Friday, May 31, 2013

Shriners Visit

As a quick review of Ayden Jane's 'history' of scoliosis, she was x-rayed at 3 and there was no sign of scoliosis.  When she turned 4 we did not even check her because she was still so active and there was nothing that looked fishy.  Jen, our PT, is great with checking these things and our doctors did visual checks.  Then the 'winter of infection' came.  While she was sick that winter her tone turned to mush.  By the end of last summer we saw some outward signs that things were not quite right and we knew it was time to get a look at things anyway.  No only were some things not quite right with her back, but she was internally rotating her right hip and her knee.  So, her second x-rays for scoliosis were done and revealed a 23 degree curve.  Yikes!

We talked to some folks and decided to hit the experts at Shriner's in Philadelphia.  We were told via phone that 25 degrees was the point were intervention was required.  Now, this could be bracing or casting, but we were not excited about the thought of either.  We made the appointment, got Ayden Jane back into some new orthotics and went to work via PT.  Jen even had us use a knee brace to keep the leg in a better position and help AJ remember to sit correctly.  November we had her third set of x-rays and her curve was reduced to 12-13 degrees.  This cleared us from having to worry about casting ect. but Dr. VanBosse wanted us to be checked again in 6 months.  He was comfortable with transferring us to Shriner's Greenville.

Yesterday we made the trip and had Ayden Jane's 4th set of scoliosis x-rays.  I was really hoping for a further reduction in the curve, but am relieved that her curve is at least stable.  It was still 12 - 13 degrees.  Even better is that he feels that with her age it will likely remain stable and we only need to check in with him once a year.  (unless we see anything of concern)

We were hoping to see some friends while we were there and head to the zoo together, but alas little E started up with  a fever.  We still hit the zoo and the great little playground right next to it.  Ayden Jane loved the baby giraffe and was amazed at the size of the elephants.  I was amazed that after sleeping in a hotel, hours at the hospital, nearly 3 hours of walking at the zoo and playground time Ayden Jane did not even sleep on the way home!!!  She talked for 4 1/2 consecutive hours.  Gary and I took turns pretending to listen and there were times where she was just narrating the puzzle she was doing or 'reading' a book.  Sometimes I really wished this kid watched movies!

Shriner's itself was great and made me think of Kayla a little bit.  She is looking to go to Medical School and thus needs to do an internship.  What a great place that would be.  She attends Clemson which is nearby so she just might check it out.  Who knows...

Monday, May 27, 2013


So, May is/has been Prader Willi Syndrome awareness month.  I am not entirely sure what to do with that.  I am not shy when talking to folks about what Ayden Jane does deal with.  People who know us learn pretty quickly that diet is a big issue.  That Ayden Jane has several therapies and that doctors, blood work and x-rays are just a part of life.  That her immune system is not quite right and she has a hard time recovering from illness (likely not a PWS thing, just a thing complicated by PWS).

However, many of the things that are down right frightening about PWS are very much kept at bay with Ayden Jane.  Most people would just not be able to connect those things and Ayden Jane.  She just seems even more comfortable and a part of her peer group as she gets older. I was just chatting with a friend tonight about how AJ is just so totally comfortable socially now.  She is confident in who she is and can play by herself and yet she fits right in with other kids and plays very well with them. I guess a part of me doesn't want to scare people off??  Or make them look differently at AJ?  Things like, yep, we treat food carefully, but we have no need to lock or panic.  I frequently leave brownies or cookies out on a plate in the kitchen and I don't even think about it.  Ayden Jane does not deal with the behavior problems I hear about.  She is stubborn and challenging at times, but more in a 5 year old way than a PWS way. 

We do not deal with daytime sleepiness, cognitive delays, autism, sleep disturbances, eloping (anymore), social skill deficits, speech issues, academic deficits, skin picking, ...

I have immense respect for my friends who deal with these things and more daily.  The one thing I do understand from experience is that PWS is relentless just as any chronic medical issue.  As a parent you do not get to forget about the syndrome for even one second.  Ayden Jane has and will have PWS every minute of every day for her entire life.  Sometimes we are tired.  Tired of therapy, tired of keeping a watchful eye, tired of researching and reading every scrap of information that might help, tired of advocating with doctors for what we know we need, tired of worrying, tired of fighting insurance companies, tired....

For those who have an interest in understanding what PWS does 'offer' many people with the syndrome please check out Ali's blog: Diving into the Waves as she has done an amazing job this month of May of highlighting one bonus feature a day of what life is like for most with PWS.


Sunday, May 26, 2013

She's Back!

It has been just over 4 weeks since the strep throat hit Ayden Jane and she has finally recovered.  It was almost like switch flipped and she returned to all of her stamina and energy.  We are now back to walking the dogs daily.  Ayden Jane is in charge of how far... and she prefers the 2 mile route in our neighborhood.  Yep, she is playing all day and still walks 2 miles most nights.  I'd say that's pretty good for any 5 year old!

Here is one day in the life of Ayden Jane.  Thursday AJ stayed for lunch buddies at school.  So, she had school from 9-12 and then stayed to have lunch and hit the playground for another hour or so.  Home at 2 and played outside with the dogs.  Then Noah had a couple of friends over and Ayden Jane basically cracked them up and got them to dress babies....  They all left and Ayden Jane moved on to Mckenna and her friend that was over.  They all went outside to the trampoline and played hide and seek.  I took the friend home at 6:30 and as we headed back down our road, Ayden Jane's friends from down the street were looking for her.  She hopped out of the car (it was about 7) and headed off to walk down to the field with them.  Finally, she came home about 8:30 and hit the showers!!!  So glad summer is back!

Now if I can just figure out how to get better liquids in Ayden Jane!  She just refuses to drink water and with all the playing outside and the weather heating up....  Wish me luck!

Heading to Shriners in Greenville on Wed.  Sure hope all this exercise has helped with her scoliosis.  More on that tomorrow.


Monday, May 20, 2013

Recovery and Maturity

Ayden Jane continues to slooooowwwly recover.  It is really unbelievable how long it takes for her body to recover from illness.  It has been nearly 4 weeks since she first got sick and we are not there yet.  I'd say her hunger (or lack of for a kid with PWS) has diminished nicely.  She has even passed up a meal here and there.  Her tone is improving and getting close to where it was prior to getting ill.  Her energy is returning but her stamina still lags.  She will start into playing or walking... and then need to stop and rest.  The necessary rests are getting shorter and less frequent.  She has nearly given her nap up again.  Her behavior has been good and she is craving lots of rough play.  Her belly is showing some distention as 20 days on antibiotics have taken their toll.  Slowly though, she is returning to the seemingly non PWS kiddo with PWS that she typically is.

We went to a birthday party this past weekend.  It was a small party and Ayden Jane only knew the birthday girl!  It was so fun to watch, though, as AJ just hung with the girls.  She joined in the games and figured them out as quickly as any girl there.  She was very tired after even though she did not really play too hard as the party was indoors.  I think the social thing can be very tiring, but I can honestly say that Ayden Jane is really getting the hang of it!!

It all just makes me know she is ready for Kindergarten.  It will be overwhelming, but at the rate she is going she will adjust way quicker than I could imagine.  Next I need to find some VBS's this summer or camps that she can join in to keep up the great social skills.

Sunday, May 12, 2013

Is it Funny?

Ayden Jane is on track in every area but one.  Her handwriting.  Not even sure you can call it handwriting.  The good news is that she can now hold the pencil well and trace well.  Basically, her hands are getting stronger and more coordinated.  The hard part of this whole leap, though is to be able to take what is in her head and make it come out her hands.

I imagine it is a bit like when I try to draw something even remotely complicated (I an no artist).  I can see what I want to draw in my head, but no matter how hard I try it just does not come out of my hands, so to speak.  Ayden Jane knows the letters.  She knows what they look like and can trace them, but to make them just appear on paper from what is in her head?  Well, it is hard.  Eventually I know that she will learn to make each one but I believe it may be a fairly painstaking process.

Tonight when Mckenna was drawing with Ayden Jane and making Mother's Day Cards for grandma, AJ asked Mckenna if it was funny when she scribbles.  It took a minute for Mckenna to catch on to what she was talking about until Ayden Jane said, "my friends at school think it is funny when I scribble."  The tone was definitely in the category where she is uncomfortable with it.  Not entirely feeling picked on, but a bit sad. 

I basically used it as ammo to get her to try to draw real things.  She just rushes so much, I guess because it is such an effort it is hard to go slow?  The few times we have gotten her to slow down and try to really draw something I have been surprised.  I think this summer's work is cut out for us.  Continue to improve reading skills, learn to make letters, get lots of exercise and lean out AJ.

Saturday, May 11, 2013


Special needs kiddos, or kids that just develop a little slower or differently sometimes have trouble making friends.  Or, at the very least, are a bit socially awkward and have a bit of trouble keeping up.  A parent wants most for their children to be happy and healthy and be the best kiddo they can be whether they are special needs or not.  Lets face it, the ability to be a good friend and make/have friends is a pretty big key to happiness.  I have to say that at this time Ayden Jane is in good shape in this department.  Definitely this is facilitated by the awesome parents at her preschool, but it is so great to watch.

First up there is A, her very best friend.  They have so much fun together and it melts my heart.  A is moving far away in the month of June, and Ayden Jane is sad but dealing with it pretty well.  She says that she and A have made a plan to draw pictures and write notes about what they are doing and send them to each other.  So cute.  Now that AJ is finally feeling better we hope to get the girls together a few more times before they head out in June:(

Then the other night at the soccer game Ayden Jane made a friend and scootered for most of the game with her.  It was almost comical that there was another little girl there (a friends daughter) that was sad because Ayden Jane was not playing with her...  Secretly it warms my heart that AJ is so wanted as a friend that there wasn't enough of her to go around. HA!

Lastly, (this week) we went out to the beach for our first spring time boogie board and a boy from school was there with his family.  In the past, she was not quite able to connect with him at the beach and preferred to do her own thing.  This time, however, she jumped right in with him and hit the waves.  I am sure is was just a small event for him, but it was so cool for me to see AJ just so naturally and easily navigate several peer situations.

Now praying she can use these skills to make new, good friends in Kindergarten next fall!

Friday, May 10, 2013


You may or may not have heard of oxytocin.  During the research conference in Canada last month there was some discussion of a study in France where oxytocin was given to newborns and it has promise to resolve some of the most challenging features of PWS.  I guess a part of me would be sad that Ayden Jane would have missed that window, but I can honestly say that I would be so excited for the next generation of PWS kiddos/families that I am way more happy about the thought. 

So, how does it work?  I have no clue in scientific terms but from what I understand, the low to no oxytocin levels are the start of a chain of events.  Or maybe I should say they don't start a chain of events?  Anyway, in a typical kid the oxy is there and the kids feed and their metabolism and intake balance works.  In PWS kiddo's they have no hunger at first and they do not develop a typical metabolism...  PWS infants given oxytocin suddenly begin to feel hungry and know when they need food.  The study is ongoing but the 2 or infants given the dose look promising. The idea is that if we can jump in that early on and correct this level that a much longer term development chain will be positively impacted

So that's all well and good but what does it have to do with AJ?  First of all, Dr. Miller reports that PWS babies who were induced have better eye contact ect. as infants than others.  The drug given to induce is Pitocin which is a sort of oxytocin.  My discussion with her was that Ayden Jane was induced and it took a long time.  This means that she was exposed to a fair amount of oxytocin for a good long while.  It is possible that this will somehow translate into positive things down the road.  I know it's a stretch, but it's nice to know that such a long day back in Oct. of 07 brought some hidden benefits.  We really did come close to c section a few times, but I hung in there.

Yippee!!!!! And Some Added Musing

So, Ayden Jane has been on Augmentin now for 24 hours and 3 doses.  The change is awesome.  Not to say that she is totally back to herself, but I can already see that she is going in the right direction.  First up, the amount of liquid that has come pouring out of this child is unreal.  Last night she managed to soak through 2 pull ups and soak 2 beds.   I know, more than you need to know, but when the infection really starts to kick in AJ swells.  Basically, she just retains so much water all over she looks puffy.  I took a picture last night of her in her pull up so I can compare in a week or two.  (I won't post them 'cause, well, she might hate me later)  Her cough is less thick and her energy is better.  Her eyes are even beginning to look clear! 

I figured the next best way to aid the clearing of congestion is with saline.  I went for the all natural saline called ocean water.  It was great.  We had not been out to the beach this spring because I knew the water was still a tad too cold and it would be a battle to keep AJ from turning blue as she swore the water was not too cold.  Noah had been surfing and said the water was warm enough and finally we had a beautiful day so off we went.  There were some friends there with their 3 boys, one of which is in AJ's class at school.  She and C went boogie boarding and had a great time together.  So fun to see AJ interact so well with other kids and enjoy the ocean!  She got her saline wash when she took a couple of good tumbles, ha!

So from this time around of illness I feel I can say for sure that Ayden Jane requires something stronger than the first round antibiotic and Augmentin seems to be the ticket for now.  Also, I believe that PharmaNac has strengthened her immune system so that she does not crash as hard from illness.  The severity of symptoms this time around was much less and the time it is taking to rebound seems to be much less as well.  It's not perfect but I'll take it!  During the illness we more than doubled her PharmaNac dose.  She just seems to burn through it when sick.  No digestive track issues with the increased dose while sick.  I will now back off to her regular amount of 1/2 tab. 

I am posting for the first time on my new computer!  Woo Hoo!  The posts should be coming much more regularly now.  I have a few unrelated things to touch on.

Thursday, May 9, 2013

Antibiotics and Ayden Jane

So, Ayden Jane was on Penicillin for 10 days.  During that time I have no doubt that it took care of the srrep throat, which it was prescribed to do.  The viral infection that seems to have tagged on to the end of strep seems to have created its own bacterial infection. 

The pediatrician (not my regular one) saw her Tuesday and basically said that she has been on antibiotics throughout so more antibiotics aren't going to help.  In essence, he did not believe, or even consider it a possibility that AJ could have developed a secondary infection that penicillin would not take care of while she was on antibiotics.  His advice?  Wait 10 to 14 days and then come back if she was not clearing up.

Well, almost 24 hours off of the Penicillin and Ayden Jane woke up this morning by choking on / coughing up thick green, black crud.  She was way more congested, not improving.  I had emailed Dr. Miller in the intermediary and she felt that AJ needed Augmentin.  That it was entirely possible that the penicillin was not strong enough, especially when AJ was already fighting something else, to rid her of the secondary infection.  Sooooo, I called Dr. Clark.  She is awesome.  We talked through what I needed and by the afternoon AJ was on Augmentin.  Hopefully in the next couple of days I will finally see vast improvement!

On the Pediatrician note, it was not our regular pediatrician and since this has happened a couple of times where we could not see our regular one and the others were, well, useless would be the nicest way I would put it, we are looking into changing pediatricians.  Jen has given me a recommendation so I will be working on that next week.  Well maybe the week after since I work so much next week.

I just want to see Ayden Jane healthy again!!  I want to bring down those IGF 1 levels so we can bump up her gh.  I want to get her active and leaned out over the summer.  We are in much better shape this spring than last, so I will just be thankful for that and move on.

Sunday, May 5, 2013

Sick and Tired of Sick and Tired

Ayden Jane had her first signs of illness 2 weeks ago Wednesday.  The high fevers from strep throat were taken care of by penicillin but then this yucky congestion set in.  Arrrrg.  So, we are now on 6 days of congestion which is settling into her chest.  She is already on an antibiotic but she is just not clearing it.  It is bizarre how much she can let sit in her throat without coughing.

I called last week to ask her pediatrician about it all, but he was out of town so I chatted with Dr. Miller and she said that it is not uncommon to pick up a viral infection with strep....  But to take her in early this week if she is not 100% clear.  Also I bumped up her Pharma Nac to try to keep the goo thinned out.

I will be taking her Monday or Tuesday because we are no where near 100%. I think what bothers me the most is that when she tries to exercise she just gets so tired she has to stop and rest.  Just before all this, we were walking 2+ miles at a good clip with the dog. Yesterday she was all excited and not even a half mile in asked if she could sit down and rest.  Poor kid.

In other news, Kayla is home!!!  Ayden Jane stuck to her like glue the first couple days.  If Kayla managed to leave the room undetected it was a matter of seconds before Ayden Jane was asking/looking for her.  Maybe tomorrow Kayla can unpack without 'help'.

Ayden Jane quotes:  Mom, if we can see God in Heaven how come we can't see Him here?  Does that make any sense to you?

Then last night about 2am when AJ woke me up:  Mom, can you pray for me to only have good dreams.  You can whisper because God is everywhere so He can hear you even if you talk quiet.

Talking to Kayla this morning: I'm right cause I thought it in my brain and everything in my brain is right .