push it to our last stop.

Around Tuesday, as we were in Wheaton the weather in Charlotte looked terrible for Friday. Dreaded thunder and lighting - nothing stops a roller coast quite like that! I kept watching the weather and finally told Ayden Jane I did not think it was going to work. That we would have to take her there another time. She was sad, but honestly I think she dealt with it really well. Probably because Noah was there when we looked at the weather and he just has a way of convincing her in a way that there is no arguing. It's like magic.

We left Wheaton with the weather still not looking good, but I kept an eye on it. I decided we did not need to make it all the way to Charlotte, just close enough that if the weather looked doable in the morning we could get there. I was exhausted but the weather looked passable so Mckenna and I talked it over and we went for it. Ayden Jane was so excited.

Little did I know that it was one of the best decisions ever. There were no crowds, no lines, no waiting on even the giant coasters. Ayden Jane LOVED every thrill ride in the place. She truly proved to be as fearless as we thought as it come to motion. She wanted more speed, more spinning, more heights.

Mckenna showing Ayden Jane the way.


Ayden Jane's favorite. With no lines she just rode it over and over and over.


Heading up for a giant free fall.


So tall and she LOVED it.

Sunday, April 3, 2016

Spring Break - Part 2

After spending some great time in Indianapolis we headed on to Wheaton (a Chicago suburb). Noah is in school there at Wheaton College, and we enjoyed hanging with 'Uncle Pete, Aunt Fo and H'. Great friends from my college days and their cool little dude. It was a crazy busy week for them but they were so kind to let us crash their home.

We enjoyed catching up on life, letting Ayden Jane and H play, hitting local parks and, of course, Chicago pizza. Noah came by and we just relaxed for a bit.

Our other highlight during our time there was going downtown. It was high on Mckenna's list of things to do and although we didn't quite get to see everything she wanted, we were treated to much more than we expected. We had a wonderful day at Millennium Park, saw The Bean and enjoyed the Chicago Institute of Art. I was pretty lost but Mckenna was right at home and knew more than I realized about artists ect. Ayden Jane was her typical curious self and wanted to take it all in. I'm sure we only scratched the surface of what there is to see and learn there but it was great. We also managed to squeeze in a nice walk down Michigan Ave and view of the lake front.



Ayden Jane and K renewed their friendship. There is something really cool about having friends that share your medical baggage, but with K it is even more than that. They share an awareness of the syndrome and how well they are doing with it. It was funny to listen to them talk numbers of carbs and diet ideas like it's typical conversation for a couple of kids to be having. K is a great kid and I have no doubt he will have Ayden Jane's back for many years to come as they continue to figure this thing out.

This family... words will never be able to express what they have done for Ayden Jane over the years. They not only have the brains and background to further understanding and treatment for PWS. They worked incredibly hard to improve life for K and have shared all they have learned openly, and they care so genuinely for others who walk the same life. Their family has sacrificed time, energy and finances in ways I am sure we will not ever comprehend and the benefits to many, many future families are great.

Saturday, April 2, 2016

Spring Break 2016 - Part 1

Wow. What a week we have had. Mckenna, Ayden Jane and I left Pawleys last Friday in search of adventure for spring break. We covered a lot of miles, enjoyed the company of friends along the way and had a grand time.

First up was a quick visit to get a glimpse of a sweet baby boy. It was brief but a very important part of our mission. Next stop was Indianapolis to visit with the amazing Walker clan!


We piled in on them for Easter fun. Egg hunting, a trip to the zoo - what more could we want? 


Ayden Jane loves knowing other families who have kids with PWS and eat like she does. She has a heart for the ones younger than her and asks often about how they are doing. So fun when we get to visit and she sees for herself the amazing things kids like E are doing. It is also a treat to just do life for a day or two with families who totally get the quirks life with PWS throw at you. Types of food, the need to eat when it's time, reminding to drink, even those darn shots at night. Talk of supplements and doctors and new possibilities on the horizon is good for the soul.

It was a fabulous way to start of an amazing week with my two awesome roadtripping partners.