Tuesday, December 30, 2014

Happy New Year

The change of year is always an interesting time. It is a time I enjoy the fact that I have written throughout the year because it is so fun to look back to what was going on last year, the year before... Amazing how time goes on!

I see 2014 ending more than just 2014. It is ending with such hope. I will try to explain what I am seeing/feeling when I look at Ayden Jane these days.

In the fall three years ago Ayden Jane's journey took a sharp turn. Up until that point Ayden Jane was so lean and active and healthy... Then the very first illness that either uncovered or started her immune issues set in. So began the "winter of infection". It took about four months for us to finally clear her body of infection. Left in it's wake were high IGF-1 levels, crazy blood sugar drops, labs all out of whack and added weight we did not need. Recovery took removal of adenoids, discoveries that vaccinations did not even take and crazy results from some labs.

Just as all began to even out, fall came and colds sent us back down a similar path. Experience helped us fight illness more effectively but it still took so much out of Ayden Jane and she tirelessly worked each time to get back to health. With each round we added a bit more weight than we could get off but onward we charged.

This was our pattern for three years! It broke my heart every time!

So why do I have hope that this will change? We have found something that has filled the gap in Ayden Jane's immune system.  It is the last day of December and Ayden Jane has stayed entirely antibiotic free! She has had a couple of colds that ran a totally normal course and then she recovered just like any other healthy kiddo. I cannot find words to describe what a miracle this is to me. I lived in fear of the next cold that would turn to a sinus infection or pneumonia and lead Ayden Jane back down that path.

As part of that and definitely magnified by the use of artificial sweeteners there was a constant challenge of balancing blood sugars. Ayden Jane required something to eat about every 2 1/2 hours and I was constantly watching to see that she did not crash.

Now, no artificial sweeteners and a working immune system and the addition of some MCT oil (which she previously could not handle) and I don't know how to express what I see. That spunky little kiddo that I had up until 3 years ago has returned. That may sound odd as Ayden Jane has accomplished so much in those 3 years but there is just a difference. She is a determined soul by nature but there is such a spark and an ease about her now. So much less stress surrounding our days and nothing but possibilities on the horizon. I see 2015 as the year Ayden Jane takes off. No more set backs. Just soaring to new heights.

Sunday, December 28, 2014

Christmas Fun!!

Gary and the kids were messing around out back at Grandma's house. These are just a couple but I love how much fun they can have together.

Ayden Jane must have had quite the interesting story to tell.

And this is just our smiling Ayden Jane. She has taken to the crazy fake grin whenever there is a camera around but it's pretty cute anyway :)

Sunday, December 21, 2014

Good Stuff

Ayden Jane has done an amazing job of not even trying to back out of her deal with the 'fake sugars' and water drinking.  As a result her hunger, which was not really a problem before, has subsided even more. Here are 3 examples:

Thursday: AJ had a full day of school with Christmas party, games, extra recess... and then she stayed after school for a club she goes to for another hour.  When she got home she asked for a cheese stick and some cherries.  Fair enough. After her snack we went for a 'run'. She was tired and we walked more than we ran, but we still covered 2 miles. A little while after we got back Ayden Jane asked about supper. It was definitely past when she is used to supper. I told her Gary would be home in a few minutes so she could choose to wait for him and we would all eat together or I could go ahead and fix her something. She chose to wait. A few minutes turned into an hour. Ayden Jane got tired of waiting and decided to just go to bed (about 7) with no supper.

Friday: AJ had another full crazy day at school with a pajama party for the Polar Express, games, extra recess, art... all fun stuff. She came home and skipped after school snack and waited for supper until 6.

Saturday: Ayden Jane woke up about 6:30 as usual. The odd thing was she was in no hurry at all for breakfast. Now that's a miracle!!

Add to that a bit of an increase in energy (possibly boosted by the start of MCT oil today, but it was just a 1/4 tsp):

Sunday: She played 'cross country' with her babies before church.  Yep, she was running laps around the house (outside) taking turns with each baby. I have no idea how many laps she ran, but I know it was a lot. After church she swam for a couple hours and I thought she would be toast.  Nope, she came and asked me if we could take the dogs on a long dog walk.  Ummm no, but we can do a short one (as in a mile and a half) before candle light service. Off we dashed back out the door. Came home and from dog walking, and off to candle light service at church. You would think she would sit calmly with all that activity. NOPE. She wiggled and fidgeted her way through. We just got home and she is currently giving her babies rides on a stuffed horse all through the house. I honestly do not know when she will turn off this evening. I hope it's soon because I am tired!!

Wednesday, December 17, 2014

Ayden Jane's Surprise

Here is the video of Ayden Jane opening the surprise "from Dr. Miller" (remember I asked Dr. M if I could use her in an elaborate scheme to get AJ to drink water at school) Ayden Jane spent a couple weeks drinking an entire water bottle of water at school and thought that I was reporting each day to Dr. M. The kid just needed a little extra push and since she will do almost anything for Dr. M it worked like a charm!

I had told her that if she kept it up and stuck to her bargain of not drinking any fake sugar drinks except on her one day a week Dr. M would send her a surprise. In reality, I found a cute water bottle on sale and really wanted Ayden Jane be excited over the Hint waters so I packaged them up and told her they were from Dr. M.  I figured it was about time Dr. M got to be the good guy in all of this.:) Here is the result and many thanks to Dr. M for playing along. I think we can safely say we have made the transition to drinking water and have left the majority of the artificial sweeteners behind.  Yahoo!!

Ayden Jane's surpise

Monday, December 15, 2014

The Running Bug

I think Ayden Jane has caught the running bug. She has talked about running a few times and even ventured out to run a bit. She just wasn't sold. She would say when I am older...

Yesterday we were hanging around the house and Ayden Jane said she wanted to run a real race where she wore a number and ran with lots of other kids. I told her she had to at least be able to run a whole mile without stopping and then we could look for one.

She responded by saying well then lets go do it. Yep. She ran the entire mile and chattered away the whole time. She was quite proud of herself and is laying plans to keep working and get to run her first real race.

I am thinking about giving her entrance to a local race and a Fitbit to help with training for Christmas. Well, the race entrance is definite - still thinking about the Fitbit.

I am hoping to capitalize on the big kids being home and taking turns running with Ayden Jane. Maybe taking her to various places to keep it interesting. If they don't buy in I guess it will be a busy couple months of training for me!

I enjoy when Ayden Jane decides to do these sorts of things and challenges herself. That determination and drive makes me crazy sometimes but it truly is an awesome asset to Ayden Jane. She has no concept of a challenge too big to conquer. She just fearlessly takes things head on and is then so proud of herself when she accomplishes them.

I guess we are off and running!

Saturday, December 13, 2014

Making Progress

Major breakthroughs in liquid happening here. I know to the average person, getting a child to drink water is not a great feat. I can say that my other kids like water and drink lots of it. Then there is Ayden Jane. I don't understand why but I know it can be a PWS thing to just not like water. Not sure if it is the taste, the texture or something totally different but the combination of not really being thirsty (thanks again PWS) and truly not liking water makes it rough.

When younger and tougher to explain things to, drinking water was just not happening. I wish I could have avoided the use (and addiction) to 'fake sugars' along with all the rationalizing about which ones were more acceptable than others... Not sure if I had it to do again I would be able to do it differently.

On the flip side, so glad we took the stand now. Somehow the timing seems just right! We had two great accomplishments on this front this week.

First, we wanted to go out to eat at a favorite, local, family owned restaurant. We have been many times and Ayden Jane would drink diet coke or iced tea with splenda. We told her we wanted to go but we were just drinking water. She started to balk and come up with ways around...  I just told her we wanted to go but if she could not handle just drinking water than we just wouldn't go. She looked at Gary - She looked at Noah. Both just told her they were just drinking water too. She finally looked back and me and said, "sure we can do that." Again, I know it sounds a little lame to be excited about such things but asking a 7 year old to give up special things and stick to MORE dietary rules can be rough. Honestly, she took about 3 sips of water the entire meal but at least there was no complaining!

Second, on our walk home from school yesterday Ayden Jane was finishing up her water bottle. She had a fun busy day at school and had not had a chance to drink it all. As she was walking and checking to be sure Dr. M would still count it if she drank it on the way home she said, "you know Mom, I like the other stuff better but actually water is not terrible if you give it a chance."

Who knew, just getting her to buy in, a little bribing, nearly dehydrating and a solid week of water being our biggest goal in life would create the necessary breakthrough!  Whew!

Thursday, December 11, 2014

Fake Sugar Part 2

Ayden Jane is doing great trying to remove the artificial sweeteners from her drinking habits.  The only trouble I'm having is that she will just skip drinking all together as the answer.  Arrrrggghhhh.

I held out as best as I could for 4 days. I am trying hard not to fall back on a lot of coconut water or "spiked" water (spiked with juice) as I just do not want to trade one problem for another. (artificial sweeteners for carby drinks) At the end of the fourth day, I told Ayden Jane she could trade her fruit at supper for some apple juice in her water to make it taste good.  She went for it and sucked down about 20 ounces in 3 seconds flat...

So what's a mom to do when the choice is becoming carbs, artificial sweeteners or dehydration?

I knew that Ayden Jane had been coming home from school thirsty and downing an entire green tea or vitamin water or whatever. Now she would come home and grab a Hint water and say she liked it but ... a sip or two is all she'd drink. When I asked her about it she said she liked it but she just couldn't drink a lot of it at one time. So, that meant she needed to sip water all day long at school so that she was not already behind the game by the time she got home.

I designed  a reward system - okay, I resorted to bribery.  I sent a water bottle to school with Ayden Jane and the plan is that if she finishes the bottle of water throughout the day she is rewarded. Her teacher set a great start with giving her a penny (token reward system in her class) for drinking the whole thing and staying healthy. She drank it the next couple days and I told her that Dr. M is so proud of her for giving up the fake sugars and drinking her water each day at school she is sending her a prize! Dr. M was thrilled to find out that she was getting to be the good guy for once and was sending AJ a prize. haha.

I found a really cute kids camelback water bottle on sale and ordered a variety pack of Hint waters. AJ thinks that I report to Dr. M each day whether or not she drinks the whole water bottle and if she finishes out through Christmas the prize will arrive! I think "Dr. M" might even write AJ a note telling her how proud of her she is.

Hey, I figure Dr. M is the fall guy for me a lot. I guess she deserves to be the good guy once in a while.

Sunday, December 7, 2014

Fake Sugar

Okay, so it's confession time. Although I have done a good job keeping artificial sweeteners - fake sugars as Ayden Jane calls them - out of AJ's food, I have failed as it comes to drinks. I have known that they are not good for AJ but have rationalized my way through because she absolutely will not drink water.

Dr. M sent me an article the other day about fake sugar and the negative effects both short term and particularly long term.  She asked me to read through it with Ayden Jane as the answer to all AJ's questions about fake sugar. Then let AJ know she wants Ayden Jane off them completely!  Yikes!! And by off them she means all of them. No rationalizing that this one is better than that one...

So, I talked through all of it with Ayden Jane. You see that means no more diet green tea at home. No more vitamin waters, no more life waters.... Dr M suggested something called Hint waters. They are EXPENSIVE but are sweetener free.  As to what they are slightly (thus hint) flavored with I have no idea what but are not sweet at all. There is no sweetness to the taste but there is a mild taste.  I don't get it...  Luckily, I found 2 flavors at a store up the road and Ayden Jane gave them the thumbs up.  Whew.

Now Ayden Jane was ready to make a deal with Dr. M. No more fake sugars at home but when we go out to eat she can have the usual...  It was a nice try, but Dr. M gave her a counter offer. She could have fake sugars once in a "great while" when we go out.  Of course, "great while" had to be defined for little miss AJ. The answer brought tears... twice a month at most. Ayden Jane and I talked and negotiated and we compromised on once a week. I figure that is a huge win for me in that it really will add up to a big reduction. Dr. M told her she was proud of her for doing her best.

That's when Ayden Jane really got to me. AJ's response to Dr. M: I love you and I will do my best. I know you are just keeping me safe and healthy. I told Dr. M, I wish I could have gotten a picture of the expression on Ayden Jane's face as she said that. Just picture sad-heavyburdened-determinedtomakeyouproud-feelingloved. 

Wednesday, December 3, 2014

Pirate Voyage

I went on the first grade field trip today with Ayden Jane.  Her teacher asked if I could go because it was a dinner theatre sort of thing. Just imagine pirate acrobats, sea lions, Christmas story....  And lots more combined. The kids loved it

I was there basically to manage Ayden Jane food wise. The menu was pirate fare, all things eaten with fingers:cheese biscuit, potato wedge, chicken leg, corn on the cob and a cookie. They served the kids either coke or water to drink. Obviously AJ had water.

We knew the menu ahead of time so Ayden Jane had asked if I could bring her a couple things to replace what she couldn't eat. We are working on independence so it asked her what she thought. She decided I should bring a cheese stick and a square of dark chocolate.  She said she thought she could have the chicken and corn on the cob and cheese stick for supper and eat the dark chocolate when everybody had a cookie.  I thought it was a great plan and Ayden Jane did great.

The potato wedge was actually a half a seasoned baked potato.  Ayden Jane asked me about it and I told her that potatoes are a lot of carbs. She asked if it would shoot up her insulin and make her feel bad and not enjoy the show. I told her I didn't think it would cause too much insulin but that she really hasn't eaten potatoes before. She decided she would like just a piece of it to be safe and she thought she'd be okay because she wasn't even eating any fruit.  I told her that was great thinking and she did great!

I love watching her learn to navigate these sorts of things so well and set herself up to feel good and have a great time.

Tuesday, December 2, 2014

Return to School

I will back up and post some video of roller skating and other fun stuff we did over break but since my computer is in the shop and I'm not good at doing all that from the iPad I will move on to the return to school.

Ayden Jane has been getting frustrated with a program called accelerated reader. In this program the kids read books and then take tests to earn points. Her school does a great job with rewards and Ayden Jane wants in on those points!

She has done okay ona couple books but is struggling a little. My guess is that she is in a hurry to get points and does not read the questions carefully...  Then she gets so upset when she doesn't get enough answers correct.

She told me about it a few weeks ago but I did not communicate how frustrated she is with it to her teacher like I should have. So yesterday when she came out of the building she burst into tears. We talked through it and decided to go chat with her resource teacher. Her classroom teacher, mrs. C, would totally help too but since AJ has an iep but has yet to ever need support I thought I'd see if she wanted to tackle this one. Honestly I think AJ just needs to slow down and do a few well to get the hang of it. Then she should be fine.

In the meantime she will go at the end of the day to mrs. R's room and reread the book with her or the assistant and then they will listen to her take the computer test. Basically, make AJ read it out loud so she will slow down.  The cool thing is mrs. R has not started the program with her kids so Ayden Jane is going to "teach" her all about it.

This is all a perfect example of why Ayden Jane is doing so well. She is absolutely determined to keep up with the other kids in her class. It does not matter to AJ how hard it is sometimes she will find a way to achieve the same things!  Sure it's sad to see her cry, but I can't help but be amazed as the tears are never combined with thoughts of giving up. Just an expression of needing help to form a new plan. 

Thursday, November 27, 2014


Today is Thanksgiving. Our little family has much to be thankful for. Our joys far outweigh our struggles. My house is full with happy, healthy, active kids with bright futures. Gary has the day off and we are prepping for a full day of thanksgiving and family fun!

I get a lot of questions about Thanksgiving and Prader-Willi syndrome. For some families Thanksgiving is rough because it is filled with stress. I know. Strange statement but it is true for many kiddos with PWS that stress and excitement tend to come hand in hand. Excited about the big meal but already thinking, plotting about what they will get to eat and hoping for extra treats....  Wondering how much of this or when or if the days schedule will be messed up. Wondering if food will be unattended or if aunt Sally will remember to bring ________.  It's rough for those families.

It can be stressful on parents as well, especially if family members or guests are not on board with PWS. Will Uncle Fred slip treats to little Johnny even though he's been told not to. Will the herd of cousins leave uneaten food or drinks lying about. There are strategies for dealing with all of these issues but I am no expert on any of them.

One of the things we can add to our list of what we are thankful for is that Ayden Jane is not stressed at all by Thankgiving. She loves the "feast " and really loves that her entire family is all home. (We should have a few more years where that's the case).  She simply asked me if she can have some of everything she is allowed to eat. Of course the answer was yes and off she galloped to play.

We make a few adjustments, like this year Noah suggested we make her some chocolate covered strawberries to enjoy while the rest of the crew eats a super sugary, strawberry jello salad with pretzel crust thing.  She does get mashed potatoes with lots of butter and thinks she is a queen!  How could we not give her grandmas famous mashed potatoes though... She enjoys the green bean casserole, lots of turkey (usually wants the whole leg all to herself) and any other vegetables.  Sometimes I bake her some apples and walnuts with honey and cinnamon but didn't pull that one off this year.  She is unconcerned about skipping rolls or stuffing or her brothers baked Mac n cheese.  Things I could do for her that may come in handy down the road are make Parmesan crisps or other carb free crackers or even cloud bread.

Honestly, Ayden Jane just makes it easy because she is so thrilled by the little gestures. Noah's thoughtfulness of the chocolate strawberries has already made her Thanksgivivng. So, I will keep other families for whom holidays such as this are challenging to navigate in my thoughts and prayers and thoroughly appreciate the gift of yet another relaxed, joyful Thanksgiving. 

Tuesday, November 25, 2014


So some follow up/adjustments on the questions and answers from Dr. Miller and an update on the less is more approach.

First of all the calorie calculation. I did the math and by the formula Dr. Miller gave us Ayden Jane needs 1200 calories a day. Honestly, the couple times I have checked her calorie intake (maybe once a year?) she runs more in the 1600 + category.  So significantly over 1200. Secondly, the answer to Ayden Jane's question of number of carbs per day surprised me a bit. Dr. Miller's recommendation for Ayden Jane (and this is one that varies by child) was for her to eat less than 30 grams of carb per day.

So yep.  I had some things to think about. Ayden Jane being Ayden Jane wanted to count up the carbs in her day. She was getting at least double that many!  Mostly because she was eating grapes and oranges and other more carb heavy fruits with nearly every meal and snack.  I also use a bit too much honey to sweeten some things.

I tossed the question back to Dr. M as to whether I needed to cut calories. Also told her that by our count Ayden Jane was eating double the carbs...  She replied that Ayden Jane looks good. She said to NOT cut calories because AJ burns them like crazy and needs those extra calories. As for the carbs, that is where we should make adjustments. Not that we have to because things are going fine but that if I want a lean, mean, AJ machine trimming off those extra carbs would make it happen.

So, back to Ayden Jane. After reviewing our carb counts she asked me, "mom, so what are we going to do about this carb situation?"  We decided that since she can now (finally) swallow capsules that her carnitine fumarate capsule will go down that way and we will be able to cut some honey. Next up we decided that we will replace some of the servings of the higher carb fruits with berries.

We are now about a week into less is more lunch and a few less carbs. At this rate she may just lean out over the holidays!  I must admit I forget she is just a little first grader sometimes. She is working so hard to be the healthiest, smartest, happiest kid around. So far she has not let PWS slow her down. 

Friday, November 21, 2014

Less is More

Ayden Jane has amazed me once again.  The other day, she asked to pack her own lunch. You would think that she would pack a FULL lunch but on the contrary. It was a light lunch. In fact, we asked her if it was enough and offered ideas for more to pack in it. The answer was fascinating: I always eat everything you pack but when you pack too much I feel yucky by snack time. Like my sugar is low. 

Now, we have been fighting this dropping sugar thing for a while and I have talked through it with Dr. M, tried adding extra fats or extra proteins...  With no other ideas left and still feeling like something wasn't quite right, I just sort of decided that the early snack would have to get her through and I would just have to hope she out grew whatever was going on.

So I tried Ayden Jane's less is more technique with her lunch. I am happy to report that after 4 days of the new improved 'less' lunch Ayden Jane comes home from school less hungry and with more energy.  Yep, you read that right. I asked Dr. M if it is possible for her to simply be over producing insulin in response to a bit too much food after a relatively long time without food. (4 1/2 hours). That's what we think was happening! Then, since she is not starting the roller coaster of high insulin, low insulin... she feels better, has more energy, and is less hungry.

I'm glad one of us was smart enough to figure it out. Today she was checking up on me. As I handed her lunch to her on the way out the door she stopped and said, "Just 3 things right Mom? Not too much?"

She has been a happy bundle of energy this week. On the second day of the less is more trial she came home and didn't even make it inside before she was playing with the dogs and having a blast. No snack at all. So much energy I pawned her off on Gary and they took the dogs on an adventure.

So cool that this kid is starting to be able to read the signals her body is giving her.

Tuesday, November 18, 2014

FPWR conference - Hope

I returned from the trip to see Dr. Miller to turn around a few days later and head to the Foundation for Prader-Willi Research (FPWR) conference in New York. I missed the last one in San Antonio and had really wanted to get to this one but it seemed unlikely.  A few last minute things fell together and I was off! All I had to do was drive to Richmond and stow away in Ali's car.

I will cover some of the research stuff tomorrow, but for today I want to explain a bit of the other stuff you take away from a conference like this. You see, on a daily basis I do not run into any one in Ayden Jane's schools or on her teams or at our church or .... who deals with PWS. We have lots of fabulous friends who are supportive, have taken the time to learn, love Ayden Jane, take the extra time it takes to chat with her sometimes, and even don't walk away when I need to talk about it.  lol

When you get to the conference ALL the people there live daily with a child or grandchild with PWS. No explanations need to be given - about anything. Our kids are not the same, our journeys are not the same, our challenges are not all the same, our approaches are not even all the same but we are all working toward the same goal. We want to celebrate all our kids CAN do and work to find answers to many of the common challenges of energy balance, chronic hunger, speech delays, anxiety, social skill deficits ... 

So we, parents on a mission, learn all we can and listen to long scientific presentations that make us wish we had paid more attention in science class. We rally around hope. We find ways to use our talents to support the research. We rally around hope. We celebrate the research and fundraising accomplishments of the past year. We rally around hope. We cheer the accomplishments of each others children. We rally around hope. We learn from those ahead of us on the journey and support those behind us. We rally around hope.

There is so much more, like how much fun it is to play and squeeze all the little ones but the most amazing part is that the hope we have been rallying around for the past 7 years of our journey is turning into reality. The amazing technical research is making it to the point where treatments are developed and being tried. Sure, there are lots more hurdles and so very much more we don't know and need to do, but the forward progress is astounding.

There is no way to properly say thank you to the brilliant folks and talented people that are so instrumental in this fight. Can't wait for Austin next fall!

Monday, November 17, 2014

Great Conversations - Part 2

I have so much to say, but I think I will just post Ayden Jane's questions along with Dr. Miller's answers for tonight.

AJ: How do you know how much your body needs in a day to be healthy? and how do you decide how much of each kind of food?

Answer: Based on how tall ou are, how fast you are growing, and how much you weigh (compared to how much we want you to weigh), doctors decided how many calories you need in a day to keep you healthy.  For you, personally, it should be about 9 calories/cm of height/day.

PLEASE: notice that things like numbers of calories are specific to each person so if you are unsure find a doctor to help you calculate what your unique body requires.

AJ: Why can my body not use some sugars - like bread and cookies but it can use other sugars - like fruit? How do you know?

Answer: The difference is in the type of sugar found in these things - fruits have fiber in them, that allows the sugar to be digested more slowly in you rbody, so the sugar can be used for energy vs. turned into fat. If you eat what is called "simple sugars" which are grains, cookies, potatoes, pasta, they do not have fiber and so get digested quickly, which causes your insulin to increase a lot and that turns all of the calories directly into fat and also causes your blood sugar to fall lower and the fall in blood sugar will make you more hungry.

AJ: What is bad about fake sugars if they do not make me feel bad?

Answer: Fake sugars do exactly what simple sugars do to your digestion - they cause a huge release of insulin and insulin causes all calories to turn into fat and also causes your blood sugar to drop.

AJ: How many grams of sugar is too many? per day? per meal/snack?

Answer: It depends on the person, but in general, I like to keep the amount of sugar at <30 day.="" grams="" p="" per="">
AJ: Why does my belly tall me I'm hungry sometimes even when I know I already ate?

Answer: Unfortunately that is part of Prader-Willi syndrome, but it can happen to all of us. It tends to happen when we are sick with a cold, when we are tired, and if we eat too much sugar or fake sugar. The only way to fix it is to eat a small amount of protein (a bit of cheese, a few nuts) to settle your stomach and then that feeling will go away.

AJ: Are you close to having the medicine to fix my Krebs cycle?

Answer: Yes - the study for the medication that should help it will hopefully start in December.

Tuesday, November 11, 2014

Great Conversations - Part 1

It was such a great trip to see Dr. Miller.  We made some new friends who were also there that day and had all our questions answered.  What more can you ask for?

On the way down, Ayden Jane was thrilled to find out that she was going to get typed up answers to her questions.  She thought it was so funny that she had given Dr. Miller homework! During our appointment we received a copy of the answers to refer back to which was great! Especially since Ayden Jane tends to alter what she heard over time to suit what she wants....

The most awesome conversation Dr. Miller had with her though was when Dr. M drew her a diagram on the exam table paper. She walked her through how food enters the mouth, travels down the esophagus and into the stomach where it is digested.

Here is a paraphrased version as I didn't hear it all, so I am relying on Ayden Jane to explain it. 

They talked about how the stomach digests simple sugars very quickly and they are then rushed into the intestines. When they hit the intestines fast in a bunch the intestines tell the pancreas to send out lots of insulin. She explained that the insulin then roams around in the body and sucks all the energy it can and stores it away into fat. (AJ already knows that her body can't use the sugar for energy since it won't enter the Krebs cycle - that was last summer's lesson)

So, after the insulin has been high and sucked up all her energy and dropped her blood sugar down to low, she feels yucky and her belly/brain starts to say it is hungry. (just like it would for us, just magnify it)

So, in the end, the goal is to eat in such a way that the insulin does not spike so rapidly or high. If there is not too much insulin the body won't store energy in the fat (make her gain unnecessary wt) and she will not feel hungry. In fact, the body is incapable of gaining weight without insulin ... Makes me think twice about what I'm eating too!

So why are fruit sugars okay, or at least better, than simple sugars like in bread, pasta, cookies... ? Because the fruit has fiber which slows down it's digestion so the sugar is released slowly. That means the insulin does not burst out either so there isn't too much insulin floating around to cause the problems. 

After the appointment when we were chatting over lunch, Ayden Jane summed up her time with Dr. Miller: I like that Dr. Miller talks to me like a big kid. She makes it easy to understand how all the big words I know fit together and explain what's going on inside me. 

Apparently, all this time AJ has been listening and learning and just frustrated that she only had the pieces to the puzzle but couldn't figure out how they all went together.

Wednesday, November 5, 2014

Time to Step it Up.

Wow.  It's November. I was not good with posting in October but it is time to get back to it.

Ayden Jane is cruising along. She is staying healthy (more on that coming soon) and is just easy these days. She has so much 'verbal energy' there are times my ears hurt. It is all good though because she is talking about all sorts of things and is not arguing or 'stuck' or just questioning for the sake of questioning.  It is a very typical kiddo spouting off every thought that comes into her head.

Most entertaining is first thing in the morning.  Mckenna is my least talkative child and the polar opposite of a morning person. Let's just say that Mckenna does not appreciate Ayden Jane's zest for mornings...

Lots of things are coming up around here.  Ayden Jane and I will head to Florida to see Dr. Miller next Monday.  AJ is very excited and has a list of questions prepared for Dr M.

1. How do you know how much your body needs in a day to be healthy? (I have introduced the words metabolism and calorie) and how do you decide how much of each kind of food?

2. Why can my body not use some sugars - like bread and cookies but it can use other sugars - like fruit? How do you know?

3. What is bad about fake sugars if they do not make me feel bad?

4. How many grams of sugar is too many?  per day? per meal/snack?

5. Why does my belly tell me I'm hungry sometimes even when I know I already ate?

6. Are you close to having the medicine to fix my Krebs Cycle?

It should be fascinating to hear the answers. What is most amazing is that Ayden Jane really wants to know and Dr. Miller is so great at explaining really complex things to her in a way that she totally grasps them.

Tuesday, October 21, 2014

Busy - Life's Theme

This fall is racing by. Ayden Jane is doing great in school and she is participating in several after school activities.  She is one happy, busy little first grader.

Mondays she has school, of course, and then swim team for an hour. It is a great little group of kids and the coach does about 15 - 20 minutes of a 'land' work out with the kids first and then they swim for about 45 minutes. Ayden Jane is the youngest and she is doing great.

Tuesdays she still has hippotherapy (therapeutic horse back riding). She has been going to the barn since she was 18 months old. She works hard on the horse doing lots of strengthening exercises and really loves when she gets to jog.

Wednesday she has soccer practice for her little rec soccer team. She does her best and is improving. She has a long way to go before you could call her a soccer player, but she runs around and has fun so it's all good.

Thursday she has her other day of swim team.

Add in there a couple of dog walks or bike rides or trampoline time or playgrounds or beach time and we manage to keep her pretty active.  She is of the mindset that the busier the better so off we go.

Instilling the idea of daily exercise has been a great benefit to Ayden Jane. Since she is a routine kid she does not feel that any day is complete unless she has had some exercise! What I notice is that daily exercise keeps her energy level high and her brain sharp.

I know, it's just like so many other things for her dealing with PWS. It is actually true for all of us that we would feel better, think better, sleep better and be happier if we committed to daily exercise.  It's just that our typical bodies can cheat on that and still get by.... No pass for Ayden Jane, but she doesn't mind. She thinks it's wonderful that we take long walks together or bike rides or that we  'have' to get exercise. She uses this need to get time with me, her Dad or her siblings - but of course we don't mind.

Thursday, October 16, 2014

Parent/Teacher Conference

The first quarter of first grade is coming to a close. Not sure how that is possible, but it does bring on time for parent/teacher conferences. I was a little nervous for Ayden Jane's; not sure why. Just as background, yes, Ayden Jane does have an IEP, but it is solely for the purpose of receiving OT. She does not have any other support and is a typical kiddo in first grade with a few quirks that deals with a lot of medical crap with style.

The numbers stacked up like this. Her standardized testing which is a 'MAP" score done on computer placed her as average and low average with percentiles in the 40's. Of course I would rather not see 'low average' written on things, but since the word average is on there I am good with it!! Yep, not one area was labeled low or below average.  Woot!

Next up was the running record. AJ"s school uses the Fountas and Pinnell running records for reading as a way to track reading level. Ayden Jane finished last spring as a level C. She is now sitting at a level E. I'll take it!

Teacher feels that Ayden Jane 'gets' the math they are doing. She just is sure to position herself next to AJ during teacher directed activities because AJ will decide she knows what she is doing and try to work ahead. The problem comes in when Ayden Jane takes off and is wrong she is one tough kid to reel back in!

Her teacher is concerned about the quality of Ayden Jane's handwriting because she feels the second grade teachers will not accept it and will melt AJ down over it. I told her that I am not too worried about that because she is writing letters now quickly and the quality of her sentences is excellent. She is able to come up with good, grammatically correct sentences. She can spell phonetically and come quite close to the correct spellings. She is spacing between the words all the way through the sentence and it is all a fairly smooth process. I asked her to just keep doing what she is doing because I see huge improvements. I just really think that if we can keep the ability to put thoughts on paper flowing, we can clean up the quality of the handwriting in time.

Mrs. C says that Ayden Jane is always happy, never a behavior problem, works hard and listens intently. Nope, can't ask for any more than that.

The only other thing of note she added is that when she works one on one with Ayden Jane, that is when she sees just how much Ayden Jane is picking up in school and how smart she is. I just love that Mrs. C sees the smart little brain in there even when her ability to express what she knows is compromised.  

Sunday, October 5, 2014

Let it GO

No. That is not a reference to Frozen. As Ayden Jane turned seven this week we had so much to celebrate and be thankful for. We did that! There are so many ways that Ayden Jane is blowing PWS out of the water and making it seem like just some little thing she deals with. Yep, kicked some butt at swim team, reading like a champ, can trade wits with a teenager, manages to be thoroughly happy without sugar in her diet, fearless in her approach to life... I could go on.

Just when you start to be excited about these things, PWS manages to sneak up on you. Well, it snuck up on me, not so much Ayden Jane. Saturday as I watched her soccer game, PWS just glared at me. Her team played a team of girls all her age that train together and are prepping for club soccer. They are sweat kids, good friends and several are in her school. They are the group of girls that would likely be Ayden Jane's good friends ... IF she had all her of chromosome 15 intact.

Instead, Ayden Jane wandered around a bit lost. Honestly, she was super tired from the awesome birthday week. It was her first game so she was still 'taking it all in' as she does during the first of most anything. She was more concerned about drinking and going to the bathroom than playing. She was obviously not in sinc with the rest of her team, or with anyone else for that matter.

Now I need to take a lesson from Ayden Jane. She was happy. She was not worried about much of anything, she was just enjoying a pretty day on a soccer field and doing what she could do and not worrying about she couldn't. I don't know why I let it bother me when she lags behind upon occasion because it doesn't bother her. Generally, she kicks it into gear in her own time and it all comes together.

Somehow, I need to figure out how let go of the things that she is just not going to be great at, even if she wants to be and be thankful for the things she does do well and the incredible can do spirit she has. I guess that sounds pretty obvious and like it shouldn't be too hard. It's just that it sneaks up on me. I get angry that her body is uncooperative and takes away the ability to simply run and play with ease. I begin to go down that dangerous path in my head of thinking about what she would be like if she had all of her DNA. I begin to allow bitterness for what she cannot do and the struggles she faces creep in when I should focus on how awesome she is and all the things she CAN do.

Saturday, October 4, 2014


Ayden Jane was properly spoiled for her birthday. Last weekend, the prebirthday activities began with Kayla and Allie coming in from Clemson. They rode bikes to the beach, swam at the pool and played whatever Ayden Jane wanted for the entire day on Saturday. (which actually started more like Friday night) Ayden Jane is one lucky little 7 year old. They even showered her with presents!

Next up was the actual birthday. She asked to take chocolate dipped strawberries as a treat for her friends. It worked out great! Some of the kids looked at them like they were foreign objects from some crazy planet, but in the end almost everyone loved them. A few even said it was cool that they were on Ayden Jane's 'magic diet'.  (see the book Gordy and the Magic Diet) After school she had soccer practice and then Grandma came to visit and brought presents. Yay! She loved it.

For the grand finally, we took Ayden Jane to ride her first roller coaster. To say that she loved it is quite the understatement! She loved all things fast, high and spinning. The more wild, crazy and dangerous, the bigger the smile and greater the joy.

Sorry the pictures are not the greatest, but lighting and the speed at which they were going were a challenge.

Tuesday, September 30, 2014

Fine Motor and Written Expression

It has been fascinating to watch Ayden Jane work on learning to express her thoughts on paper.

I am happy to say that she can form great complete sentences in her head. She totally gets what a sentence is and attempts to express herself in complete sentences. She is also doing well with spelling. She spells well phonetically so short words that follow the rules are not a problem. She does not do great spelling (nor great reading) sight words but it is coming. All very first grade things.

Now, when you sit with her and she attempts to write a sentence you start to really understand. She just has a really hard time holding the whole sentence in her head and then doing all the little pieces to make that whole sentence come to being. The first word is usually good. It is spelled correctly and generally there is a space behind it. Then the next word will usually have most of its letters but the space behind that word disappears and a trail of a few letters from the remaining words is tacked on.

Last night we worked on it and I told her I could see the first word 'Here' and the space, but read the rest of the sentence as one long word. I asked her where the other words were. She looked at me confused. I asked her to tell me her sentence and then tell me how many words it has. She did it orally with no problem. So I told her to look at her paper and tell me how many words she wrote. She looked and then started to giggle. "Why does my brain stop working when I write my sentences?"

She erased the line of letters and I watched (and helped a little) as she completed the whole sentence. As she finished a word she would recited her sentence to herself again and it would take a minute to figure out what word she should be working on. Then she would try take a minute to use her finger and try to figure out where the word should start. Generally, by this time she didn't remember what word she was writing and had to start reciting her sentence again.... and repeat.

I was amazed both by how hard this process is for her and her perseverance to stick with it until the very end. 

Monday, September 22, 2014

Little Things

I have so much to catch up on but instead I'm going to 'talk' about the little things that have made my crazy days wonderful.

There are times when Mckenna has zero patience for Ayden Jane. Yep. There are times when I have zero patience for Ayden Jane. Typically, these times are in the morning as Mckenna is the polar opposite of a morning person and AJ wakes up talking. Luckily, Ayden Jane is out of the car in just a few minutes as we head to school in the morning. Mckenna proceeds to complete the ride in silence. Occasionally I try to talk but only grunts come in return and nothing is remembered so I generally just go with silence and then text her during lunch if I need to let her know something.

On the flip side there are times like this past Sunday. Mckenna was sitting in church with AJ waiting for things to get started. She began a game where she was writing a word and AJ had to try to read it and draw what the word said. Then they would switch and Ayden Jane would write a word and Mckenna had to draw it to guess what it said.

Ayden Jane loved it! She loved the game, the attention and just being able to play something that was big kid like.

Secondly, Ayden Jane brought home her spelling test. Yep. 100%. We worked on the words a little at a time through the week and she did great with the few she didn't know right off. I know it was not a hard test but I was still so excited. Just to pile it on, her teacher made a note on the paper that said she asked Ayden Jane spell them orally as well. Why is that a reason to jump for joy? I just love that Mrs. C has Ayden Jane do the written work like all the kids but takes the time to go behind and make sure to ask her to tell her orally. She places high expectations but makes sure that there is support.

To top it off, Ayden Jane wrote her first sentence with words spaced and mostly spelled properly. I have made many attempts to get Ayden Jane put space between her words as she writes but she would just tell me she did. (she didn't!) Somehow Mrs. C worked a miracle! It was so fun to watch AJ spell each word and then space them out.

Little things that are such big things.

Sunday, September 14, 2014


Wow, what a couple of weeks. Ayden Jane and how her body responds to illness or stress is such a mystery. She had the short lived virus and then as she began to recover we had the blood sugar drop I posted about a little ways back. I wish the story ended there.

I basically noticed trouble maintaining blood sugar through the school day.  Ayden Jane was daily bottoming out, mostly likely due to the amount of energy her body was putting into refueling whatever it is she depletes when ill added to the energy it takes her to concentrate and just do school. Once she was behind and her sugars down she was thrown for the day. We played catch up after school but it was wiping her out something fierce. 

I was called to school the following Monday because Ayden Jane was in the nurses office complaining of a headache and that her brain wasn't working right. I brought the glucose meter with me and she came in at 71! I chatted with Ayden Jane and realized that she was not getting snack when I thought. I was told snack would be about 1:00, before recess. It was actually not happening until after 2:00. She just couldn't wait that long. 

I adjusted snack and, the following day, went to check on her at about 12:40 to make sure she had it on time (she had a substitute). When I got to her she said she had snack but still did not feel good. That's when I realized she was about 1000 degrees. The air conditioner in her classroom was not working and she was simply overheating. I went to the administration and we came up with a plan to cool her down. Finally, nearly 2 weeks after a simple 24 hr virus she could adequately recover.

I kept a very close eye for a few days, maintained her sugars as evenly as I could... and finally this Saturday she had loads of energy!

The frustrating thing is that she puts on so much weight when her body goes through this process. In talking to Dr. M we feel like somehow after and maybe during illness, when her body is stressed she begins to store energy. She stores energy even when she does not have enough energy to adequately function!

We are thinking that there might be an underlying adrenal issue. Not a critical or acute one, but enough of one that after the initial response to illness is mounted the adrenals are depleted.  We are going to try stress dosing AJ with Cortef at the first sign of the next illness and see if we can head this whole awful cycle off at the pass.

Sorry for all the 'science' rambling... I'll try to be more entertaining next time.

Monday, September 8, 2014

Visiting Noah


It was a beautiful day for a trip to see Noah at Coker.  Ayden Jane was very excited and so was Noah! We arrived quite early, just as the team was beginning to warm up. Noah jogged off the field and was greeted by Ayden Jane running toward him.  So sweet for a momma to watch. Giant hugs and giggles.

I don't have pictures (but I should) of the surprise Ayden Jane received that same night. Kayla and Allie came from Clemson to watch the game with us. I had not told AJ they were coming. At first it was because I wanted to be sure it was going to happen. Later, because I did not know when they would arrive and did not want to hear the constant, "can you text them and see how many more minutes?"

It was a fun evening of soccer watching, chasing and playing on the sidelines and visiting with Noah at Zaxby's after.

I wish the picture was more clear, but it was so cute as Ayden Jane ran across the field to get Noah after the game just like she had done for years. She held his hand for a little while as they walked and she just looks so little next to him.  Holding a hand seems like such a simple little thing, but it is one place we have always seen/felt AJ's sensory oddities.  When little she would absolutely NOT let you hold her hand. It made for interesting parking lot navigation! Now, she occasionally reaches and holds your hand for as long as she can stand it. Know if she takes your hand you are special!

Friday, September 5, 2014


Not going into details, but there have been some... ummm, challenges that our family is facing unrelated to PWS.  Yea, I know!  Surprisingly, other stuff happens in our lives too.

This evening was a rare treat. We had nothing else going on and Gary and I took Ayden Jane to the beach.  We were there a long while and I saw this rainbow up in the clouds. The sky was bright and the weather was beautiful. Far in the distance you could see a bit of a storm brewing and this rainbow was just out ahead of it and barely visible because the sky was so bright.  I had to zoom in and hope it would show up on the photo.

Over time the storm grew slowly closer. As the backdrop of grey from clouds and rain formed thicker behind the rainbow, it caused us to be able to see the rainbow more clearly.  More defined. More solid ahead of the storm.

Finally, we were chased off the beach because the wind was getting strong and cool, and the impending storm was close. This is the point, though where you couldn't help but stare at the rainbow. It held strong as it's colors shown bright 

On this day, I could not help but thank God for his promises. His promises that stand true in sunny weather and when the storms rise. He is there when things are good, but sometimes it is harder to see Him because we are enjoying life.

As the storm approached and the sky got darker it seemed as if the rainbow was staying out ahead of storm. Like the rainbow was guiding the storm and maybe even had the power to calm it.

In the end, as the winds blew and the ocean became rough and even the birds seemed to be uncertain, the rainbow shone calm and bright and strong, unwavering from the weather around it.

I choose to trust in God's promises of provision. God has provided for us on many a clear sunny day. I don't often remember to thank Him or, sadly, even notice often enough, but I know it is true. One look at the incredible people our kids have become and I know God has been there through many sunny days.

He provided in countless ways when  Ayden Jane was tiny and a horrific storm enveloped our family and our world was crashing - strength beyond measure. As Ayden Jane grew and I had so much to learn - wisdom and discernment. As the strain of me not working mounted - financial provision. As the minutes turned to hours turned to days turned to years - patience. As our little family pulled together under His guidance - love.

I hold onto His promises and am so thankful for the rainbows. I am thankful they stand out during the storms and I will be sure to look hard and find them when the sun comes out again.

Tuesday, September 2, 2014

Things Missed

I'm not sure exactly where this post is going to go. It starts with thinking about my big kids and just how big they are.  I am so very proud of the young men and women they are becoming and I have the typical thoughts of how I blinked and missed them growing up.

Only, it's more than that. This is one place having a child with special needs or chronic medical needs steals a piece of you.  I guess that sounds harsh and I don't mean it in an angry way toward Ayden Jane, but rather the frustration of one more thing Prader-Willi Syndrome has caused our family.

You see, I look at pictures of the big kids before Ayden Jane entered the scene. I remember so much. I remember endless basketball games in the driveway with Noah. I remember Kayla, Noah and the neighbor kids in and out of the house. I remember conversations and games they played. I remember Mckenna and her love of animals. I remember her having friends over and being invited places. I remember Gary and I having friends over and going places and hanging out with other families. It was all so simple.

After Ayden Jane was born it was as if I disappeared for a season. I blinked. I poured so much into Ayden Jane (and needed to) I feel a little cheated out of time with the big kids. I wish they could have just waited for me... but it doesn't work that way. They went on and grew up.

Now I don't want to make it sound like I never left the house or spoke to them... It was just that there was always a part of my mind that had to be on Ayden Jane so I was not able to fully enjoy them like I had. For example, watching Kayla's volleyball or Noah's soccer games was great, but there was no way to just focus on the game and enjoy.  You know the chatting with the other parents and cheering as if time outside of the event stopped. No, it was watching the best I could while working on stairs with Ayden Jane or navigating the concessions or just keeping her from falling. I enjoyed the games, but a piece of me was always distracted.

One thing I think Gary and I did right in all of this is that we made it priority for Gary to build/keep good relationships with our kids even though his time was more and more limited as the sole bread winner. Mostly this involved him taking them out to eat wings and watch games. Sometimes it was to see movies or just make a run to sonic for a drink. I won't really ever know how the conversations went, but it was a tradition that the big kids continued with each other even when he was not home. I also love that Noah and Kayla have extended this to Mckenna and will take her places and do things with her. I do know that there was always lots of laughter involved.

It would have been great to be more a part of their times together but I also know that for me to have been there would have made those times less carefree. I wish I had been the involved mom at the schools or sports teams. I wish I had been able to shake the worry or constant thought of how to tweak things for Ayden Jane and just enjoy the big kids.

PWS caused me to blink long and hard and frequently. In spite of that, I am blessed to have been a part of the lives of three awesome 'big kids'. I am thankful that God kept His eyes on them while I blinked.

Monday, September 1, 2014


Last Wednesday, the day after Ayden Jane had been sick, she had a great day at school.  She stayed all day and when she got home she had a quick snack and then PT for an hour.  After PT she trooped off to her room to play a bit before 5 and I woke her at 5:30 for supper.  We were going out to a little local place.

Her snack had been at three so it had been two and a half hours since she had eaten. I could hardly wake her. She typically wakes up freakishly fast like you just flipped the light switch so I knew when she did not pop up to go to the restaurant that something was up.  Honestly, my first thought was that she had gotten into a sugared drink or something. So far she hates the way she feels if she eats sugar so I haven't really had to worry about that, but we all are sort of just waiting for the hyperphasia to set in.

I kept trying to wake her.  She was super groggy but finally made her way down the hall.  Then the eyes rolled back, she lost balance and hit the floor.  I helped her to the sofa and tried to talk to her to see if she knew what was going on. She sort of did but mostly just wanted to go to sleep.

I checked her blood sugar and ketones.  She was at 72 for blood sugar which is not considered very low for most people but is obviously too low for Ayden Jane.  Her ketones were 5.8.  I don't really know that much about ketones but Dr. M told me to keep them under 4.  Whoops!

Now that I knew what was going on I just needed to get her to eat something.  JUST... I actually had to convince her, bribe her and finally force her to eat something.  It really was remarkable how quickly she became alert and reasonable after she took a bite or two.

In the end I am pretty sure that the problem was a result of the illness. I believe her body was just working really hard to recover and that plus school plus PT just required more fuel.

Now, as to why she refuses food when her sugars get low and ketones high?  I have no idea.  Why she did not ask for a snack but instead chose to curl up and go to sleep?  I have no idea. What I do know is that Momma needs to pay attention for her after she has been sick.  Don't even want to think about how far out of range she would have gotten if I had not woken her.

Wednesday, August 27, 2014

First Grade So Far

Whew.  Never a dull moment.  So the first 3 days of school went fabulous!  Ayden Jane took her basketball and played all recess long with her new friend. (and I doubted her) She had 3 great days and her teacher seems to be an excellent match.

I picked her up a bit early on Friday to go get molds made for her new orthotics and chatted for a moment with Mrs. C. Two things I loved! First of all she said right up front, "you were right about her handwriting it is awful!" Now some people might get offended if a teacher told them their child's handwriting is awful, but what it said to me is that she is willing to be up front with Ayden Jane's weaknesses. I was very happy not to get the platitudes of all is peachy keen and all that when it's not. Secondly, she also said that even in the first 3 days Ayden Jane has made it clear that she has it all going on inside that little head of hers. So what more could I ask for than a teacher that will meet her challenges head on and already sees that just because she can't always get it out doesn't mean it isn't all in there.

So, you imagine my surprise when I got a call from the nurse at noon on the following Monday. Ayden Jane was complaining of a headache but had no fever or other symptoms.  I picked her up and she came come and took a nap.  She was not too full of energy that evening but did not seem like she was 'sick.' I sent her Tuesday morning and I got a call about 12:30.  Same deal.  Headache and then she started to say her tummy hurt.  Hmmmm.  Nurse said there is a stomach bug going around with a fever and nausea/vomiting. AJ came home and promptly took a nearly 3 hour nap!  When she woke up she look great and was full of energy. We went to horses and then she kicked a soccer ball with me at Mckenna's practice.

I sent her off to school this morning and I am hoping for no phone calls!  We shall see!

I can add that she has had homework each night this week. Some things were a struggle, but not because she doesn't understand the work, because she is sure she knows more than mom! Actually, the first night was a bit rough but by last night she had it down and it took her just a few minutes to complete.  Little by little we will get there.

Thursday, August 21, 2014

First Grade

This was at the end of Ayden Jane's first day of first grade.  She summed up the day saying that it was awesome, her teacher is REALLY nice and she is sure she will be able to learn everything she is supposed to in first grade.  I'd say that's a success!

I brought her scooters and we rode them home together.  Possibly not my best choice with a heat advisory in effect, but we made it.  She did fall asleep on the sofa after a snack and a drink, but I was not surprised.

She could tell me a good bit about her day and knew just what she was supposed to do for 'homework' (bringing in items to help describe her talent, a favorite and what is special about our family)

Today was day two and it seemed to go just as well.  She did ask if she can take a basketball to school tomorrow.  Ha!  The kid has never played basketball in her life!  Apparently, a new friend wants to play basketball with her so she is all in.  I'm not sure it's going to work out as AJ plans, but there is no telling her no!  I hope it doesn't cause a melt down on day 3 but I'm not going to start telling her not to try things now!  She seems to pull off anything she puts her mind to so I will send her with ball in hand and hope for the best!

Just one of my Marlin moments:)  You know, from Nemo - "You think you can do these things AJ but you can't"  I love how she keeps proving my inner Marlin wrong.

Sunday, August 17, 2014

Getting Ready for First Grade!

I thought I'd post the notes I am using to chat with Ayden Jane's teacher before school starts later this week.  On any given day, Ayden Jane is as typical as the next first grader.  However, there are quirks the teacher needs to be aware of and extra things to keep AJ safe.

I always stick with a one page summary and use as few words as possible.  Lets face it, if it is too long the teacher may read it once, but it will not be useful for keeping around and referring to quickly.  I will meet with the regular ed and special ed teacher together even though Ayden Jane does not actually go to the resource room for services.

Prader-Willi Syndrome (PWS)
Approximately 1:15,000
Diagnosed by genetic testing showing deletion or duplication on the 15th chromosome

There is a very long list of challenges associated with PWS and for a full picture of the disorder you can visit fpwr.org and pwsausa.org. 

Medical concerns for Ayden Jane:

Dietary concerns:
Grain free sugar free diet (no crackers, pretzels, pasta, cookies, juice…)
Ketogenic – her body and brain use fats best for energy
Similar to diabetes, maintaining blood sugars evenly is important
Low metabolism – truly requires less calorie intake than a typical child
Does not feel ‘full’ and will continue to eat if offered – no off switch
Could become hyperphagiac
Extra focused on meals/snacks

Immune issues: 
Does not ‘catch’ viral illness easier but struggles to recover
May have odd skin reactions
Cannot fight bacterial infections – strep throat, sinus, pneumonia…
Please let me know of illnesses that ‘go around’

Odd and ends:
Diminished pain – Let me know if she complains for more than a few min.
Temperature regulation – Can over heat easily, even in winter with coat.
Strabismus – glasses

Sees OT for writing – it is awful ….
Takes in way more than she can express
Started preschool WAY behind her peers and has been slowly gaining since.  Pretty squarely average currently with the exception of handwriting.

Ridiculously hard working and independent – approaches everything with an uncanny optimism and confidence

Monday, August 11, 2014

Out of the Shadows

If you have read much at all of Ayden Jane's adventures than you know she is doing great.  My head knows this.  In my head I know that she has beaten so many odds and that even when she was thrown off track when she got ill, we were able to get her back on track... each time.  My head knows that she is right where she should be academically.  My head knows she gets along with other kids and makes friends.  My head knows that she is disciplined and determined and willing to take on the challenges of navigating food.  My head knows she is smart and funny and sees the world in an amazing, creative way.  My head knows that she has reduced her scoliosis curve and the 'chances' of it returning are small.  My head knows that she is confident and happy.

I think you get the picture.

The problem comes in the heart.  I know she is right where she should be academically, but what will happen in 1st grade?  I know that she is managing really well with food but when will hyperphasia make her hungry constantly.  My heart worries that the ways in which she is different will cause her to be ostracized.  My heart panics a little each time I look at her and think I see her alignment slightly off or her weight climbing.  My heart is scared that the next illness will be the one we can't recover from.  Basically, my heart worries about so many things that I just took for granted with my other kids.  It just casts a long shadow that I pretty much just get used to...

Not to say that there isn't plenty of joy and awe and even a bit of pride in my heart as I watch her.

Just that there is a constant struggle of head vs heart.  All the great things in I know in my head are often a bit shadowed by the concerns of the heart.  There are days where the things in my head are so clear and bright it is like sunshine.  I love those days. Then there are days where the fears in my heart just well up and the shadows return.  The fears and what if's begin to creep in.  I think the constant fight to stay one step ahead of the next problem, to look at the outside and try to 'see' what's going on inside, to live a 'normal' life when things are in fact, not 'normal' just take a toll.

As I get ready to send Ayden Jane off to first grade in about a week, I am excited that she is doing so great.  But those shadows.  They keep following me and casting doubts.  Funny things about shadows, they dull the brilliance of light and make you see things that are not really there.

The best I know to do is to use my head and ask God to take charge of my heart.  Only He can calm the fears and doubts and give me the patience and strength required to fight the daily, exhausting battle of raising such an amazing, complicated little miracle.  (Of course, that actually applies to raising all kiddo's)

Saturday, August 9, 2014

Questions on the Beach

It's been a busy week and with all the rain before that, we missed being on the beach.  We headed out today and Ayden Jane had a great time riding the waves.  It is so much fun to watch her have so much fun.

Typically, after she works hard playing in the waves she has a snack.  We usually pack some nuts and a drink and plop down on the boogie board and watch the ocean while she snacks.  Often this is a time when the deep questions (or hysterical comments like, "why are that guys shorts so stinkin' tight?") come.  Her body is calm and she is relaxed.

The question that started things off today was, "how do people get big bellies and does it make them feel bad?"  (the feel bad is in reference to health, not feelings)  Which led to: Is my belly big?  What do they do to make their belly big and how do they make it small again?  Why can some people like Noah eat so much and not get a big belly and other people can't eat very much?  It was the first time I included that people with PWS have to be careful because it is easy to get a big belly and extra hard for them to lose it.

I introduced her to two new words: metabolism and calorie.  She is so ready to understand the entire big picture of metabolism and food and sugars and managing it all ...

The best part is that Dr. Miller is going to take charge of explaining it all to her in November when we go to see her.  I do my best but Ayden Jane finds something I can't explain and then gets stuck on that.  I am ready for her to truly understand the big picture and start the process of managing her own food.  Her discipline is astounding and she truly has no problem cutting things out or limiting things if she understand why.

We will keep a 3 day diet log and send that on ahead so it can be analyzed and Dr. Miller can show her what is good and what needs to be improved ect.  I sure hope Dr. M doesn't have too busy a day because this kid's questions can go on forever and she really, really, really wants to understand it ALL.  It is challenging sometimes because she is 6 and wants to understand stuff that can be tough for an adult to fully comprehend.  (I might have to record it :)

Tuesday, August 5, 2014

Excited About Labs

Lab work is a part of life with a kid who's a bit medically complicated.  Since Ayden Jane's body does not do a good job of monitoring and adjusting to life or getting some nutrients out of the foods she eats it is how we know if all is well on the inside.  Generally speaking, I can tell if something is off on the inside by how well her brain and body are working.

We had labs done last week and got results today.  Everything looks fantastic!!!  You have no idea how rare a treat that is to be able to say.  I don't have the actual labs in hand, but Dr. C emailed a summary of the results to me.  Thyroid - good, carnitine - good, IGF 1 - a tad high but no drop in dose, cbc - normal, cholesterol - good, insulin - good ... the list goes on.

So how does this match the outside?  Ayden Jane has been great lately.  No hunger, no behavior issues, energy to spare, great sense of humor .... just a joy.  Today we spent 7 1/2 hours with friends at the water park.  She took a break for lunch and that was it!  She did more slides than I did and I was exhausted.  I wish I could have counted the number of stairs she climbed and she carried her own mats or tubes!  

If I can just keep this kiddo healthy and keep all bacterial infection out of her little system there will be no stopping her in first grade!!

Saturday, August 2, 2014

A Good Life

A week or so ago I posted about a conversation Ayden Jane was having with Dr. Miller about 'fake sugar' and drinking water ect.  Ayden Jane had made up her mind to drink a cup of water everyday after that.  No small feat as I have tried most everything to get her to willingly drink water!

After a few successful days, Ayden Jane wanted me to email Dr. Miller and tell her...  (not sure how Dr. Miller handles all the email)  Anyway in the process AJ asked her a few other questions and Dr. M told her she was going to have a conference call about a medicine that would help.  Naturally, AJ was thinking about something to fix her Krebs Cycle.

Dr. M explained that this is something different that would help her not crave the sugar she can't have or even fake sugars.  I had to explain what crave meant.  I told her that it means to want something really bad so that it was hard to have it around and not get some, sort of like bananas. Her response was pretty awesome and makes me really glad we have chosen to feed her the way we do.

Ayden Jane was actually sort of confused.  She said that the medicine didn't really make sense and wouldn't really be helpful for her.  She told me she just wants the fake sugars in drinks because she hates water but has to drink something and that she doesn't crave sugar in food.  That she just wants to be able to eat things without having to worry about accidentally eating something with too much sugar and then feeling awful.  I know this is a concern for her as she sometimes will choose not to eat something because she just isn't sure and has things she wants to do that day.  I'd say her biggest food anxiety is accidentally getting a sugar load so she likes to just stick with foods she knows make her feel good.

Now, I know that Ayden Jane is more sensitive to sugar/simple carbs than most with PWS.  I don't know if this is because she has eaten this way for so long or if she would be that way anyway and would have just adjusted to not feeling great.  (that's an awful thought)  What I do know is that Ayden Jane is 100% happy eating the foods she is allowed.  She is 2 months from being 7 and hyperphasia is not an issue.  There are no locks because she doesn't need them.  She is not bothered by food left on the counter and can walk past it without a thought.  She can hang out with her friends while they eat cookies or candy and be totally content with a coconut square or keto chocolate.  We can go out to eat and she is not concerned in the least with foods she does not eat.

Not to say she wouldn't eat more than what she needs of what she does eat.  She is not constantly hungry and does struggle if something comes up and she feels hungry but needs to wait. Her body is definitely not great at tapping into stored reserves of energy like the rest of us can do.  The satiety signal is definitely not great.  Sometimes she will declare herself full, but it is rare.  Basically, she knows when she needs food (most of the time) but would eat more than necessary if she were not using her brain (and us) to limit portions.

It's not a perfect system and I do look forward to someday freeing her from so many restrictions, but it truly is a good life.  She is happy and healthy and content even though she has to work hard and be careful.  She is a fantastic example of doing your best with what you have and enjoying life to the fullest.

Friday, August 1, 2014

Problem Solving

This morning I went out and ran some errands.  Okay, so I took a quiet breakfast to myself at Hardees and read a news paper then ran some errands.  I left Noah in charge of Ayden Jane with breakfast done and AJ dressed.

About 11 o'clock I got a call from Noah.  Apparently, Ayden Jane had asked for a snack and as Noah was talking with her trying to figure out what was okay food wise.... AJ lost it.  Some of you will understand what that means.  It does not happen often for Ayden Jane (as long as she stays healthy) and for that I am thankful!  It is hard to explain if you do not live with it.  Basically, she just gets over emotional, cries and argues and cannot reason or think through anything.  There is not much to do but let it pass and then deal with it.

Luckily, when Noah called I was pulling into the neighborhood.  I diffused things and after a few minutes Ayden Jane was ready to talk about what had happened.  She began to smile and giggled that the argument was silly.  She told me Noah still loved her when she even though she was difficult sometimes.

Then she came out with this question:  "does Prader Willi Syndrome make me loose control for no reason sometimes?"  Wow.  Loaded question.  Short answer - YES.  Definitely did not want to give her any excuses though!  So we had one of our famous long discussions ... which I ended with no matter what the cause, you need to learn to use your words and smart brain to solve problems before you lose it.

Ayden Jane answered, "I'm pretty sure I can do that.  So, should I use my words to tell Noah I am sorry first, or eat lunch first."

A couple times during the day she was getting frustrated with Mckenna (who tends to like to push AJ's buttons...) and she told her, "I am using my words to tell you I don't like what you are doing and I want you to stop.  I'm going to lose control if you don't listen."  What 6 year old talks like that!  She is hysterical and her understanding of her own challenges and effort to over come them continue to amaze me.

Wednesday, July 30, 2014

Look Out, Here Comes August

So as I sit here on nearly the last day of July peeking at the August Calendar it hits me.  Summer is ending fast!!!

August 6 Kayla gets home from Honduras.  Hooray!!  Can't wait to hear the stories of her adventures there.  I think in ten weeks she was able to get a true feel for the place, get bored a few times, build some great relationships, use lots of Spanish, get very attached to cute kiddos, enjoy some vacationing, totally rest her mind from school, learn to appreciate a much slower pace.... oh so many things!

August 13 Noah heads back to Coker College.  He is less than excited.  Preseason soccer in August in SC is going to be pretty brutal.  He has a great room mate set up and is happy to go see friends and start the actual soccer season.  Hopefully the transition to a new coach will go smoothly and the season will be a great one.

August 17 Kayla goes back to Clemson for her senior year!!  I can't believe she will be graduating from college in the spring.  Wow, time flies.  Hoping she has a great senior year and enjoys every minute of it.

August 20 Mckenna and Ayden Jane head back to school.  Mckenna to 8th grade and Ayden Jane to first grade.  Both are returning to the same schools they were in last year but the new school year who will I have for a teacher, who will be in my class excitement is building.

As for me?  I do not yet have a contract to return to teaching but I trusting God to pull that one together for me in his timing.

Well, Gary is working hard on the grill after hours at the beach and the pool, so at least summer isn't over quite yet!!  I am going to enjoy this summer right up to the wire.  This one has flown and I am not quite ready to trade bathing suits for new school clothes or bare feet for school shoes just yet!

Monday, July 28, 2014

Just a Typical Kiddo

Lately Ayden Jane has been in some settings where she is playing with kids around her age that we don't really know.  I am way better at just staying back and letting her figure it out.  It's a little embarrassing to admit, but as Ayden Jane ventured into social situations when she was younger I would often get embarrassed for her or worry about her getting her feelings hurt.  I didn't give her much credit (or the other kids for that matter).  I almost had to either not watch or run interference.  The whole experience would make me uncomfortable.

This summer Ayden Jane has had lots of experience in settings where she joined a group of kids playing.  Swim team was one of them.  I did tell the coaches at the end of the summer how great swimming has been for Ayden Jane and clued them into some of her challenges developing muscle and with motor planning.  That was basically because I wanted them to know how much they have helped her.  The other kids know nothing.  They just accept her for who she is and she has made a good friend a year older.  They both want to swim year round together.

Then this week at the neighborhood pool Ayden Jane joined 3 kids playing with their uncle.  Since we live in a resort area, there are often kids we do not know at the pool.  Ayden Jane struggled to understand the game they were playing and she did not do a great job maintaining personal space with the uncle, but I just let her do her thing.  It struck me that in the past I would have looked at her awkward attempts and been uncomfortable but now I just let her go for it.  She does figure it out and fit in before it is all said and done.  She is kind and tries hard and that's really all the other kids need to find a way to get her in on the games.

Last night there were 2 of her friends as part of a big group of kids playing sharks n minnows in the deep end.  Maybe 10 kids total.  Ayden Jane joined right in (she was close to the youngest) and took her turn being shark when tagged with no complaints.  It took a few extra explanations of this particular groups variation of the game, but she got it.  She swam and played with the pack for two hours!!  One of the boys (2 years older) even attempted to teach her how to do a flip into the water.  It was very cute.  He is the youngest of 3 and was totally enjoying being the big kid teaching AJ a cool trick. 

Now I am not saying that there are no differences between Ayden Jane's social skills and the other kids.  I think, though, that her social skills are better than I give her credit for at times.  She is just a bold spirit so she will speak up when I would shy away.  She will ask questions if she doesn't understand even if it has already been explained.  Then other times, she just feels like playing by herself and will choose not to play with others and disappear into her imagination.

So, I have been doing some of my best parenting this summer by letting her stretch her wings by playing with whoever she wants, or not.  Letting her find her way into the packs of kids.  Mom has been on the sideline watching Ayden Jane grow up!