Sunday, November 8, 2015


Sometimes I get questions about whether Ayden Jane struggles with _________. You can fill in the blank with one of the many 'joys' brought to us by Prader Willi Syndrome. I'll work my way through a few of the typical ways in which the syndrome affects families. I say families because it is not just the person with PWS that is affected.

First up is food. That's the first one most people ask me about because at age 8, Ayden Jane should be feeling some of the effects of  'the hunger.' Honestly, I don't believe she deals really at all with hyperphasia. That is, I don't believe she is constantly hungry. Her day is not consumed with thinking about her next time to eat ect. I will say that doesn't really feel full like most of us do, so if not taught to understand a portion ect. she would easily over eat. It's an interesting balance and one where we just make food about nutrition. Being sure your body gets what it needs in the right amount to keep energy levels good but not extra because then it just turns into added weight which will slow her down.

Next is anxiety. I'd say this one gets us all. Her anxiety is not so high that it is visible all the time. It more just lurks under the surface and you just never know exactly what is going to cause it to break through. Today is a good example. She was at children's church and needed to use the bathroom. Someone cleaned up her spot and she couldn't find a paper that was apparently important to her. She burst into tears as she walked out. We stepped into an empty room for a minute and I told her to get it together and then she could go back and see if she could look more, or solve the problem with one of the leaders. Another classic time to see it is when she is pressed for time on something. Part of the point of hurrying a child is attempting to get them to have a sense of urgency so they will get with the program. Well, with Ayden Jane, if you hurry her that sense of urgency just turns to a panic that is similar to the book, If You Give a Pig a Pancake. Only in Ayden Jane's case, she always goes through a string of events that ultimately lead to the destruction of the universe.

Socially. Ayden Jane has come a long way on this one. When younger, she struggled mostly because her little brain just couldn't process things fast enough to keep up with the other kids. If you listen, you will realize that exchanges between small children are actually very quick and short. Sort of the polar opposite of the types of conversational exchanges Ayden Jane made when little. On the plus side, she has not really been excluded, just not really included. You know, no body moans because Ayden Jane is in there group, or they don't go out of their way to avoid her. Just that when someone is looking for a friend to do something with, seldomly do they invite Ayden Jane to be that friend. More in an oversight way than anything else. This is an area that Mom could do better and invite friends to come do fun things with us. For now, we are good because she has found a couple neighborhood friends where the friendship is very mutual. I think in the end, socially, Ayden Jane can learn social cues and rules to games and give and take with other kids but sometimes she needs a bit of help to sort it all out.

Behavior. Often times children with PWS have a lot of challenges with behavior. Whether it is the rigidity that makes it so hard to handle a change that comes up during the day or an underlying tiredness that many feel or something in the energy balance of food they have eaten or just the emotional instability that can come with the disorder... take your pick. I would say that Ayden Jane does feel many of these things to a mild degree but is cognitively sharp enough to talk herself through. For example when her schedule is disrupted at school for some reason, she just needs someone to give her the new schedule and she can see how it's going to work. Or if snack is missed in the morning she can plan for herself when a good time to have it will be. We are very thankful that by and large all we hear from school or other places Ayden Jane goes is how well behaved she is.

Attention. Kids with PWS often struggle with attention. In Ayden Jane it is most evident in large groups. She simply cannot screen out all the 'extra' stuff so that she can concentrate on what she is supposed to be listening to. This makes instruction a challenge in whole group at school sometimes. Many kids with PWS end up needing some form of medication to help them focus well enough to keep up with their peers.

Motor Planning/processing speed. Yep. This one hits us. Learning to get her body to do something takes extra work. Ayden Jane cannot easily watch you move a certain way and repeat it. When she tries to climb or do something for the first time it is super slow. With repetition and cuing certain muscles she can learn to do most anything but nothing comes easily and each thing needs to be taught. Additionally, when trying to play sports like soccer, the amount of time it takes for the thought to reach her feet and make her feet move is just too long.

Temperature regulation. This is a weird one. I'd say it doesn't bother her a ton, but when she gets too hot - it's all she wrote. The odd thing is how quickly she overheats when it is cold outside and she is wearing a coat. Something about keeping that heat in throws her thermostat way off.

Decreased pain sensitivity and body awareness. We choose to spin this in our favor :) Ayden Jane will try things without fear and when she falls she can brush it off like no other. The body awareness thing just means our toes are often stepped on, doorways often run into, things spilled and, well you know the term bull in a China shop.

All of these things just add up to a life that is a bit harder to manage. Learning and playing are just a bit harder for Ayden Jane than other kids. Handling disappointment is harder. Excelling is harder. Living with her is harder. She takes a more time to do everything...., our days need to be a little bit more planned, her needs are just a bit more urgent. Basically, as her family, we just always feel that heightened need for vigilance.

I can say, though, that with the added concern and effort comes an amazing joy. Every accomplishment feels like winning a championship. Being a part of a kid who lives life with every ounce of her being is contagious. Yep. she rocks. She is happy. She loves life and is thrilled with all she CAN do and is pretty sure she can do anything if she works hard enough. God has placed such a sense of worth inside her that a confidence just pours forth. She is a walking billboard that says, I am a child of God and He has made me exactly as He wanted - perfect, PWS and all.

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