Saturday, June 30, 2012

Sleep Study

Last night Gary took Ayden Jane to her first sleep study.  I have heard lots of people talk about how miserable these things are...  I personally cannot imagine sleeping with all those wires attached and knowing someone is watching everything!

So how did Ayden Jane do?  She had the time of her life!

To AJ it was one big adventure with Daddy.  She was so excited to spend the night at the hospital just like Daddy does when he works.  She went to MUSC which is about an hour and a half or so from us.  They left with time to stop at Chik-fil-a, a big treat.  Gary said she never stopped talking...

Once they got there, Ayden Jane quickly realized she was the total center of attention and she loved it!  She asked about the camera in the corner and got a tour of the 'control room'.  She was so excited that the straps and things were purple.  She really liked 'Cambela' (aka Pamela).  The only trouble they had with her was getting her to stop talking and go to sleep!

Here are a couple of pictures!

Sunday, June 24, 2012


While things are stalled out with Ayden Jane we will use this intermission to give an update on the big kids.  I will do my best to make a mission of getting some pictures taken in the interlude as well.

Kayla is back at work at TMP.  She lifeguards and teaches swimming at a summer program for kids that need a great place to be in the summer.  She loves those kids and it is fun to hear her talk about them.  She is working as much as she can as the poor college student and hanging out with her friends.  Ayden Jane is loving having her home.  The other day AJ asked if Kayla will come home to live after she is all done with college.  I told her that Kayla would probably get a job and have her own house.  Now, Ayden Jane regularly informs Kayla that when she gets her new house, AJ will be coming to live with her... Good luck with that Kayla!

Noah is working coaching soccer camp.  He is great with the kids and has a good time with it.  He is also still playing, working out and looking forward to playing soccer in college.  Where?  Who knows...  He is planning to major in exercise physiology and wants to head toward a masters as a Physician Assistant, Exercise Phys., Orthotics and Prosthetics... or something he has not thought of yet.  Noah and Ayden Jane have an awesome relationship.  Ayden Jane may not expect to live with Noah, but she knows that anything she does with Noah will be fun!

Mckenna is coming into her own little being.  She is having a great summer.  She just finished up a 3 week drama summer program and is now off at church camp.  Still yet to come is another week of camp in July at at Clemson extension camp with lots of messy outside learning and fun in the salt marsh.  We plan to do lots of surfing and swimming and hanging out too.  She hasn't really had time for that sort of fun yet.  Mckenna is a different kid with lots of interests.  On any given day she might spend the day with her nose in a book, surfing in ocean, playing soccer, locked in her room working on some sort of performing art, drawing and discussing color and shading... She is growing up fast!

Saturday, June 23, 2012

Getting Impatient

Immunologist comes back from vacation next week.  Sleep study next week.  Getting impatient, not a panicky sort of impatient, just ready to get this over with sort of impatient.

I guess it just stems from the feeling like there is nothing I can do to fix this stuff at this point.  Not that things get magically fixed, but I do operate well with a mission and plan.  At this point, it feels like I have neither.  Well, I do have a plan.  Sleep study then surgery, but there is nothing for me to do day to day.

Tuesday, June 19, 2012

A Little Wacky Inside and Out

I stopped by the hospital and got the labs from our recent testing.  Basically, they show that Ayden Jane is wacky inside and out.  The eosinophils that have been so high for months on end?  Totally normal.  White blood cell count? still in range, but oh so barely.... curious.  Immunology results?  One of the values is about double out of range high (IgA) which can mean everything from something big to nothing at all.  Two others (IgM and IgG) were very much on the low end of normal which could indicate that she has a harder time getting over some illness.  (obviously that's not a news flash! LOL)

At any rate, AJ's immunologist is on vacation until next week so we won't be putting much more thought into the blood work until then.  Sleep study is next week with results a couple weeks after.

In the meantime, we will pass the time staying active, having fun and going to vacation bible school next week at her beloved preschool!

Saturday, June 16, 2012

IGF - 1 Still High

We got the results of Ayden Jane's IGF-1 testing.  Basically, that is the measure to see how to dose growth hormone (gh).  Right now in Ayden Jane it is sort of also a measure of how she is using gh.  Her levels are still way too high even after we have dropped her dose and she has had time to recover from this winter's illness.  Not good.

We are still seeing some effects in her that show that she is not using the gh well.  For example, she has added weight (the enemy of PWS) and her tone is not as good.  Also she is back to napping longer and every day.  She also goes to bed earlier.

So, we are on a hunt to figure it all out.  Sleep apnea could play a part in inefficient use of gh so with any luck the tonsils and adenoids coming out could help a lot!  I sure hope it is that easy...

Still will need to track down the poor response to fighting bacterial infection though. 

Again, remind self constantly:  AJ is smart, funny, active, coordinated, verbal.... we just need to figure out what to tweak and all is well.

Friday, June 15, 2012

ENT Visit

I am trying to catch up with all the things that have been happening with Ayden Jane.  Sorry for those who read for entertainment, as this stuff is not so entertaining.  I just trust that it is informative one day for somebody who is dealing with something similar with their little one.

Anyway, we made the trip to ENT yesterday.  I have gotten pretty good at meeting new doctors and am pretty confident now in forming opinions of them and whether or not I need to move on...  I really like this guy.  He chatted with us and even joked that when he first walked in he wasn't sure he was in the right room because he was looking for a child with PWS.

In the end, her is the plan.  Definitely need to take out the adenoids.  Now in kids with PWS this is often a partial adenoid removal so as to preserve the quality in the sound of their speech.  Sometimes if the entire adenoids are removed it causes a nasal sounding speech.  (Not sure why)  Anyway, we are definitely going after those adenoids.

Part B of the plan involves a sleep study.  Basically, he said the tonsils are only mildly enlarged and he would not typically take them, however with her history, PWS and being on gh he wanted to check for obstructive sleep apnea.  If there are any signs of that then out with the tonsils as well.  I am totally on board with this plan and was happy that he was pretty well up on the whole PWS thing.  When I mentioned other doctors, a rough winter... he was happy to receive input from whomever.  Now that is the kind of Dr. I like!

So, sleep study is scheduled for June 29.  Should be interesting as AJ has never had a sleep study before.  (I know, gasp! A child with PWS, on gh and has never had a sleep study!!)  Gary will go with her for the night as he is totally comfortable in a hospital setting and AJ loves to anything with Daddy.  Glad the appointment is not too far away.  I would love to have this all taken care of before the end of July.

Thursday, June 14, 2012


We decided it was time to tackle all things related to the horrible winter illness wise.  First up, chronic congestion.  We still use claritin and it helps a little.  Not enough.  With the time spent in the ocean with it's natural saline washes... and the fact that in every other way she has been healthy for a while AJ just shouldn't be congested.  It is not on and off, it is daily.  Answer?  A trip to ENT (info coming soon).

Second challenge is to figure out why Ayden Jane seems to have so much trouble fighting bacterial infection. Also tied into immunology, why are her eosinophils and IgE so high when there is not a significant allergy to be found.  The tests we ran (waiting for results) include things to see if her immunizations actually caused an appropriate response.  That is one way to see if the immune system is working properly.  Also, specifically Dr. Streck wants to see if she has any signs that the pneumoccocal something vaccine has been responded to properly because that is the most common culprit in sinus infections.  The other one Gary says is a marker of inflammation.... this is so new to me.

We drew the labs a few days ago along with another IGF-1 level for Dr. Clark to see if  Ayden Jane is starting to use that gh better.  Definitely nervous about these labs as they could be great news, no news or rotten news.  Of course, having to wait on the beach today was not so horrible.

Wednesday, June 13, 2012

Swim Team!

Well, Ayden Jane is still over the moon about swim team.  It is adorable!  She has had 3 'practices' so far.  The first practice ended up being almost one on one.  Her coach most of the time has been coach Nikki and she has the patience of a saint!  Also, she just approaches the whole thing with all the expectation that Ayden Jane is capable of doing .... well everything.

We were concerned about night 1.  Ayden Jane was swimming in a pool she had never been to before and her friends that she was going to swim with were not able to make the pool switch.  Then when we got there we found out that, at this pool, parents are not allowed to sit on the pool deck.  They can watch from windows at one end.  Hmmmmm.

Ayden Jane was AMAZING!

She walked over to the group with confidence and never looked back.  She focused in a loud, echoey pool. She followed directions and communicated beautifully.  She fearlessly tried everything that was asked of her and she worked soooo hard for the entire hour.

Ayden Jane was the tiniest kid in there.  She had just one other boy who looked to be about 6 or 7 in the lane with her.  It was such a proud momma moment as she did so well.  When she was done I slipped in to pick her up and she came over smiling, "I did my very best Mom!"  Her coach was so complimentary...

I knew the following week we had lab work coming and an ENT appointment ..., but in that moment PWS was invisible.

Now I will need to work on some pictures of my adorable little swimmer in her cap and goggles!

Saturday, June 9, 2012

Independent, Determined, Fearless

One thing Ayden Jane does not lack is confidence.  She has no idea that there are perceived limitations about her because of a bit of missing genetic material.  She attacks life with an expectation of success.  She may actually think she is a super hero.  You know, indestructible and capable of anything.

Sometimes being so independent, determined and fearless spells Trouble (yes, with a capital T).  Here are a couple of stories for your entertainment (since she is safely sitting next to me playing) and for me to share with Ayden Jane one day!

First the ocean.  Ayden Jane loves the ocean and she is quite sure that since she can swim, she can go anywhere in the ocean all by herself!  The other day she was on her boogie board "surfin'" as she calls it.  The water was totally calm.  I mean so calm young kids were kayaking in the ocean.  Seriously, it was more like a lake or pond it was so still.  Ayden Jane hopped on her board and headed out beyond the tiny breakers and started kicking her way to Europe!  I called her in and she wasn't making any headway so out I went.  She was out over MY head at this point and I had to tow her back in.  She was great for a bit and the out she went again.  The second time when I started to come for her she jumped off her board and dove under the water to 'hide' from Mommy.  She thought it was great fun until Momma made it eminently clear that there would be no more boogie board if we EVER had that problem again.  Problem solved, except for the part where she is still convinced that she would have been fine.

The second time (this week!) she exerted her independence....  Was Thursday morning.  During the night there had been a bit of musical beds.  Ayden Jane ended up in bed with Gary and I was in AJ's bed.  Ayden Jane always wakes up between 6 and 6:30.  I woke up but had no clock it 'felt' like time for AJ to be up, but the house was quiet so I rolled over and enjoyed a few quite moments of pseudo sleep.  As it was nearing 7 I started to wonder, then heard the dogs barking.  I got up and as I was heading out of AJ's room the doorbell rang.

My first thought was that Ayden Jane and Gary were outside feeding the dogs and Ayden Jane was being funny.  I went to the door and sure enough Ayden Jane was there... in her pull up... with 2 people I don't know.

I had forgotten that Gary was leaving really early for a class.  (he works nights so is never leaving in the AM)  The nice couple had found Ayden Jane out and about on her bike and returned her home.  AJ was about .75 miles from home getting ready to cross a busy street to go play on the elementary school playground!  Ummm, excuse me Ayden Jane but you HAVE to have somebody with you!!

She understands that she cannot do that again or she will no longer have a bike.  I don't think she really gets the danger involved in what she did.  I tell her that she could have gotten hurt, hit by a car...  She says, but Mom I know how to ride on the side of the road.  I told her a mean guy could have taken her.  She answered that she knows better than to go with a mean guy.  I told her somebody could have taken her whether she wanted them to or not and she informs me that she if fast enough and strong enough to get away from anything.  UGGGGG.

Since she was tiny we have noticed that 'protective reflexes' and other understanding of danger just did not exist.  She can learn them, but she has to experience to learn.  Don't touch the stove it might be hot - burnt hand.  Hold on tight when you swing so you don't fall - banged head.  Ask for help to get down if it's too high - jump off the top bunk and discover the meaning of too high.

She amazes me daily with things she learns to do purely by shear determination and perseverance.  She gets it in her mind and she does not quit!  Sometimes I think if I used every ounce of my ability like she does, what could I accomplish?  It is an incredible way to live.  No "I can'ts" no fear of failure,  no limitations in her own mind, positive that everything will work out.

I pray we can keep her amazing spirit intact and teach her only enough reality to keep her safe. Along the way I also pray the rest of us will live more like AJ.

Wednesday, June 6, 2012

Will I ....

Ayden Jane asks questions and makes statements starting with "will I" and "when I am" a lot.  Here are some examples:  will I still see Mrs. Stella (OT) again when I am in the froggy fours?  Will I learn to swim all the strokes?  When I am big, I will ride my bike without training wheels...  Most of them are pretty typical for a 4 year but the other night as we were headed home from horses, Ayden Jane asked, when I go to college will I take my shot, shot with me?

Wow.  There is a lot in that question.  When I go to college...  It used to be sort of hard on me when she mentioned thing like going to college or getting a job... because I would wonder IF these things would happen. I think now I am to the point where I don't wonder IF they will happen I more wonder what they will look like. Ayden Jane has proven to us that she may not do things the same way or as easily as other people, but she will find a way to make them happen.  I sometimes wish I had her confidence and persistence.  It is so cool to see someone use every bit of their ability and even beyond sometimes.

Saturday, June 2, 2012

Shot, Shot and Swimming

Ayden Jane still calls her nightly gh shot her 'shot, shot'.  For most of the past 3 years of gh, I have given her shot after she has gone to bed.  She slept through it so it just seemed easiest.  For some reason, lately, she has decided that we should do shot, shot before bed.  I am good with that as I honestly often forget to give her the shot until closer to midnight and I am sure that is less than optimal.

Last night as we were heading down the hall, Ayden Jane asked me if Mckenna does her shot, shot herself.  It never occurred to her that maybe other people don't have a shot.  Hmmmm.  I answered her like I answer all of her questions, just matter of factly.  I said that she is the only one that has a shot, shot.  Her next question was, will I have a shot, shot when I am big?  I said, yes, but you will be able to do it yourself.  She spent some time trying to decide how old she should be before she does it herself.... ahhh the love of numbers.

The swim coach called tonight.  At first I was worried that they decided not to take our little 4 yr old, but the trouble was that the pool they were to train in is ... well, as of today in foreclosure.  (the just rented time in this pool)  That means he wants Ayden Jane to train at a different facility.  Of course, it is quite a bit further and the times are different.  grrrrr.  How could I possibly not do swim team after Ayden Jane has gotten so excited!  We will not get a chance to get started until Wed. morning, but I sure hope Ayden Jane is good with this new pool.  Also, her little friends will not be able to participate as the times and location will not work for them.

I guess we shall see!

Friday, June 1, 2012

The Deep, Deep

Here's a big surprise, we went to the beach today!  Okay, so it is not really news anymore for us to go to the beach but what made is extra special was that Gary came.  He had the night off (or so we thought) and headed out to the beach with us.  We went out around noon and figured that AJ would last and hour or so...

First she went about her usual business of 'surfing' in the breakers.  She is so independent that she just takes her board and heads out (goes waist or so deep) and the kicks hard to catch the waves.  All the while she has to stand in the current, withstand the pummeling of the waves and hold onto the board in the wind.  It is hard work!  It is hilarious because she never stops talking or singing the whole time.

After a bit she decided to go out into the deep, deep with Gary and Mckenna.  Then the real fun began!  The water was rough today and the current strong.  The waves were coming fast, one after another.  Ayden Jane was thrown off her board a few times and was crashed hard.  She thought it was all the greatest thing ever!  She had so much fun she cannot wait for the next time Gary can head out with us.

After a good 2 hours of the beach we headed for the pool.  I totally expected the crash at any moment but she kept on going.  We made it home between 3:30 and 4.  So, 3 or 4 hours of swimming!  Oh how I love summer!