Jesus Loves Me...

For Ayden Jane, like most infants with PWS, feeding was completely uninteresting. Well, for the first few days she was just not capable. She was sort of off in her own little world, completely disconnected from this world. She was oblivious to the sights, sounds and feel of all that was around her. It is often said that the hypotonia of PWS is best described as the child feeling like a rag doll. I would extend that 'unreal' feeling to trying to get her to eat from a bottle. At first she was just like a doll. Put the bottle in there, swish it around, tap it... anything to get her to notice it was there.

After about a 5 days, she was 'real' for long enough periods that she was able to attempt to eat. I say she was able because she physically could latch on to the bottle and drink a bit. It took lots of coercing and some really cool tricks the nurses taught us, but she could get down a few cc's before we put the rest through the ng tube. As the days went by Ayden Jane became more and more alert and feeding became the only reason we could not take her home. Each feeding was the highlight of my days or sometimes the low point.

I went to feed Ayden Jane late one evening before heading to the Ronald McDonald house for the night. It was quiet and the night nurses were going about their jobs taking care of the babies. I held AJ and began to quietly sing to her. (Now, if you know me at all, you know I don't sing. Certainly not in public anyway). Jesus loves me this I know, for the bible tells me so. Little ones to him belong, they are weak but he is strong... As I hit this line, Ayden Jane began to eat. She kept eating as I softly sang and when I was done she stopped. I started again... she ate each time I hit that line. What an amazing message to me at that time.

Fast forward to this past week. Ayden Jane and I were all curled up in bed to read stories before bedtime. After we read, Ayden Jane asked me to sing a song. This would be Gary's fault as he sings with her frequently (which I love). I was just trying to squirm out of it when I said, "why don't you sing me a song?" AJ decided that was an okay plan. Whew. Then she started, "Jesus love me this I know, for the bible tell me so. Little ones to Him belong they are weak but He is strong. Yes, Jesus love me, yes Jesus love me, yes, Jesus love me. The bible tell me so."

I was transported back in time. I could feel just how she felt in my arms: so helpless, so frail, so weak... but He is strong. I had never heard Ayden Jane sing more than a line of a song without help. How perfect is it that her first complete song would be the one she relied on from the very beginning.

Here are a couple pictures of our little angel from around the house today.

First, playing in the leaves outside. She loved jumping in them and hiding in them.

Here she is after playing with her baby. I promise, I took my eye off her for just one second! Pretty sure the baby in the background gives you a clear picture as to what she got into.

90 Day Eval

I didn't realize that today was our 90 eval. These are not formal evaluations, but an informal review of the goals we had set 3 months ago to see what AJ has accomplished, what still needs work and a chance for me to bring up things I think are important to work on. So, how did it go?

First of all, Jen is so positive that I think she could tell me that the world is ending tomorrow and I would only be able to notice how sunny today is. It's awesome. On the other hand, Ayden Jane is pretty great and her progress always gives us lot to celebrate. (when parenting a special needs child you get great joy over celebrating little things). There were a few technical terms that Jen was very excited about and I was excited about the fact that Jen was excited.

Big skills she now can control, going from high kneel to half kneel, holding and playing there, putting her leg back and switching to the other... Doing the bottom half of a jumping jack. You know, legs together, legs apart, legs together... Crab walking across the room, catching a ball, kicking a ball... There are more but you get the picture.

We are keeping some big muscle work on the list and will be getting AJ to work on building shoulder girdle strength. It is still lagging behind and 'linking shoulder girdle and torso' is the first to go when growth or anything else causes a bump in the road. We are both really excited to see what will come of the next 6 months when summer returns and Ayden Jane can get back on the playground bars, in the pool, on her bike, in the ocean.... She is sooooo craving big muscle work that I am just having a hard time keeping her going. We are adding in pedaling a bike, roller skating and other play skills that are age appropriate.

One thing we both agree on is that we have gotten away from some of the fine motor work. Typically that is taken care of by an OT, but Jen is awesome and handles it all. We will be back to working on cutting with scissors and learning to trace and write letters. Ayden Jane's sensory needs just sort of over powered the fine motor work the past couple of months. It was interesting when Jen was assessing things today how willing to work ridiculously hard Ayden Jane is when she is working large muscle groups. Then when it was time to check those OT skills she becomes a master of avoidance. Yep, she will do a hand stand against the wall until she can't hold herself up anymore, but try to get her to trace an 'A' and she will turn into a silly kid who will do anything other than what you want.

We have some plans to continue to address the sensory needs. (Hopefully we can get her to keep her clothes on) I am thinking they are going to be long term.

Anyway, we have a plan for another great 90 days.

Yes Ma'am

We have been working really hard on manners lately. It comes out of her need to 'get the last word' or just always say something back whenever we say something. At first her vocabulary was very limited and she would respond to pretty much everything with 'no.' To her, there were times when 'no' simply meant 'uh huh'. It doesn't really sound so cute though. Later, 'no' was taken over by 'why'. Me: Daddy is coming home from work, AJ: why. Me: pick up your toys so we can go jump on the trampoline. AJ: no. I finish this tower first. Hmmm. Does she really want to know 'why' or mean to answer, 'no'. I don't think so.

So, after a few battles, we have settled on the southern, "yes, ma'am". Just think of the difference. Me: brush you teeth, its time for bed. AJ: yes ma'am, I need to put my silly bands on first. Yes, she is still retaining some control and not hopping to it at the snap of my fingers, but I can work with that. It just sounds so much more respectful even though her intent is really no different. I guess I have found with all my kids (even the students that I taught) that it works so much better to teach them what to do instead of trying to teach them what not to do. Now, when AJ answers with 'no' or 'why' when it is not an appropriate answer I just tell her that she forgot her 'yes ma'am' and tell her let's try it again. I will repeat my part and she will answer with 'yes ma'am' first.

So on the way to school today I reminded her to use her yes ma'ams with miss J and mrs. E because it makes them so happy to hear it. She answered me, "yes ma'am momma." I, of course told her how nice that was blah, blah, blah. Then AJ told me, "Mom, that was my last yes ma'am. I don't have any left for miss J and mrs. E." She plays this game with hugs and kisses and says she has to go shopping to get more... It was really just a ploy to get out of using her manners at school, but I had to laugh because it was quite ingenious.

Updates

Well, it's going to be one busy week. First of all, we are doing a fund raiser at our local high school on Wed. My big kids did not want to be left out of helping raise money for FPWR, so they have worked out a fund raiser at their school. There is a 'stud muffin' volleyball game which will give us some proceeds and also we are selling muffins and drinks at lunch time as part of pregame festivities. Should be fun.

It is also a peek training week. I will run 10 miles tomorrow, cross train tue. (likely bike), run 8 miles on Wed. and run 20 miles on Sat.

Mckenna starts back to soccer Mon. and Wed. night and has a tournament out of town next weekend. Ayden Jane has speech, music and horses on Tue. and PT on Wed. We have the fundraiser to prepare for on Tue. and do on Wed. Luckily, Kayla and Noah are pretty self sufficient and helpful!

I am still loving the iron. Not sure why Ayden Jane responds so well to the combination of the B12, lots of good fats in her diet and iron, but it is definitely making for a good nervous system.

My favorite part of it all is that she is so happy! Such belly laughter this week. There have been some parts that are a bit annoying like the fact that she is so LOUD and she talks from the moment she wake up until I finally get her to sleep at night. She has not completely adjusted to some new found strength and has hurt most of us at one point while playing hard. She has also had a couple of rough days at school as she can not contain herself, but we are working on it. Hopefully this week will go better!

Ninja Baby

So much is going on. I feel like I am way behind in blog posts, even though that's not really how it works! Ayden Jane has just had such a spurt of, hmmmm, cognition I guess. She has always been funny, but it is just up a notch or two. One example: I told AJ to go get socks so we could put her shoes on. She disappeared down the hallway. She came back down the hall giggling with Kayla's socks in hand. Now, first of all, remember Kayla is 17 so those are some big socks. Secondly, they were in Kayla's top dresser drawer. When Kayla asked her how she got them she laughed and said, "I ninja baby." That is what Kayla calls her when she gets into mischief in her quiet little manner. Ayden Jane was totally just messing with Kayla.

Food has been a big discussion lately. Ayden Jane knows that she does not eat things with a lot of sugar in them. She has/had sort of just accepted this fact but now is questioning why. Same thing for things like pretzels and gold fish. It is not an, I am hungry and I want to eat them sort of questioning, just wants to understand why it is different for her... Of course, her humor is not far away. AJ: Mom, I eat a pretzel. Me: yes, I know, but pretzels are not really good for you so you don't eat them often. AJ: (with a big grin because she knew she was making a play on words) No, Mom I did eat a pretzel and it was very good for me. Note to self, change the way I phrase that one. Now, when she asks why she doesn't eat ________ the answer is, because it does not make you strong and healthy. Let's see her get around that one!

Tue. was the first time we have been to horses since AJ has been on the iron. We were there a short time, even before we got on the horse. A couple of the people that work there were amazed with how sharp AJ was. Little things like, she asked one lady, where is Jenna? She answered, she thought Jenna was on her way but was a bit surprised why AJ asked. AJ pointed to her hand and told her that she had Jenna's helmet... AJ also had a couple of other conversations and I was told AJ was quick, talking clear, funny, really smart.

So why do I think the iron is helping her so much right now? My first observations were that the spurts were very much similar to the spurts we saw with B12. I mentioned this to a friend (who is way smarter than me) and she directed me to research B12, iron and myelin. Totally a connection. I do not yet understand it and am still reading to understand it better, but I do think that there is something going on that is helping Ayden Jane make new neurological connections or at least more efficiently use the ones that are there.

Roller Skating, Almost

I took Mckenna, a friend and Ayden Jane to a local roller skating rink. I wasn't sure how it would work for AJ, but I figured why not give it a try. Ayden Jane was really excited to go and skate. She was thrilled to put the skates on. She did great walking around in them on the carpet. She was chanting the usual, I do it myself, I skate by myself.... They had a little trainer thing to help so out on the floor we went. Too much traffic, too much noise and one fall shortly into it and Ayden Jane was officially, ALL DONE!

Now I am not completely insane. The skating rink has another section of "soft play" that has a giant slide, some jumpy houses and a really cool tree house sort of thing that is really cool. Off we went to play over there and let the big girls skate.

I do really wish she would have had a bit of fun on the skates before she fell. I really think if she would have had a little success first, then she would have popped up and kept skating. Then again, maybe I am a bit nuts for taking a 3 yr. old to a crowded skating rink when she has never actually been on skates before.

On another note, I have backed off just a tad on the iron. I am splitting an 18 mg iron capsule by 3 days instead of 2. It seems to have calmed AJ down a bit and she has been.... peaceful. I did not say calm and quiet, just that she is under control and happy. Sort of an inner peace. Today she skipped her nap, since we were at the skating rink, and was happy all day until I put her in bed at 7. No temper flares, no arguing, no whining... (okay, a little whining). The other thing that Kayla actually pointed out the other day is that her stutter is completely gone. If you look back to the fall, she was stuttering pretty significantly. I didn't really stress at that point, but I can honestly say it is gone and don't even remember the last time she did. Pretty cool.

Random Pictures

First up, Mckenna, Ayden Jane and Sadie playing in the yard.

Next, a dreaded Christmas morning photo... Mckenna bought the wallet for Noah and Noah bought the hat for Mckenna. (Kayla was in Guatemala:(

Kayla returned and they spent cold afternoons over Christmas break playing Wii. Ayden Jane, had to play too of course! See she even has her own "troller".

This one is of the snow storm that cancelled school! Gotta love the south.

This last one is just a sample of Ayden Jane being Ayden Jane. We were outside doing yard work when I realized AJ had done her ninja move and disappeared. I found her inside the house happily playing some game from her imagination I have yet to identify. She brought the green chair in from outside, but I have no idea why she was sitting on it, on the sofa!

Ramblings

Okay, not sure why I feel compelled to spill this but here it is. I guess that's what a blog is for anyway.

Ayden Jane has PWS. Not a news flash, but it is a fact. She is more mildly affected by PWS than many/most people. Again, not really something that will stop the presses. I stay involved in the PWS community because we do share a view on life that is hard to understand if you don't live it. I sometimes worry, though that the incredible success AJ has in so many areas serves as a reminder to many that there child.... rather than as an encouragement. I can truly say that I have fallen for the kids I have met. There is something about them that draws me to them and I love to read of their successes and cheer for them.

On the flip side, we do have struggles dealing with life with PWS. Just different worries... Ayden Jane is up and running and independent, but that just gives her the ability to get into more. She is in preschool and loves it and is social. She is invited to parties... All things I hoped for, but they lead me to try to figure out how to let her be 'typical' without actually being typical. How much do you tell people. I don't want to scare them off, but I need to be sure AJ is safe. I am thrilled it looks like AJ will be in regular classes as she grows, but how will she handle the social nuances of middle school. That is hard enough with out the added challenges. You get the picture. Life for us from the outside, looks like Ayden Jane is no different than any other 3 year old. Again, I am ectastic! But, how crazy do people think I am when I limit the foods she is eating...

She has worked so hard to keep up. I just want her to be able to the life she has worked so hard at and not sound like I am a constant dark cloud.

Iron

Here is a post I put on the holistic pws site:

We have recently added iron to Ayden Jane's supplements. Actually, we tried another form a while back when potty training because her stools are/were quite loose and I was a bit afraid of potty training that! It was a liquid form from our pediatrician but it stained her teeth horribly so I just quit, potty training was successful and I just left it there.

She is very independent with pottying (and everything else for that matter) and loose stool and a 3 yr. old, well you don't really want me to go there. I decided to give the iron another go and am using the ferrochel form of iron. It is an 18 mg capsule and I am giving her 1/2. I actually put the powder inside a little ball of almond butter. She thinks it is a grand addition to her breakfast!

It has been about a week and the original reason, the bathroom, has been successfully improved. Woo Hoo.

What I did not expect was the increase in energy. I know, if you know AJ, how in the world could her energy increase. Just know it has. She is also responding to things very quickly. It reminds me of the spurts we noticed from B12.

Her language is more clear. My 17 year old even commented on it this evening. Now, this may have nothing to do with the iron as she had a nasty cold around New Year's and may just finally be back on track. She is also talking NON STOP, but that is likely just the energy talking. I do feel like she is better at 'retelling' events of her day, but that is quite subjective ....

The really strange one is that she is suddenly drinking. I mean downing beverages like she has never had liquid before. I gave her a glass of almond milk in a 'big girl cup' (no top) and she drank it straight down almost choking herself because she drank it so fast. Now I don't normally have to chase her around the house like some folks to get her to drink (sorry Ali), but she is definitely a sipper. Might finish a drink in the course of a meal... She has even taken her cup to the refrigerator and filled it with water herself and drank it all straight down. I am not sure what to think of this, and am sort of expecting it to fade out over time but... any thoughts? Good thing or bad thing?

We will go see our local Endo Feb 1st and run it all by her and then we are participating in Dr. Miller's sibling study in April. I guess we will have blood levels well checked and I sure hope it all looks great because I am loving the boost it is giving her and the nice clean bathroom.

I would like to add that Jen came today. She was impressed with her stability through her hips and noticed within minutes that AJ was doing everything VERY fast. A little too fast at first. She was also surprised how much, non stop in fact, she was talking. Hmmmm. I guess time will tell

Marathon Fundraiser

I have been quietly running in preparation for the Myrtle Beach Marathon. It is coming up on me quickly and, as usual, I am wondering what I was thinking when I signed up for this thing!!

Anyway, there is just something so appropriate about a marathon as a symbol of a successful life with PWS that just makes sense to me. They both require discipline, patience, dedication, commitment... There are goals set along the way and reaching them is cause for celebration. You get the picture.

Over the course of the next couple of weeks my training will peek with 2 more runs of 20 miles a piece as part of the training schedule. It is grueling, but I do enjoy the time to think and reflect and feel like I am doing what I can. Hey, I would like to have the brain to solve this thing on my own, but at least there are amazingly brilliant people out there who are zeroing in on this thing. I figure if I can give them a few dollars to use their giant brains on Ayden Jane's behalf than I run I will.

If you are willing to join me on behalf of Ayden Jane or any of the people out there with PWS click on the link (hopefully I will attach it right)

http://giving.fpwr.org/contributions/users/Running%20for%20Ayden%20Jane/running-ayden-jane

Ayden Jane Quotes

Lately Ayden Jane has just had some hilarious quotes. And fairly long conversations. I never think about writing them down and I usually forget them by the time I go to blog, but I am going to give it a shot.

AJ: Where Daddy going?

Me: He's getting shoes to take you to McDonalds.

AJ: Why we going to Donald's?

Me: So you can get apples and play and Daddy can get tea and talk to Mommy.

AJ: Daddy take me with him?

Me: Yes. Daddy won't leave without you.

AJ: Daddy always take me with him cause he love me.

Kayla was in her room and Ayden Jane came in with big news.

AJ: (holding a slinky) It looks like a silly band BUT, (pause and grin) it's a slinky.

Playing on the trampoline.

AJ: Let's play a game.

Me: Okay, what should we play?

AJ: Freeze.

Me: How do you play that?

AJ: Stop jumping when I say freeze. Okay? Now start jumpin' dawg. (way too much time with my teens)

After I picked up AJ from school.

AJ: What we gonna do now?

Me: How about we go see Grandma and Grandpa?

AJ: Sure, that's a great idea. Grandma have apple juice.

Me: I am sure she does.

AJ: I push the button? (door bell)

Me: Yes. You can reach it all by yourself now.

AJ: Whose gonna answer the door, Grandma or Grandpa?

Me: I don't know. If they are home, one of them will though.

AJ: Mom, we are going to visit, of course they will be home.

Me: Well, they don't know we are coming to visit.

AJ: Can I play a minute?

Me: Yes. We can stay a play a minute.

AJ: I will play. You talk to grandma and grandpa...

While doing a floor puzzle.

AJ: Mom. Whatcha wanna talk about?

Just a small sample, but I have to say that days like today, I tune her out. If I didn't I would go crazy. I don't think she stopped talking from the moment she woke up! I know, I know and trust me, I am ohhhh so thankful.

Use It or Lose It

I am really not sure what is going on with Ayden Jane but I do think there is an element of 'use it or lose it' with her. We have just finished a great, relaxing Christmas break with zero therapy. Yep. ZERO. It was too cold so horses was cancelled 2 weeks prior to the holidays and then we had the 2 weeks over the holidays off. A month off the horse. Our PT always takes the 2 weeks over Christmas off to be with her own sweet ones. I even allowed myself to not really do our own home therapy. Ayden Jane looked awesome right about Christmas and I just got a bit lazy.

Around New Year's weekend I noticed AJ's posture looked poor. Her belly started sticking out again, she began hyper extending her knees again, W sit came back with a vengeance... Not that these sorts of spurts are entirely unprecedented, but it was such a huge swing.

I knew Jen was on her way to save the day today. She took one look and agreed her belly is/was completely turned off. It is like the tone just shuts down even though the muscles are all still there. I still don't get it. grrrr. Anyway, when Jen came through the door the first question from Ayden Jane was, "did you bring mr. potato?" (okay, I know that is irrelevant but hang with me.) The next thing Ayden Jane said was, "you bring more tape for my legs?" She hasn't had her legs taped in at least a month, but she asked for it, got the blanket to lay on (just like we did it the first time) and hopped down on her belly. She was hilarious after the legs were taped just watching how she loved the feel of her legs/body with its new support. By the end of the hour her belly looked a bit better. Then she was wild woman for the rest of the night, climbing everything and getting LOUDER by the hour. She went to bed easier and happier than she had been in days... It was like she had regained control of her body and was 'centered'.

So, after 3 days back at school, returning to horses, speech therapy and PT, AJ is more energetic. It really is like activity and energy breed activity and energy for her. I am guessing it has more to do with neurology than anything else. She has made amazing neurologic connections for a kid with PWS. This is shown in the fact that, most of the time, her tone is good, her motor planning is great, her balance it good, she is doing great in the speech area, she is not delayed cognitively, ... I guess, somehow, those pathways just need constant activation or they are still prone to 'turn off.' Maybe someday she will get beyond this, but for now, there is no more getting lazy!

I am definitely going to have to enroll AJ in some activities as she gets older. I think either swimming or gymnastics are my best bet. I am a bit biased toward swimming due to cost and proximity, but not sure I can find much for a 3 yr old. I guess to the gym I go to see if there is anything that will work for her and our budget.

Phases/Stages of PWS

I am beginning to think Ayden Jane could be entering phase 2A. Hmmm. Not sure what the phases of PWS are? Not sure I understand them either, but here is the best description I have found. It is off a list serve I am a part of:

1a difficulty feeding (age 0-1.5)

70/108 kcal/kg/day

1b no longer having feeding problems (age 1.5 - 2.9)

Mean age of starting this phase is 2

66/102 kcal/kg/day

2a weight gain without increase of calorie intake (2.5 -8.5)

Mean age of starting this phase is 4.5

45/96 kcal/kg/day

2b weight gain with increase of cals and appetite (age 3.75-13)

Mean age of starting this phase is 8.

45/96 kcal/kg/day

3 hyperphagia, lack of satiety (age 3.75 - adult)

24/29 kcal/kg/day

4 appetite decreases, similar to 2b (adulthood)

20/29 kcal/kg/day

Only thing that makes me think it is possible is that Ayden Jane still is not eating much. I don't have to be sure and feed her every couple of hours. She leaves food on her plate at meals and turns down foods she previously would eat whether 'hungry' or not. Just seems to be a decreased need for calories. This has stayed steady for over a week now and I don't think she is loosing weight. She is still alert, active and acting pretty normal. (whatever that is for a 3 yr old.)

I will be going to see Dr. Miller in a couple weeks for a sibling study so I am sure we will sort it all out. It really just reminds me of my other kids who just went through phases where they ate lots and then weren't so interested. Sort of like chowing down when gearing up for a growth spurt. Then ....

I think it is encouraging that AJ seems to be aware that she does not need the added food right now. So far I am still able to trust her sense of hungry... to some extent. It has been sort of nice not to have food be such a huge part of our day lately!