Thursday, July 30, 2015

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!!

Sunday, July 26, 2015

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 :)

Monday, July 20, 2015

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.  

Wednesday, July 15, 2015

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.

Sunday, July 12, 2015

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.

Tuesday, July 7, 2015

Exercise - part 2

Ayden Jane has taught me that life with PWS is the perfect model of the phrase "use it or lose it."

We have gone on our walk 4 days in a row now. The first day I was surprised to see Ayden Jane break out in a sweat early on and be very sleepy in the early afternoon. Day 2 was slightly better, but she still was tired... I will say that both days I loved her ability to sit and focus after the walk!

Day 3 she started out saying it was our day to run. Remember we had worked up to running a good solid consecutive 20 minutes. After a minute or two she was exasperated and declared running was not a good plan. We talked about it and she accepted that after being sick and the time off we needed to just work up to running again.

Plan in place is that we will do the full 1.6 miles every morning. That we will walk one day, run the the next day....  The running we did for the first run day was 5 minutes. She wanted to go home straight away after but I re explained the deal. We go 1 1/2 miles every day, just the amount of it we run will vary. So far she has totally bought in so tomorrow is run day and we will up the number of minutes we run. It's all an adventure to keep exercise interesting and Ayden Jane motivated. It's not hurting me an either.

The other part of the plan is that she is to go 10,000 steps a day on her fitbit. With the help of our early morning exercise, she generally has about 5000 steps by 9 am. The fact that part of most days is spent in water where she gets no reading keeps this goal challenging.

Next on her list of motivators is that she has her scoliosis x-rays coming up August 6. Jen (PT) has told her that if her x-rays look great and her right leg looks great from all the walking/running she will either let her have much smaller inserts in her shoes or a trial run of NO orthotics.  Ayden Jane is over the moon excited about this possibility. She also knows part of the deal will be that if she gets the go ahead on no orthotics then she will have to keep walking/running to keep things strong. Mrs. Jen will be checking up on her!

Finally, she is going to run the inflatable 5k in Rock Hill SC in October with Dr. Miller and her daughter A. It should be a blast!! One lucky little kid we have that so many people are in her corner.

Monday, July 6, 2015


A lot of the challenges of PWS are related to food. Limits on what she can eat and to some extent, how much. Remembering to drink liquids, not using artificial sweeteners or sugar, being mindful when away from home.

This is only one variable in the equation however. Exercise has never really been a problem for Ayden Jane. She and I did lots of things together that often included walking dogs, riding bikes to the playground, taking a walk on the beach, even heading to Walmart and wandering the store can be turned into extra steps and exercise.

This summer, we started with being sick. Then traveled a bit and now she would just rather hang out with her friends. "Playing" seemed like exercise to Ayden Jane so getting her out to do something else was getting to be a problem. I thought through it, emailed Dr. Miller to see if I was crazy and decided it was time to explain to Ayden Jane that playing and exercise are different things. Both are good, but they are not equal. It used to be that exercise was a given so adjusting food was all that was needed for weight maintenance. She has been getting frustrated that she has been good about food and her 'number' is not where she wants it.

I told Ayden Jane that because of PWS she needs to exercise everyday. That just playing around in the pool, swinging on the swings with her friends, painting and coloring, even catching bugs is not the exercise she needs.

She was upset at first but soon into the conversation I realized her concern was less about the exercise and more about making sure she was not missing out on anything. So we just figured out how she can have it all. Basically, we backed up breakfast a little and we are walking/running 1 1/2 miles every morning. She knows there is no heading to neighbors before 9:00 and no neighbor kids allowed at our door before then so as long as we are sure to be done by then Ayden Jane is in.

Today was day 3 and Ayden Jane is getting more and more happy with the whole plan. Whew. The timing was good because her replacement fitbit just came and she loves that she starts her day with nearly 5000 steps. I can say she has lost a LOT of stamina over the past month. I'm excited that her goal is to get back to the point where she can run the full mile and a half. Sounds like a perfect goal to me.

Friday, July 3, 2015

Summer Fun

I have umpteen posts in my head... Just going to start with one and do my best to get back to writing regularly.

Ayden Jane has had such an amazing burst of social skills. I really don't know what has brought it on, but I am guessing it has been a magical combination of MCT, growing up and neighborhood friends.

I'll start with the MCT oil. It is interesting stuff and I honestly don't understand it. (which always makes me a little nervous). There is no denying that it really keeps her brain going though. With it, she just processes pretty much everything faster. What does that mean? It means the fast paced conversations of kids, the rules to games she wants to join, the ebb and flow of the day are understood and followed quickly enough that she can keep up and not frustrate others.

I do think that some of her skills are just the result of getting a bit older. She was a little delayed with speech and it seems like the slower processing and challenges getting her words out put her behind in this area. She has been gaining ground for a good while and now she is partially just catching up finally.

Neighborhood friends have been a huge help as well. Ayden Jane has had friends but none she can just run out the door and find and play with. Growing up in a world of therapy, older siblings, doctors and lots of medical talk does not exactly give you good peer relation skills. Being that I am an old mom, most of my friends don't have kids Ayden Jane's age and I just haven't made myself find others to spend time with. I feel bad sometimes as I think back to the families we just did life with when the big kids were little and how much time they spent with great families of friends... The neighbors have a daughter AJ's age and a son 2 years younger and the three of them have become quite the trio. The parents are wonderful as they are not worried or bothered by Ayden Jane's extra needs and they have taught their children to be the same way.

So, how do I know there are new social skills pouring forth? That it's not just comfort with neighbor kids? I see it in new situations. I see it at the beach when just AJ and I are there and she meets up with new kids or even just kids she only slightly knows. She catches on much more quickly to games and is relaxed and happy, and the other kids are inviting her to join in. I see it on the sidelines of the soccer games as she joins in and kicks a soccer ball with a new friend or I watch another friend run by and say, "tag, your it" and Ayden Jane immediately respond by taking off running after her.

She's relaxed and 'on the same page' with the other kids. She is not trying to analyze things or asking a million questions on the rules of the game. Even just the body language of the interactions is that of mutually enjoying playing or chatting together.

It feels strange that Ayden Jane didn't always have adequate social skills. It almost seems strange to remember that she has spent most of her years a step behind the action. I am excited to see how these skills play out in school next fall with a new classroom of students.