Hoop Dreams

OK, I got some of the leftovers off my plate. Drew up a press kit page for the handbells web site, revised a document regarding Joy's strengths and our ongoing concerns for her upcoming IEP, and (this is a big one) got the files sent to the printer for the book of my late mother's autobiographical writings that I've been working on editing.

Now I can write about the weekend.

Our big event this weekend was our church's annual winter retreat. Every February we gather at a retreat center by a state park about an hour's drive from here for a weekend of fellowship and a Sunday morning worship service. There are games, there are crafts, there are communally-prepared meals, there's a talent show, there's sledding and skiing and hiking. It's a wonderful time.

February 2008 (i.e. last year's) retreat was a disaster as far as Joy was concerned. She was still slumping from a big regression that had started that Christmas, which stole her language and affected her behavior. She was having several knock-down seizures a day, each of which took an hour of intense comforting to recover from. In retrospect, we think there may have been a virus involved too somewhere along the line. At any rate, we took her to retreat anyway, and she just got more and more miserable as we went. In between seizures she went tearing around the center fussing and leaving a trail of destruction. We were hoping that naptime would be at least a break, but instead of napping or playing quietly, she stood up in her tented pack-n-play and screamed bloodly murder. For ages, with no sign of stopping. Finally JoyDad took her home. I stayed at the retreat with Rose, exhausted and wrung out like a limp washrag. It was a hard time.

This year was a huge contrast.

Touch wood, y'all, but Joy hasn't had a knock-down seizure since mid-September. That was the first major thing. Second, she did not have the Christmas regression. Third, her boundaries have improved and she's more willing to hang out quietly on my lap while something else is going on. Fourth, her mood is happier on the whole.

With this background, I was willing to even take the girls to retreat solo on Saturday morning. (JoyDad came up later that afternoon; he had a memorial service to attend at noon.) Joy was contented throughout the slightly nerve-wracking trip as the snow was falling pretty hard. When we arrived, we had a friend all lined up to watch Joy while I unpacked the car. Joy hung nicely with me through the next event, which was a group game involving speed-singing of hymns and competitive reading of Bible verses all full of unpronounceable names. Much hilarity!

Since Joy has been skipping naps most days anyway, we didn't even try to put her into her travel tent. Instead, another friend volunteered to watch her for a while so I could go participate in a book discussion. Funny enough, the book discussion actually was sparked by this humble blog, from when I posted back in November about Tracy Kidder's book Mountains Beyond Mountains. A friend from church who keeps up with Elvis Sightings was intrigued by my recommendation and organized a group of people to read the book and discuss it at retreat! It was a fine conversation, with thoughtful articulate people, several of whom had been to Haiti themselves and/or work in medical professions (the book deals with a charismatic physician whose primary work is among the poor in Haiti). All in the context of beautiful snow falling gently outside the huge windows.

When I got back to Joy, her baby-sitter -- who has a grown son with a spectrum diagnosis in context of a different genetic condition than Joy's -- said they'd enjoyed their time together. "She's very smart, you know," she told me. Bless her for seeing it!

Our smart little girl kept it all together despite the lack of nap. She even surprised us at dinner time by eating her entire helping of macaroni with her spoon, with no prompting from us! She usually digs in with her fingers.

But when are we going to get to the hula hoops, you ask? Well, I'm glad you brought that up.

I mentioned earlier that the retreat features a talent show. This year, Rose decided that she wanted to do a hula hoop act. So she and JoyDad concocted a number wherein JoyDad played Wipe Out on his electric guitar. She has never taken a dance class, and I (not a dancer by any means) have been her only hula hoop teacher. However, she has such a sense of rhythm and has done a bit of group dance routine stuff in gym. And with those natural tools, she put together a wonderful piece where she shimmied and strutted back and forth with that hula hoop a-swirling, dropped to her knees and came back up repeatedly, and then let the hoop drop to her ankle and hopscotched it around with the other foot. All in perfect time to the screaming guitar.

The crowd went wild.

Video of this amazing act does exist, but we've got some technical hurdles before we get it onto YouTube. If you'd like me to e-mail you the link when we get it ready, say so in the comments or send me a message.

But that wasn't all. See, Rose decided to share her hula-hooping delight in another way. She brought all seven of her hoops along, and made them available in the main gathering room for the use of all the retreat participants. (I did promise that I'd replace any breakage.) It was amazing how the hoops resonated with all ages and shapes of people. From the short to the tall, from the curvy to the beanpole, from the toddlers to the highschoolers -- male and female both! -- to the highschoolers' parents, a huge range of folks got into the act. The height of the hilarity happened after the post-talent-show ice cream feast, when JoyDad plugged his guitar back in and played music to hoop by: more Wipe Out, Johnny B. Goode, one song after another after another. He played till he started to wear out the fingernail on his pick hand!

Well, some of the attendees were joking that hula hooping might lead to... DANCING! (verboten among the more conservative Mennonites, but this crowd is pretty far out on the progressive end of the Menno spectrum, as it were.) And after JoyDad unplugged, sure enough, out came a couple of iPods to plug into the sound system. Line dancing! Cotton Eye Joe! Jump Around! My fellow Mennos and I boogied till I think I strained every muscle in my body that hadn't already been hula-hooped out of whack.

Then I played Boggle till midnight. Which made 20 hours straight of wakefulness, seeing as how Rose had awakened me at 4 am Saturday morning to present me with one of her two front teeth which she had finally wiggled out.

Then Joy woke up at 2:30 and started commenting loudly... I'm afraid I don't remember much of the next morning's worship service, though they tell me I was physically present. It was quite the night.

I actually did make it to the regular evening worship on Sunday too, after everyone had headed home and sorted themselves out. Rose surprised me during the sharing time part of our service ("joys & concerns"). She stood up on the chairs and declared loudly that she had a joy. She wanted everyone to know she was so happy that people had so much fun with her hula hoops at retreat.

There. As JoyDad commented on my last post, this is probably the only blog on the Interwebz that combines hula hoops, dancing Mennonites, and screaming guitars!

Two Versions of a Tent-Camping Story

Each fall, our congregation reserves a couple of group campsites at a state park and we gather out in the woods for a loosely-planned weekend of fellowship and camping. This year was the first year since Joy's birth that we've actually dared to try & tent-camp overnight as a family. Herewith, the campout story.

The Diagnostic Version

We almost didn't go. The night before we were going to drive up to the park, Joy woke up crying THREE times. This never happens. She almost always sleeps through, and if she does wake, she usually stims and babbles herself back to sleep, rather than crying loudly. JoyDad reported that on one of the occasions, she was grabbing at her head. Plus, after the third waking, we gave her ibuprofen and then she finally fell asleep for the rest of the night.

We suspected ear infection. She's had quite a few, though it's been a while. It's very hard to tell, though, because she has an enormous pain tolerance and an out-of-kilter sensory system and doesn't have the language to tell us when something hurts. She had just the slightest edge of a fever. So I took her in to Urgent Care when they opened for the morning (of course this always happens on a weekend, right?)

Well, Joy's ears were clear. And her throat looked fine. And her mood was improving by the minute. So when I got her home from the check, we packed up and went.

It turned out to be a lovely time. We set up Joy's PeaPod travel bed inside the tent, where she took a nice afternoon rest and then slept the night without complaint. She didn't really do any dashing at all, nor did she try to play with the campfire. Instead, she ran around within the confines of the camp area, but deliberately kept looking back to interact with whomever was tailing her. She also managed to come on a bit of a hike with me and a couple of friends. And she ate well, and smiled a lot, and generally did great.

Oh, and no seizures, though our party-count-clock has been set back with a night-time seizure last week. (Which shouldn't count. But I think it does.)

So why did the campout go so well?

1) Maybe she's settling in to a recent epilepsy-meds dosage increase.

2) Maybe having had a whole summer to experience a backyard fence has helped reduce her urge to dash.

3) Maybe the intensive autism therapy is starting to get her into an increasingly interactive mode, which we'd love to credit for some of the wanting to be with people.

4) Maybe we just hit it lucky with a good-mood weekend.

And maybe I'm wayyy over-analyzing this and should learn to live in the moment! So, here goes again:

The In-The-Moment Version

We were so happy to be able to go camping with our friends from church, after briefly thinking that we wouldn't be able to go.

It turned out to be a lovely time. We set up Joy's PeaPod travel bed inside the tent, where she took a nice afternoon rest and then slept the night without complaint. She didn't really do any dashing at all, nor did she try to play with the campfire. Instead, she ran around within the confines of the camp area, but deliberately kept looking back to interact with whomever was tailing her. She also managed to come on a bit of a hike with me and a couple of friends. And she ate well, and smiled a lot, and generally did great.

Oh, and no seizures!

And I got to play Boggle with friends (and with Rose) by lantern-light after Joy went to bed.

What a blessing and a Joy.

Linear Nevus Sebaceous Syndrome, or, another order of noticing things

Update! I'm pleased to announce a new resource for linear nevus sebaceous syndrome, called LNSS Connections. The site introduces linear nevus sebaceous syndrome & related neurocutaneous syndromes, and links to a new support group for people dealing with LNSS. Please come check it out!

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There's another layer to what we noticed when.

Joy was born with a visible sign of things to come, a blotch on her scalp -- that's a newborn photo at right.

On the first day, it was written off as maybe a scrape. On the second day, the word was linear nevus sebaceous. It was, we learned, a fairly common blip, a blotch of inappropriate dermal tissue packed full of oily glands -- hence the "sebaceous" -- on which no hair would grow.

JoyDad went online and found some rather scary pages about linear nevus sebaceous syndrome, accounts of kids born with nevi all over their faces who ended up with various combinations of other symptoms involved: blind, deaf, serious cognitive impairments, severe epilepsy. However, I don't remember him making a big deal of those findings to me, because the message we got from the doctors was that most such nevi have no further implications, beyond the cosmetic issue and a somewhat elevated chance of pre-cancerous changes further on down the road. The nevus wasn't even expected to grow, beyond just keeping up with her head. (Nine-months photo above left; the blotch was peach-colored most of the time, turning bright pink when the rest of her face went red, as in a crying jag or the like.)

We consulted with a pediatric plastic surgeon, who ended up doing three outpatient surgeries over the course of a year, the first when Joy had just turned one. No complications from the surgeries, other than a scar that her hair grew to cover, with a promise of potential scar reduction surgery some years later.

The seizures started between the second and third surgeries, at age 15 months, summer of '05. They deserve a post on their own, so I won't go into a lot of detail except that we have an excellent pediatric neurologist, who even happens to be a fine Boggle player! He first floated the idea that the epilepsy and nevus might be related in summer of '06, but in such a low-key way that we didn't really glom onto it and press him for more details and a definitive diagnosis of linear nevus sebaceous syndrome (LNSS) until November of that year.

LNSS is a rare congenital disorder that was first identified in 1957 and then independently in 1962, and is also known as Feuerstein-Mims-Schimmelpenning Syndrome. My understanding is that it's rare to the point that the number of cases in the US numbers in the 100s. It's a genetic disorder but a mosaic one, so it's only happening in the affected areas rather than systemwide and there's no blood test for it. The classic triad of symptoms are the nevus (check), epilepsy (check), and cognitive issues (check). It can also, however, include a whole slew of other systems such as eyes, ears, skeletal, and genito-urinary, and sometimes a diagnosis will be made if one of the triad is missing but some of the others are present.

We are fortunate that Joy seems to have the classic triad, and nothing else. She's seen a geneticist, had her eyes checked (one of the most stressful doctor visits we've ever had, but that's another story), and had her hearing checked (inconclusive because she has the attention span of a gnat, but we don't have any evidence for being concerned).

For Joy, LNSS makes sense as an umbrella diagnosis that more or less explains all the rest, including the autism. However, it doesn't give us much guidance as to what to DO about any of it...

I'd link to a good summary site, except I haven't found one that I liked recently! There isn't even a Wikipedia entry, for cryin' out loud. I haven't found an online support group specific to LNSS either, though there is an Epidermal Nevus YahooGroup that gets sporadic traffic, where a variety of kinds of nevi are included and only a few of the participants are dealing with the syndrome beyond just the nevus. Maybe there's a mission for me in that lack of information, at some point.

Next year's new words

Merriam-Webster announced yesterday that they've added over 100 words to the their New Collegiate Dictionary.

Among the new words: edamame, fanboy, netroots, dwarf planet.

I think, however, that they're still perhaps missing a few.

The other night I was playing Boggle with friends, and the word "stim" made its way onto my list. Here's how it's defined in the Double-Tongued Dictionary:

stim v. to self-stimulate; (specifically) among autistic people, to fixate on a comforting or compelling thing or action (such as rocking or humming); to perseverate. Also n., a (self-)stimulating thing or behavior.

Joy's got a lot of stimming going on this summer. When let loose on a lawn, she immediately falls to pulling out clumps of grass and running it between her fingers. At the park, the gravel or the chunks of old tires are much more interesting than the play equipment.

But stim isn't in the mainstream dictionaries yet. So I couldn't claim a point for it, even though another player with connections to the autism community backed me up on the definition.

Wonder what year it'll be before the list of new words added will include stim? Or perhaps neurodiversity?

Meanwhile, on the next round, the player who knew "stim" and I both came up with the word "celiac." And cancelled one another out, because you only get to count points for words that nobody else finds!