She is Different

This post is one of those that is rattling around in my head. Parts of it are clear and parts of it make me feel awkward. Here we go anyway.

A couple days ago I was at Shriner's Hospital for Children with Ayden Jane for her scoliosis check. It was awesome. Her curve is measuring 8 degrees but the doc was very impressed with the progression of Ayden Jane's x-rays (honestly, he lost me in that conversation).  He was very surprised by her tone and improvement in her core. He asked what we have been doing and Ayden Jane piped up that she works hard with Mrs. Jen to keep her body strong and straight. I told him she swims, rides horses and we have added running. He was very interested in the story of how things started after the winter she lost her tone and the right leg seeming to be the key. I think he and Jen would have a great conversation about it all.

What really surprised me was after we finished the orthopedic talk, he asked me, "What else are you doing because I have had many patients with Prader-Willi Syndrome over the years and she is different." It took me back a bit for a minute. Usually, I hear she is doing great and is on the mild end of the spectrum....  His simple statement and later expansion to include, "she is not just mild PWS, there is nothing about her that presents as PWS. She is different."

That phrase just keeps rolling around in my head. It still jolts me a little. I mean, I know Ayden Jane is doing fabulously right now but to me, she is just a kid who has worked hard but still fights PWS. It makes me thrilled to hear and at the same time breaks my heart for others. I mean, if she is different - then how do others shift from PWS to different. I mean if she is mild, then others can sort of slide down the spectrum and join her. What does it mean if she is different? Is there a track to jump? How exactly did we make the leap?

It was fabulous confirmation of all we have done over the years. Sometimes I wonder if the time and effort and financial sacrifice of staying home to sort all of this out over the years was worth it. I start to get on myself about how I should really have been able to find some sort of work to fit in with it all to ease the pressures. In the end, I know that God has His hand all over this. I pray for wisdom as I tweak things with AJ and as I attempt to help families who are a few years behind us on this path. I pray that God will use us as He sees fit and I am doing my best to trust Him to guide our next step.

She is different. It will echo with me for a while as I try to understand the full extent of that simple statement.

Perspective

I watched Ayden Jane play at the beach today. More specifically, I watched her work crazy hard to keep up with twins one year older (who are very athletic). She rode boogie boards with them. The waves were big and the current strong but she kept at it. She was a part of the gang and she was very happy to be with them.

In the water she can hold her own. Then they decided to play a beach tennis sort of game. Um, racket sports and Ayden Jane do not get along. The kids were so kind to her. She took turns and tried. The boy did his best to teach her and was so patient but it was obvious that Ayden Jane was not coordinated enough.

As I watched, I was incredibly proud of and in awe of her, and at the same time felt sad for her. It's a strange thing to watch her join in with other kids. Well, to be honest, these kids are typical born and raised beach kids so keeping up with them in the water is really rough. But that aside, watching her attitude as she is fearless to try. Not just try to keep up with the boogie boarding because she is pretty good at that but to continue to try to play the tennis game. She does not get upset or angry when she can't do it. She doesn't get embarrassed that it is hard for her to do. She just keeps trying her best and is thankful to be a part. (okay, I'm pretty sure she didn't realize that she was terrible, but that may be her greatest gift)

So I watch in awe as she is so incredibly happy to just plug away and be a part even though it takes tremendous effort. I wait for her to get frustrated. I think she will soon tire of not being able to do new things the first time. I couldn't be more wrong ... for now.

I pray she will always have the innate confidence to try new things and join in with new kids.  Along with that, however, I pray she will continue to find kind children. We have been blessed so far. She has stumbled across mostly all patient children who have been very kind to her. She feels like the pool, which is a vacation spot in the summer, is her own personal endless sea of new friends to be made. She talked a crew of 4 cousin's she met at the pool visiting from another state into playing a game with her. She plays fair, doesn't complain when she gets caught or is it and smiles the whole time.

I still wish things came easier and she was truly good at some things, but I need to learn from her that the joy really is in just playing the game. Being the best or winning is not really a concern.

Cranking up the Carnitine

So as usual, I was not satisfied with things going well. I needed to know if they could be even better! I did the math and decided I was going to go for a third dose of carnitine. It will put Ayden Jane at a total of about 46 mg/kg.

For 2 days (today being the 3rd) I gave Ayden Jane a third carnitine dose at supper. I was worried it would be too much. That she would not sleep, or she would get edgy or her mind would race and she would not be able to organize the energy.

I admit, the first night there was not a lot of sleep. She was up several times and was even playing with the dogs during the night. The first full dose day was not as crazy as I thought it might be. She did go supper hard and then fall asleep - not unexpected. Her focus was actually improved though, rather than scattered.She managed to organize 4 other kids at the pool into playing a game of categories with her which was pretty funny to watch.

The second night she slept better, a bit restless - she came in with me while Gary was at work - but better. I was very curious how today would go. She did great! No lull, played independently for a long time with her dolls, took the dogs on a very long walk with me, swam hard in the ocean fighting rough surf and keeping up with some friends for 2 hours, more playing at home, helped clean up and read her magic tree house book wonderfully before bed.

So far, I think that the dose of 46 mg/kg/day is going to be the winner. She is eating 3 meals but honestly not really asking for snacks. Well, other than the fun blueberry/coconut milk push up pops we made today. Her energy has maintained with meals: breakfast between 7-7:30, lunch 11-12 and supper 5-6. If we have been super active, she may have an afternoon snack or choose to have supper early and late snack if necessary. It is wonderful not to have to take food with us everywhere we go!!

So Fun

The added carntine has been just the ticket for Ayden Jane. Her energy level has been great, she is leaning out without really even trying and is just plain happy. Along with the added energy has come having much more fun with her friends and the disappearing of her heat intolerance.

Examples would be that I noticed the heat was not bothering her on our morning walk/runs. Even if we did not get out the door as early, she was not phased. Previously, she would start to sweat in the first few minutes and then her energy just drain out of her. I noticed it was so much improved so of course I had to test it. I took her to the playground with the thermometer saying 97. She lasted 30 minutes and wasn't terribly hot, just ready to go. So strange...

As for energy, last night sealed the deal. We went to Noah's soccer game and it did not start until 7:30 and was not going to be over until 9:30 at least. Ayden Jane went over to the playground and for the first hour she played tag. Chasing, climbing ... She was playing with 2 girls from school and some other friends. These girls are high energy, athletic little girls who play travel soccer ect. I have never seen Ayden Jane keep up with these girls for more than a few minutes. She had a blast!! I'm willing to admit she was "it" a lot, but she did not care. I watched as she climbed and laughed and chased and smiled and just had a great time. So much fun.

Tonight we have an outdoor family movie night at church. They will be selling hot dogs, popcorn, candy and drinks. It does not start until 7 which is late for Ayden Jane to have supper. She asked if she could make a plan for the night. She suggested just having a cheese stick, milk, 'three spoons' and a little applesauce at supper time then splitting a hot dog and popcorn at the movie. Honestly, I was pretty impressed! She cut her typical supper down a bit chose small amounts at the movie. Not sure she could do better. Oh, and she also checked with her children's church leader and asked if they had water too, not just soda :)

Carnitine Craziness

So after several days of being back on Ayden Jane's dose of 500 mg of Carnitine in the morning I have had two observations. First of all, carntine is a very key supplement for her. Her energy has improved and she is back to not being concerned about food much. She just seems to have a consistent energy stream back.

So, of course my next question was should I increase the dose? Basically this thought was brought on by her having a reduction in energy and an increase in hunger/emotion after 4 pm. This late afternoon change is not necessarily new. I just accepted it as part of long school days or just a PWS thing. What I noticed was that with the low dose of CF, her whole day was more like her after 4 day.

I checked with Dr. Miller and at first she said she thought the dose of 500 mg was just perfect for her and I should leave it alone. Yeah... I listen, but that does not mean I don't plead my case. Dr. Miller finally gave me the go ahead to add a second dose of CF at lunchtime and see if it clears up the post 4 pm lull. I didn't ask her what dose because Ayden Jane swallows capsules beautifully but trying to hide CF in food is a pain so I just wanted to give her the full capsule. I also looked back and Ayden Jane was on a dose of 500 mg of CF in December of 2011 and I'm sure she is double that weight now!

So after a couple of days I am optimistic that I am on the right path. The biggest thing we noticed tonight was that Ayden Jane was talking super fast but totally clear. No stuttering or having to stop and think about what she was trying to spit out. She was totally happy and had no worries what so ever about waiting for supper even though it was a bit late. Her energy was also great - evidenced by her skipping all over the restaurant.

We will see what the next few days brings. Tomorrow may well be a long day, as we may hit the beach in the afternoon and horses in the evening.  

Carnitine Fiasco

For whatever reason, Ayden Jane needs a lot of carnitine and is very sensitive to the type and the dosage of it. You would think this would make me nearly paranoid with how I dose it daily. You would think...

Her current dose is 500 mg of carnitine fumarate (CF) and 250 of acetyl carnitine (ACL). So, she gets a full capsule of the CF which she just swallows and I mix half a capsule of the ACL in with her avocado in the morning.

I figured out this morning, however, that somewhere in the past few weeks I switched the location of the bottles. Of course I should read the labels every time I take out a capsule, but I honestly just got into the habit of going down the line. As a result, Ayden Jane has been getting half of the CF she needs and a double dose of the ACL.

Since the CF provides physical energy it could very well be the culprit for her some what lower energy level recently. Also, I'm thinking it may be the cause of her being more hungry lately due to that low energy. She has always mistaken tired for hungry. Also, the combination of even just a bit more tired and less active and more hungry could totally account for her added couple pounds.

ACL tends to work more on activating her brain. Ayden Jane has been a bit over emotional and more argumentative than usual but we chalked it up to it being summer and staying up late and no schedule.

So today, with the correct doses of carnitine in her? Crazy better. Maybe it was coincidence and she just happened to have a great day today, but wow did she have a great day today. It will be interesting to see how this all plays out but I'm thinking I may have my energetic, happy, lean little kid back in no time.

Her Number

Dealing with and discussing weight management with a 7 year old just seems wrong. We have taken to just having a scale out and Ayden Jane jumps on it from time to time. No schedules or charts or whatever... I get a kick out of how Ayden Jane calls her weight her 'number.'

She will ask me what a good number is and gets really excited when she gets a good number. The best part is that she is learning to make connections to things that make her number go up and down. She will hop on and the number is high and she will say, "Oh, I did eat a really big supper..." Or if her number went down she will tell me about how she skipped the extra snacks she didn't really need.

I am really hoping that her number will go down a bit and I can help her connect it to exercise. In the long run, eating wonderfully is only half of the equation and Ayden Jane needs to grasp the need for exercise. I'm am happy to help make the exercise piece fun, but she needs to understand that is not simply fun, it needs to be a way of life.