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!

Intermission

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!

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.

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!

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.

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.

Allergy/Immunology

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.