May Questions

PWS question: When and how did you tell Ayden Jane that she has Prader Willi Syndrome? What did you tell her?

First of all, we wanted Ayden Jane to know how awesome she is and have a strong confidence in God and her own unique gifts. We want(ed) PWS to be that extra thing she deals with, but not who she is. (some days I'm pretty sure we over did the whole confidence thing but...)

We used the words Prader Wili Syndrome in our home as we talked about research and doctors and fund raising.... I sort of thought AJ would put two and two together at some point.

With Ayden Jane we used age appropriate phrases like 'your body doesn't work well with sugar' and 'we see Mrs. Jen to make sure you grow strong and straight'. She was learning about how Prader Willi Syndrome effected her, just without the label.

I'd say it was within the past year that there were enough questions about why she has a shot every night, why does she have PT, OT and horses, why doesn't her body work well with sugar, why does she have to take supplements, why didn't she talk with her mouth and only talked with her hands when she was little, why ....

At some point she asked directly why her body does things differently so I simply answered, "Prader Willi Syndrome makes your body that way." She practiced saying it but didn't really want a lot more info at that moment. Over r the following weeks she would randomly ask things like, "do I get my shot because I have PWS?" 

Eventually on one of our long dog walks. Otherwise known as uninterrupted talking time, Ayden Jane really delved into it all and her understanding was astounding. She had taken in all the little snippets from conversations and pieced them together.

I can honestly say, Ayden Jane knows she has PWS, but she does not fear it. She just accepts that it is a part of who she is and how she was made. She is a bit curious why God made her with PWS but is thankful for all the things and people who help her achieve so much. She knows she has PWS but is absolutely positive that it offers no challenge that she cannot handle. 

So, short answer? We told her when she asked at around 5-6 years old. We told her a little at a time, as she wanted to know. We told her the truth. No big deal, just that PWS is the name for why you eat the way you do, why you have a shot every night...

May Q and A... School

Whew. So much for answering a question a day! It is still May though and therefore PWS awareness month, so I will continue to answer questions about PWS.

Again, a summary of several questions: How is school going? Does Ayden Jane have extra help with academics? How does she handle food choices?

Ayden Jane has had a fabulous year! She was prepared for Kindergarten because of the awesome, caring staff at her preschool. As she transitioned into a new school for K I was a little concerned because it is a good sized school with lots of staff, all who most likely would know nothing of PWS. In the days prior to the start I made the rounds. I chatted with her teacher, the school nurse, the principal, the lunch room staff ... I have revisited with people as new things have come up but I can honestly say that they all listened and followed through and that I feel that Ayden Jane is totally safe at school. That may sound strange, but when things like food seeking and/or restrictions, eloping, immune issues, diminished pain response... are involved, safety can be a concern. One year under our belt and zero issues. Whew.

Food wise, we pack lunch, send her own snack daily and supply the teacher with treats for when birthdays or special occasions come along.

As for academics, Ayden Jane is not typical for PWS. She is in a regular class all day with the exception of receiving OT once a week. She does not exhibit the behavioral issues common to PWS (in school anyway) and is not cognitively delayed. There are quirks in her processing and she talks her way through her work a lot (I mean out loud, narrating as she goes .... bless her teachers). She loves routine and structure so school is a great place. She just brought home scores for reading and places at the 67th percentile on a nationally ranked test. I'll take that!

There is one struggle. Writing. I think I'll tackle that tomorrow...

PWS Q and A: Continued from yesterday, how does she do in school?

Part two is the struggle. Writing. It is extremely common for handwriting and the actual organization of thoughts on paper to be a big challenge for kids with PWS. For the physical piece of writing, Ayden Jane has been in occupational therapy (OT) with an awesome OT for a couple years working on hand strength and all the coordination of making a pencil go where you want it. (not to mention cutting, tying shoes, doing buttons) As she started Kindergarten she could hold the pencil and trace things, but she could not make letters. Even looking at them a copying them was not possible. Without a guide to follow her brain just could not plan what was in her head and make it come out of her hands.

Now? She writes them all. Upper and Lower case. She can spell simple words. It is hard. It is exhausting or her. It is slow. Imagine when you write a word having to work very hard at correctly forming each letter, remembering how to make it. Then trying to keep track of where you are in the word to spell it.... I am NOT looking forward to 1st grade homework each night...

Here is another really odd twist. As she became able to make letters and even spell a few words, she would attempt a sentence. All that came out her hands was a string of lines. Not even letters! She would argue loud and strong that it said _______. So strange. Her brain was/is just so much faster than her hands. Now she writes a sentence as a string of letters. No spaces but you can find some words if you look hard.

In the end I believe (hope) it is connected to the moderate speech apraxia she had when little. She understood receptively everything but she did not talk. (expressive) She has made big gains in reading recently and I am hoping that much of the writing piece is partly just lagging like speech did. All the skills are in there, she just can't figure out how to access them yet. 

Ayden Jane loves school. The slow processing seems to be 'less slow'. Her attention span seems to be increasing. As with everything else she works hard. If a time comes when she needs academic support I won't hesitate to find that for her but at this time, overall, she seems to be continuing to gain ground with her peers. She is just one of the gang (well, the quirky one in the gang anyway)

More May Q and A

Okay, so I have had this question from a couple people in a couple forms and I admit .... I have been avoiding it. What will Ayden Jane's life be like as an adult? Will she be able to self monitor her eating or always need assistance? Will she live independently? Get married? Have a family?

Short answer is no one can say for sure. That answer is filled both with hope and sadness.

As it stands right now, (knowing there are always exceptions to every generalization) adults with PWS require assistance. Many live in group homes with locked kitchens and require a level of supervision 24/7. Some live at home however, they too require a great deal of support and a degree of food security. I don't know that I have heard of any with drivers licences. Whether it is because they would not be able to resist the fast food joints or because the level of executive functioning or processing speed would prevent it I'm not sure.

My thoughts for Ayden Jane and the others her age and younger? There is tremendous reason to hope! Starting gh within the first year of life has made huge improvements for many in cognitive development bringing it close to average for many. It has improved body composition thus allowing for improved metabolism and a more typical amount of calories per day. Research is offering dietary and other avenues to improve some of the symptoms of PWS. Early diagnosis leads to starting these and key therapies early has lessened developmental delays. I could go on...

Okay, so speaking for Ayden Jane, knowing that for whatever reason - genetics, luck or just stumbling along the perfect diet, therapies... God has placed Ayden Jane at the mild end of the spectrum. I work daily to prepare Ayden Jane with the skills she will need to live independently. I believe she will be able to go to college and manage a level of independence. Now will she go to Clemson and become a zoo keeper like she plans? We shall see.

Research is ongoing and some success is being made figuring out what is causing the unrelenting hunger that drives those with PWS to eat and consumes their thoughts making it hard to focus on much of anything else. There are drugs currently in clinical trials that are having success! Whether or not this drug is IT I don't know, but one close to it could be.

So, daily we feed Ayden Jane in such a way that her brain and body develop in the best way possible. We do OT, PT and hippotherapy weekly. We make exercise a part of every day. We are proactive in all areas we know may be affected by PWS. We are keenly aware that despite all this AJ may not be able to live a typical adult life but we are hopeful that by the time she gets to that stage, the current roadblocks will be removed!

I guess, Ayden Jane has taught me not to accept the word impossible or even limited. We will continue to enjoy being totally average, work toward being exceptional and thankful that she is healthy and happy no matter what.

PWS Q and A (continued)

Yes, I have missed a day (or two) but I have not forgotten those of you who have sent questions. Still room for more and I have to say I really enjoy answering. I can get pretty boring and technical when I talk about PWS and I worry I make your eyes glaze over. But I love that ya'll love and care about AJ and our family enough to want to know. 

Question was how has Ayden Jane's 'way of eating' affected the eating habits or even what is available for the rest of the family?

I still remember sitting them all down and letting them know, at least to an extent that food was now a big issue. That food was never to be left out. That drinks lying around were potential danger. That free eating on the sofa .. can you say chips anyone? was a thing of the past. I guess you would say I 'laid down the law.' Not welcome words to growing teens!

To be perfectly honest, I have never liked to cook. I am not a food person. I could literally eat the same boring thing every day and be perfectly content. Suddenly, after 15 years of feeding kids one way and settling into the meals we liked and I was tolerant of cooking, I had to learn to cook all over again! Arrrgghh.

Next, as I learned about nutrition and we all saw first hand the power of food to help Ayden Jane and harm her, we all realized that what you put in your body really is important. I think my kids know (head knowledge wise anyway) a lot more about nutrition than the average kiddo. As for their actual eating habits? I believe they eat less junk but I have not done a good job of helping them find new healthy options. You know, ways to cook veggies that make them more appetizing... 

I definitely 'shop the outside of the store' more now. You know, fresh food instead of boxed or processed found in the isles. Meals tend to be lean on the bread, potatoes or rice. The house has lots of nuts, fruit, veggies, avocados and things made out of coconut around. All of them now like dark chocolate (since that is often the only kind around).

I would say they all know how to eat like Ayden Jane and know that it is the healthiest way to eat. I would say they are more conscious of quantity when they eat. They are not at a stage in life where it is necessary to put their knowledge into practice, but maybe one day it will come in handy when they get past the carefree, eat all you want because you are young and active and burn off all calories age.

PWS awareness month day 8. Question I'm answering today is one I have heard before. Ayden Jane is doing so great, does that mean she is growing out of Prader Will Syndrome?

Sadly, no. It doesn't work that way. PWS is a genetic syndrome which means that AJ is missing some genetic material in every cell in her body. This missing piece is responsible for a lot. At this point, there is no way to 'put back' or 'turn on genes' to correct this. (although the some research is working on a form of that)

These missing genetics cause lots of different symptoms particularly with endocrinology and energy balance and neurology.

Ayden Jane has a team of doctors to help us manage these things that her body does not know how to control well by itself. When we have her balance at a good point, her brain fires well and she blends right in as a typical kiddo. Without the medical support her brain and body cannot keep up as they do not internally monitor and adjust.

So, PWS will affect Ayden Jane in slightly different ways as she gets older but it will always be a part of her.

PWS Q and A

PWS question of the day  How did/do you balance the needs of all four kids when so much time goes into Ayden Jane?

Honestly, at first there was no balance. It was all AJ all day and I missed my big kids. I loved my little bundle but at times I just wanted to forget PWS. Of course that would mean I'd have to forget her... It was a struggle.

It was rough on them at first because I cared for Ayden Jane and Gary worked more because he had to. So in a way they lost a lot of time with both of us. Friends and family pitched in and took them places and made sure they were able to attend soccer tournaments and volleyball matches. They took care of each other and were amazing.

They loved their little sister and each contributed in their own way which helped them and us. Kayla was second mom. She learned all she could about PWS and how to care for Ayden Jane. She could even do a feeding tube if the need arose. Noah loved AJ, but when she was tiny he helped more by asking me what I needed. It was truly such a relief to know that I could count on him and that he was looking out for me. Mckenna was magic when Ayden Jane was a bit older and began therapy. She could get her to work so hard, so long and not even know it was work.

So, I guess as we transitioned to emergency mode at first, I did not really balance things but others took care of that. As the long term 'marathon' set in, we divided some of the AJ work naturally using everyone's talents. I think being a part of the mission of caring for AJ and each other was great for everyone. Eventually we just slipped into our new normal and did our best to meet everyone's needs. Hopefully there was some balance in it:)

A couple of questions combined - Do you follow a specific diet with Ayden Jane? How do you decide what and how much she can eat? Dark chocolate is permitted as a treat, what makes it ok?

Ayden Jane does not follow a specific named diet. I would say it is closest to a modified atkins or a paleo type diet. As Ayden Jane has grown we have tried things and her response in energy and behavior has helped us hone in on the foods that work best for her body and the best balance for her.

When we first started feeding Ayden Jane she was not yet diagnosed with PWS. We started with infant cereals in the classic american way. She packed on lots of weight, which was almost a relief at that point, but she did not grow. She was was not lively. 2 months later she was diagnosed because the growth curves crossed and she was more 'PWS like'.

I sort of went out on my own by reading and researching and I noticed the link between cereals and AJ. I threw them all out and gave her avocado and eggs and almond butter and cottage cheese and .... She leaned out because she grew. She became more alert and active. Her personality burst forth and within 2 months, even before we got gh started, she was back to her proper ht,/wt. percentiles. Her developmental milestones did not seem so far away. From 8 months at diagnosis she was not able to sit up independently. With her new fuel, 2 months later she was sitting and crawling and into everything. By 12 months she was standing. The rest of her developmental milestone came within typical range or close.

Basically, we avoid all grains (as they are mostly carbs) and sugar. I did not know that this was radical. I just knew that her body was obviously not using them properly. I learned a lot about our need for dietary fat and in particular the need for young children with growing neurological systems need for lots the need for young children with growing neurological systems need for lots of good dietary fat. She responded to these foods very well. By the time I saw Dr. Miller and other PWS specialists Ayden Jane was 2 and they were amazed with how typical she was. They did an analysis of her diet and said that it was unique but that it covered all her nutritional needs and was allowing her to eat a lot more calories than expected for PWS kids and actually more than a lot of typical kids. In fact, her weight was so low they said that if I could manage to get her to eat a few more calories that would be great!
We just stuck with the same formula. The how much is tricky. When she was tiny, I felt like all I did was feed her and try to get enough calories in her. Now that she is older, I work to make portions small because she will over eat since she does not really have a natural off switch.
As it come to treats, dark chocolate is one we introduced early. Basically dark chocolate (70+ cocao) is higher in fat and lower in sugar. She gets just a small square but is content with just that while everyone else eats a piece of cake ect. She does not seem negatively impacted by these so we just go with it.

A Day in the Life

Ayden Jane is on a bit of a roller coaster these days.  Not sure what is causing it, or if it is temporary or permanent or ....

Here is a full circle day in the life...  Last night she wet the bed.  Not a big deal, she is not that old for that and in PWS it is common for it to be later.  Darn endocrinology.  She had to shower.  OCD means she had to wash every part and was up a good while.  Then she had to have certain pj's and pushed back and argued with Gary enough that it ended in a tantrum and tears.  Back to sleep eventually.

Woke up early as usual and chipper as the day is long.  Played happily with her toys in the living room, so Mckenna could sleep in (after breakfast of course).  She was all excited to go to Walmart with me and help with the grocery shopping.  She wrote a list to bring and was hysterical along the way.  We got there and all was well.  She was as happy as a clam and very helpful.  Then it started.  ________ is on my list (nope, but we got it anyway.)  Again and again until we said, "no."  We are not getting those.  It was a simple request, but it represented the line in the sand.

She couldn't deal with it and I took her out front.  Gary was with us so he finished and checked out.  As she sat on the bench and we talked through it she cried.  Not a tantrum angry, frustrated, spoiled kid cry.  A heart broken, quiet cry.  I told her, "the answer is 'no' and you have to accept that.  Part of being a big kid is learning that sometimes the answer is 'no' and you have to deal with that or you will not be able to go and do things.  You have to be able to let it go and move on.'  She said, "I understand what you are saying in my head but it still hurts my heart.  I want to 'move on' but I can't get it out of my head.  I'm stuck."

There it is.  PWS.  Stuck.

She did move on.  Finally.  I asked her about Mother's Day and what we should get Grandma.  I had tried telling her she needed to calm down so we could get her Popsicles for school, but that wasn't the motivation she needed.  Nothing for her would unstick her.  The giant hurt heart could only move on by doing something nice for Grandma.

We came home and had a nice afternoon.  Then Kayla took her to a friends house that she was babysitting.  The word 'no' came up on a food issue.  Kayla called and I had to talk Ayden Jane through it.  Again, if you can't handle 'no' than you can't go.

She tries so hard.  She wants to make the right choice.  She wants to listen.  She wants to be free of getting stuck.  I know that she will manage.  It will be harder for her than everyone else but she will do all she can to conquer this and she will succeed.  For right now though, it just sucks to watch her go through it and I hate the effect it has on everyone else.  They are all tired of fighting with her...

Dog Walking Makes Good Conversation

Ayden Jane and I took our usual long walk with the dogs this evening. Topic of conversation? Prader Willi Syndrome.

Her questions revolved around sugar and how she doesn't eat it and what happens if she does.... the usual. Then she branched off and asked if all kids with PWS ate the way she does. She wanted to know how kids who don't eat the way she does are different than her. Are they fine? Do they feel awful all the time? Can they do all the stuff she can? 

I was very honest with her.

She wanted to know why it was hard for some kids not to eat sugar. She said, I don't know why everybody thinks it's so amazing that I don't eat cookies or candy. I asked her if it would be hard for her to stop eating bananas and grapes. Oh the panic... I used it to show her how it is just easier not to eat something then to learn to love it, and have to give it up.

She does not know the other side of PWS. She only has two friends that she knows well with Prader Willi Syndrome.  She talks about them a lot, but they are younger and she see no difference in them compared to other kids their age.

She wanted to know why I know so much about PWS and why Dr. Miller wants to know how sheis doing even when she is doing good.(It was a long walk...)

I told her about kids who struggle and the ways they do. I told her about a friends son who doesn't struggle and how awesome he is doing. I told her she is special because she is one of the oldest kids to eat the way she does, take the supplements and work really hard from the start and it is really helping her. For the first time, I told her that her first doctors did not think she would be able to do so much. They told us that she would not really be able to keep up with her friends. That she wouldn't be able to do things like ride a bike or surf because she wouldn't have the balance.... She asked, "what's wrong with them? I can do anything!"

We talked about how most peoples bodies tell them they are hungry when their body needs fuel but also tells them they are NOT hungry when they do not need more fuel. But that PWS bodies can't do this well. She said, "I know. Sometimes my brain tells me, remember you just ate, but my belly tells me it wants more. I just tell my belly to stop tricking me and I go play."

Her understanding was unbelievable and she smiled and jumped up and down and said, "I really do feel special. I am going to do everything even if it takes extra work because I know we can find a way to make anything happen."