Monday, May 6, 2013

PWS Awareness Day #6: Hypotonia

Hypotonia, or low muscle tone, is a characteristic of PWS (and hundreds of other disorders), and there is a wide range of severity. Hypotonia is a neurological condition, involving the response of your nervous system in telling your muscles to contract or hold tension. This is what causes PWS babies to be so floppy, and although tone can improve, it doesn't usually normalize. 

Any part of your body that has a muscle (so, basically everything) can be affected by hypotonia. Legs have a harder time walking and running, the GI system has a harder time moving food through by peristalsis, the lips and tongue have a harder time forming speech sounds, joints are more prone to being dislocated because the natural tension in a normal-toned body is not there. Those are just a few effects of hypotonia.

People with hypotonia have to use sheer strength to overcome the looseness and weakness of their muscles and do what everyone else can do much more easily. Therefore, people with hypotonia often tire easily.

Dean has particularly low tone, even for someone with PWS. Fortunately, he is strong and so that has helped him overcome some of the effects of his very low tone. But anyone who has picked him up or watched him play know how floppy he is in his resting state.

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