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.