Sunday, March 31, 2013

Crud Update and Happy Easter!

So far, AJ is still a bit gooey but is not showing signs of infection.  Her nose has stopped pouring forth and sounding so plugged, but the super thick cough is not exciting me.  Just listening to her often makes me want to cough.  She has this thick sound in her throat but it just doesn't bother her so she doesn't cough.  When she finally does trigger a cough I am amazed at the sound and the amount of crud that is in there!!  We are coming up on a week of this stuff tomorrow so at the first sign of it going awry this week we will be sprinting to the doctors office.

Today is Easter!  It has been great to hear AJ ask so many question over the past weeks about the Easter story.  She is very aware of Jesus's death on a cross and what that means.  She is even more aware that Easter Sunday is the recognition that he rose again on the 3rd day and defeated death.  It is fascinating to listen to her little brain try to process it all.  She has asked lots of questions about good guys and bad guys and why anyone would want to be a bad guy or why bad guys want to hurt good guys.  Lots of questions about if a bad guy can ever turn into a good guy and how that works... What happens to bad guys when they die... Why Jesus had to die.  How did he become alive again and where does he live now any ways?...  She 'asked' me, "so God used bad guys to kill Jesus on the cross so he could  forgive all the sin we do, you know sin is the bad stuff, and that made it so everybody  can be good guys and go live with him after we are dead here?  There had to be an easier way."  Hmmmm.  Quite a few theological errors or at the very least things askew, but I'd say she is at least gaining a foundation of faith.  

Wednesday, March 27, 2013


Labs were drawn for Ayden Jane last Thursday.  I got the results Monday.  Not awful, but no what I wanted to see...

Apparently her eosinophils are up again.  That is a marker of some allergic reaction... I am guessing it is the pollen running a muck so not too stressed over that one but back on our allergy meds.

White blood cell count is a bit high as well.  grrrr.  Something brewing??  Very well could be as by Sunday I started to hear the stuffy nose and yucky cough coming out of AJ.  She is really congested today (Wed.) and if it keeps up we will hit the pediatricians office by weeks end.  Dr. Miller agrees to bump up the PharmaNAC too.  It just helps break it all up and she is better now at getting the loose junk out of there.

IGF-1 is slightly high so there will be no increasing gh this time around.  Of course, if she truly is sick again than it could be elevated from that...  

B-12 looked great.  Bascially there are 2 different measures for B-12.  One is a sort of ratio: Hgb/Hct look great (13/38.2 - right in the middle of normal).  The other way to measure B-12 is a straight level: B12 is >1500 (normal 156-914).  Dr. Miller says to not even bother testing the straight level as it is almost always elevated even in kids with no supplementation.  It's a weird PWS thing.  The ratio is the measure she wants to see if oral supplementation is enough.  Good new for us is that we can continue to supplement orally as it is being effective.  So, at least for now, I won't need to add B-12 shots into the picture and can rest assured that AJ is getting full benefit of supplementation.

So, we have ALMOST made it through the winter without illness.  Lots of yuck is loose and coming out of AJ today so I am hopeful that we can get her to clear it out without heading down the infection path.  (Prayers welcome!!)

Biggest struggle is that when sick like this all she wants to do is SNACK.  It is soooo not like her typically and I wish I could just not worry about it for a few days, but alas that is not how it works in the PWS world.

Sunday, March 24, 2013

Scooter Video

So, Ayden Jane spends a lot of time hanging out at HS and club sporting events.  Currently it is soccer season and for high school this means stadiums.  At our home field Ayden Jane feels quite at home and scooters and runs around like she owns the place.

Last game she took her scooter and found the handicap ramp a great place to ride.  She went down the ramp and then followed the side walk all the way to the end (probably 100yds) and then came back.  Over and over and over.  It is pretty amazing how good she is on that scooter!

Saturday, March 23, 2013

Growing Up

It has been interesting lately to realize how much Ayden Jane is growing up.  It  seems to happen with all kids.  We, as parents, have a front row seat for the amazing development of a totally dependent baby changing  into a hopefully, capable and independent adult.  It is a process for sure!  Even though we see them every day, sometimes we just don't notice the changes.

A few things that I have noticed over the past few months with Ayden Jane mark, to me, significant signs of growing up.  First up, now when she wants to help it is actually helpful.  I love it!!  She is goal oriented by nature so has always wanted to help, but often.... not so helpful. In fact, her helping often let to spills and crashes and way more mess than I had in the first place! LOL.  Today I was picking up and cleaning up and getting ready to vacuum.  Ayden Jane chipped in and then went and got the vacuum.  She used the attachment to clean the furniture for me and even put it back together.  She did a great, thorough job.  (not the look how fast I can do this while tripping, dropping....)

Also, when she cleaned her room, she wanted to do it herself.  She decided to clean up all the babies then call for me to come help.  She took a looooong time so I figured she was just playing and had abandoned the whole cleaning process, but she had managed to play and clean and had all the babies and their things organized. (Well, lined up and grouped!)

Another is in regards to food.  Ayden Jane has a snack at school each day.  I send in a snack of mixed nuts and blueberries.  It is the same snack she has had every day since she started school 2 1/2 years ago.  The other kids eat various snacks sent in by parents.  Sometimes it is things AJ eats and sometimes not, but AJ was always way more comfortable with having her own from home no matter what the other kids were eating.  Recently she has mentioned that what the other kids have is ________ and I can eat that...  We talked through it and I explained to her that she may have the other snack if it is something she eats, but she may not eat her nuts as well.  She would have to choose.  This didn't go over well.  Finally, a couple weeks ago at school the snack was a cheese stick.  Ayden Jane asked her teacher if she could have the cheese stick.  Her teacher answered (I prepped her) that she could have the cheese stick or her nuts but not both.  AJ told her she wanted the cheese stick and then asked if she could please put her nuts out on her hook (out in the hall where she can't see them and can take them home) so she won't think about them.  Since then, about once a week she makes this choice and has no problem.  She knows what she can eat and has not even asked to eat the snacks she does not eat.

Wednesday, March 20, 2013

Glasses and Labs

I have been slack lately so I am going to try to post a lot more regularly.  First up, eyes.  Bad news is we lost Ayden Jane's glasses.  Well, actually, I lost AJ's glasses.  She is great about giving them to me or putting them in a safe place... when she takes them off.  I am pretty sure she handed them to me but I have NO CLUE where they are.

Good news is when we went to see the eye doctor today it looks like her eyes have not changed since last Sept. when we had them checked.  I don't know that it makes any sense that the events of last winter effected her eyes, but last summer when her tone was still low and the scoliosis popped up was when I started to notice the right eye occasionally wandering in.  It was time to have the eyes checked anyway so off we went.  It was the start of glasses so of course I will always wonder the connection.  In the end, I guess it doesn't matter.  What does matter is that she needs glasses for close up work like writing and reading so I have to get those new glasses!  Since the first pair came compliments of medicaid and we won't be eligible for another pair from them until next Sept. I have a bit of shopping around to do tomorrow, prescription in hand.

Next up, lab work tomorrow.  I was supposed to be headed for Dr. Miller's sibling study in April, but it is just not happening.  Probably more like July.  The last trip to our local endocrinologist  in February we decided to just wait 'til what we thought was the upcoming trip in April to do labs.  Since we are not going we need to check IGF-1.  We bumped gh twice since our last blood work.  I am really hoping we will be able to increase yet again as she is still under dosed if you go with weight based dosing.  I have seen such an improvement in tone and energy and body composition.  She has shoulders and tone!

I asked Dr. Miller if there was anything she wanted me to check while I was at it.  She wants a blood count.  I was a bit unsure what exactly she was looking for so I, of course, asked.  It is to check on her B-12 and iron supplementation and if there are signs of anemia.  Since we supplement quite heavily with B-12 my next question was what if she is still low?  Answer:  B-12 injections...  That would not thrill me, but it would not be the end of the world either.  Shots would be maybe every 2 weeks.  So why would a person still be anemic when taking loads of B-12 and iron?  In a word?  Absorption.  There seems to be an impaired absorption of a lot of nutrients in PWS and I would guess that B-12 might be at the top of that list.  I know many who see a big improvement from oral supplementation and there are others who see improvement only with injections.

Tuesday, March 19, 2013


Well, it has been an exciting week.  And I mean exciting in most every way possible. Last Wednesday Noah injured his shoulder in a soccer game.  At first we thought it was broken because, well just looking at it, it was obviously not right!  A break would have pretty much ended his senior High School soccer season...

Long story short he subluxed the sternoclavicular joint.  Okay, in English, the joint where the collar bone attaches to the sternum was apart.  It was far enough out that just looking at him you could see it pushing forward, but not so far that it couldn't go back in.  Thankfully it popped back in a couple of hours later.  In the end he will be out about 3 weeks.

Then on Friday we had a little college signing party for Noah at his school.  I don't really know how common it is, but his school really encourages the kids who sign with a university to take the time to invite their friends (out of class) to watch a sort of photo op and congratulate the player.  It was really nice for Noah.  I will post the newspaper article later this week.

All in all, he is doing well with the injury.  He spends most of his time trying to convince us that it is just fine (then asks where his pain pills are before bed)  

Sunday, March 10, 2013

Hunger Pit Falls

Ayden Jane does not currently deal with the PWS hunger that she will likely one day.  She is hungry at appropriate times and is generally always satisfied after whatever snack/meal we give her.  That is all great stuff and I am very thankful.

There is still something obviously off in in her hunger/satiety cues.  I notice it in a few ways.

1)  She will sometimes ask if she had lunch.  If I answer yes and remind her what she had then she will usually just make a comment of some sort and move on.  Occasionally she is hungry and will ask for something little or even a piece of gum.  At this point I can do this without concern.

2)  She will eat whatever portion we give her.  She is done eating more by the fact that her plate is empty than by the quantity that was on it.  It is sometimes surprising how small a portion I can give her and she is still satisfied.  Then some things she would eat endlessly if allowed.  (I think anyway, haven't really tested that theory)

3)  The other piece that is along this line is that she doesn't realize that she needs food until she is crashing.  When she was young it was a very typical looking hypoglycemia and she just couldn't go more than 2 or 2.5 hours between meals/snacks.  Now she can stretch the time and it is very dependent on the energy she expends.  If she is playing hard (and also distracted from food) she never says she is hungry.  Then you will start to see her laying down in the game or she will start to get whiny.  At this point she quickly becomes uncooperative and sloth like.  It always seems to hit so fast, but I am starting to realize that she just doesn't 'get' the hunger cue on time.  By the time she knows she is hungry she should have already eaten!

4)  There is such an importance on food in her mind.  Not really quantity or time, but she has a few favorites and if we don't have them accessible she stresses.  I guess it is like many of us adults and our coffee!

I am certainly not complaining!!!  It is just odd.  As a mom of a child with PWS, I am responsible for making sure they get enough of everything but not too much of anything.  I find myself pushing her to take a few bites one meal and other times reminding her we just ate so no, we are not going to have a snack of nuts...  I guess it all goes back to wanting to throw the responsibility out the window once in a while and stop thinking about the intake...

Thursday, March 7, 2013

Ready to Rock Kindergarten

Today was IEP day.  I do have an advantage over a lot of mom's of special needs kids.  I used to work on the other side of the table.  I have written IEP's and have taught special needs kids.  Even with all that there is a part of you that gets a bit nervous.  After all, we are talking about cold hard numbers of how my child compares with her peers.  We discussed several forms of evaluations.

I do have to add that I look at these things as sort of how Ayden Jane functions on a poor day.  I know that sounds strange, but the biggest challenge for Ayden Jane as it comes to answering questions, problem solving... basically using any of her cognitive skills is that when we do these tests I hand off Ayden Jane to someone she has never met and they proceed to a room she has never been in before ....  I think that is basically like testing me in a room where the music is loud, the walls have flashing signs on them, there is an unusual odor and the seating is totally unfamiliar feeling and I can't get comfortable.  Oh wait, I am supposed to do my best!  And quickly because no body has all day ya know....

First up is IQ.  Ayden Jane's verbal IQ is comfortably average.  Her performance IQ is in the low/below average range.  Looking at the subtests makes me wonder if she was concentrating at all on one of them...

Next was processing speed.  HA!!  No big surprise that it was low.  Even the psychologist commented though on how much quicker in general AJ was the second day when she had her come back to finish up.  Her processing speed at home is just fine, even quick.  Out and about in places she is used to it is still good.  In new places or when meeting lots of new people it just slows until she has time....

Academic Achievement/readiness showed her totally ready for Kindergarten.  She placed in the 63rd percentile.  I feel like with all the time we lost last winter just trying to get healthy that is an awesome score!  Things are firing fast and she is learning quickly lately so she will only go up between now and the fall.

Checklists for Social/Emotional functioning by her teachers and myself did not report any issues.  In fact, her teachers scored her better than I did!

We discussed speech therapy and decided to dismiss her.  Hard not to when there just aren't really goals left to work on.  We will definitely continue OT.  Basically, AJ is listed as receiving consultative special ed. services under the category Other Health Impaired (OHI) with OT as a related service.  Her special needs teacher will check in with her regular ed teacher and give strategies that can be used to help Ayden Jane in any areas she needs additional help.  She will not receive direct services.  She has made it through private preschool with no special needs services and is holding her own in the middle of the pack so I don't see the need to add things unless she truly needs them.  Ayden Jane rises to challenges and likes to do things independently.  As long as it does not stress her out, I say let her do it!

We discussed teacher and she will be placed with the teacher they feel will best fit her needs and she will have 2 kids she already knows in there with her.  One friend from down the street who is 'quirky' just like AJ and Lilah, our PT's daughter!  Yep, Jen will be in and out as she volunteers a bit and will be another set of experienced eyes on Ayden Jane.

Not sure I could have a better set up at this point.  Ready to Rock Kindergarten!

Wednesday, March 6, 2013


I need to review supplements that we are using with Ayden Jane.  First up, thanks to some rigorous discussion on a pws page on facebook is B-12.  The first go around with using B-12 to supplement in PWS came along when Ayden Jane was just turning 1.  I researched what I could and even checked with her pediatrician for safety.  Basically, he said he was positive it could not hurt her but he did not really see how it could help.  All I needed to know was that it was safe so I tried it.

The response was nothing short of amazing.  The big kids wanted to get her a t-shirt that said B-12 baby.  That story is somewhat written up in old posts.  What applies now is that B-12 helped her energy, nervous system, response time, volume (if loud is helpful), sleep....  It requires both forms of B-12, methylcobalamin and cyanocobalamin.  It also requires support from the other b vitamins, although not is mega doses.

Over the past 4 1/2 years of giving Ayden Jane B-12 we have raised the dose quite high and I often wonder if we will one day need to go to B-12 injections.  It would totally be worth it to have a shot once a month or every few weeks because with B-12 Ayden Jane functions as a typical 5 year old.  With out it .... not so much.  Sleep is disrupted, she is slow and tired and frustrated and cannot concentrate.

Iron is the flip side of this.  I had sort of inadvertently backed of from the iron while I was trying to get Ayden Jane's gut working well.  Since iron can definitely slow down the process I had backed off.  Some other conversation on the PWS pages reminded me I needed to push it a bit more.  It also has similar effects of the B-12 and I know that the 2 work closely together so that is not odd.

Yesterday was an example of the super powers that the B-12/iron combo have for Ayden Jane.  She slept wonderfully the night before.  She woke up going 1000 mph.  She skipped back and forth, from room to room.  Paced gleefully as she talked....  Even her teacher commented when I picked her up how funny and full of energy she was.  She played all afternoon at home and then Daddy took her to horses.  He had her for a total of 3 hours including horses, dog washing (at AJ's insistence) and a quick shopping trip.  All Gary had to say was that it was only 3 hours and he was exhausted.  Ayden Jane went wide open until we finally got her to be still and quiet in bed for a few min.  (around 8:30).

I love it!  We have her IEP tomorrow and I can honestly say that days like today make me confident that the IEP really is just to have support in place in case she needs it.  But she likely won't!! :)

Monday, March 4, 2013

IEP Time

Thursday this week I have an IEP meeting for Ayden Jane.  I will get the results of her re evaluation that was done recently along with the recommendations of the school.  Honestly, I don't need much in the way of services, I just want her to be able to continue seeing Mrs. S for OT.

Here is the crazy circle I am in though.  In order to get OT 'related services' you have to have an IEP.  Needing OT is not reason enough to get OT.  So, we have enjoyed OT for the past year because Ayden Jane qualified for an IEP due to speech and language delay.  She no longer has a speech or language delay...  Hmmmm.  She is not cognitively delayed...  She does not appear to have emotional issues and even if she did, they are not disrupting her learning....

I am hoping that we will qualify as Other Health Impaired (OHI) but I am not really sure how that works.  Obviously she can be on a 504 to cover any food or anxiety related issues, but she would not get the OT she truly needs.  Guess a little reading on the state standards of OHI is in order :)

Sunday, March 3, 2013

Great Weekend

Kayla came home from Clemson for a visit and brought two friends with her.  In Ayden Jane's mind they all came here with the sole purpose of hanging out with her!  The way they spoiled her would make every one think that were true.  Ayden Jane enjoyed being the total center of attention for the better part of two days.  Here is a picture of one of their adventures!

When the girls had to go Noah softened the blow by taking AJ swimming.  We topped off the day with a movie and early bed.  Can it get any better?