When we were reviewing James' medical info before he ever even moved in with us, we saw that he has an unusual condition called Harlequin Syndrome. Not having heard of it before quite honestly intrigued by the effects it showed in him, we googled it and learned a little more about it. It is considered a rare disease affecting less than 200,000 people in the USA and is characterized by asymmetric flushing and sweating.
James will, on occasion and without cause that we can pinpoint, have half of his face turn red and blotchy, his same eye water, and there is a straight line directly down the center of his face where the other half of his face doesn't appear to have ANY effect. It is strange! :) It happened several times during James' first days with us and appears to be somewhat triggered by stress (when he was mad about a bath, for instance, he would have this happen) but... sometimes it would be completely random as well.
When we realized this was the real deal, we took note of the fact that the syndrome was associated with sweating and we kept on keeping on. We did pay more attention to his body regulation of heat and sweat, though... just in case. After all, we do live in Florida!
We took note of articles like this:
and this
It appeared that sweating was overcompensated on the "non affected" side, but we had yet to see that with James.
After almost a year with us and gearing up for our summer routines of playing outside, visiting the zoo and parks, and doing other "fun in the sun" activities that we looked forward to with our new brood of eight... We realized that this year might look a little different with James in our family. Two day trips to the zoo both ended with a lethargic little boy who threw up as soon as entering the air conditioning of the van. He then perked back up by the time we returned home (a 30 minute drive) and appeared fine the rest of the day.
We realized that James' sweating is definitely not taking care of his body's needs for regulating his body in the heat, and that we were going to need to make some adjustments to be able to do these fun activities and have him be comfortable and safe through them.
After doing more research online about anhidrosis, a condition which is characterized by the lack of sweat (and associated with Harlequin syndrome, but generally only on one side of the body), we saw there are two types of cooling vests available in the general market. One is an evaporation vest. You wet it, wear it, and the natural evaporation of water from the skin works similar to the body's sweat system. Unfortunately, this doesn't work in high humidity environments such as Florida because the rate of evaporation isn't so good...
The second type is one packed with ice packs. Although more bulky and generally much more expensive, we chose this option... with a little twist. Thanks to others within the Down syndrome online community, their creativity showed us options with creating a vest using a life vest rather than starting from scratch.
That brought us to Wal-Mart and we picked up a $20 life jacket with a zippered front and UV protection. After all, he'd always be wearing it outside... made sense!
We cut the inside lining and removed 2 of the 3 layers of floatation foam from each of the 4 sections of the vest.
We purchased soft sided ice packs to cool James.
Each of the ice packs slides in to the vest and sits just off the skin (he also wears a t-shirt under this vest!) and keeps him cool for several hours!
James has enjoyed summer activities right along with the rest of the kids and doesn't seem even a little bothered by the vest. He has stayed more active and alert during play (which is a good thing, though his activity level is SO much more than most of our kids :) ), and he hasn't had any other episodes of throwing up after a warm activity.
**This is not a FLOATATION device. it is a cooling vest. It just so happens that he's wearing it while in the kiddie pool because it was 95 degrees out and he was mostly sitting NEXT TO the kiddie pool.**
$25 sure beats the $200+ that we were finding the cooling vests for, and we are very glad to be able to continue enjoying fun in the sun this summer!
You guys are amazingly creative. That is SO cool!
ReplyDeleteWhat a great answer to this challenge! My daughter does not have the face flushing, but does have times when one side of her body is clammy, hot and sweaty, and the other side is cool. There is a distinct line down her tee shirts where one side is dry and the other side wringing wet!
ReplyDeleteDo you think you'll ever move to a cooler climate to help him, so he doesn't have to wear a vest like this for so much time every year? Poor little guy. The vest is a good idea for a temporary fix, but what about school? Will he have to wear something like this during every recess...? Is this a lifelong condition? Poor sweetheart.
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