lennox gasteaux

I just got off the phone with Eva. She is in the hospital with Kai again. Over the past day or two they were required to increase his dosage of medicine (it's part of the schedule to increase it over time) and his condition got much worse. He was vomiting, not eating/drinking and lethargic all of the time.

They are now switching him to a new medicine, but it is normally only used for older children so it is dangerous. Side effects of this medicine are bone marrow and liver damage.

Eva and her mother are Austrian citizens. Eva is here on a student visa as she finishes her PhD at Stanford. Frau Jettmar's visa runs out next week so she would have to go back to Austria for at least a week leaving Eva by herself. I had planned on going down as often as possible while her mom was away to help, because caring for Kai is a difficult task even for two people, but her mom has been granted an emergency visa extension by the INS. On Tuesday I will have to pick her up at the CalTrain station and take her to the San Francisco INS office so she can receive the new visa.

Eva tell me they have now decided which label to give Kai's condition. It is called Lennox Gasteaux, and this is apparently the label they give when they don't know what else to call it. I've tried searching for information on the Internet but so far haven't turned up much (1,2). One article I found paints a bleak picture:

About 20 miles from Auburn's campus a freckled, six-year-old boy named Michael Barlett cannot speak, communicate or even feed himself.

Supervising Michael has become a 24 hours-a-day job for Sally and her husband since they received custody of him four years ago. "You feed him. You do everything for him," Sally said. "The only thing he does is walk and hold his own sippy cup. He is not like a regular kid where you can just open the door up and let him run outside and play in the front yard. He doesn't comprehend 'I can't go out into the road.' He just wanders."

But I do find it encouraging that at least that child learned to walk.

I'm concerned about Kai's ability to walk. Obviously avoiding a life strapped to a wheelchair would be a good thing, so earlier today I posted a request on craigslist asking for any old unused baby walkers:

I'm hoping to try to design a safe walker for him, where he could wear a torso harness over his clothing which is suspended (perhaps via bungee cords?) from a support structure attached to the walker. I'm hoping a couple of kind people could donate an old walker for me to experiment with.

I'm surprised at the dearth of information on Lennox Gasteaux on the Internet. I will certainly start posting anything I find out about this disease here. I also have copied one article which only exists in the google cache to my website (here), this one talks about a high fat diet which has met with some success in controlling seizures in otherwise untreatable children.

Comments

[Laura]

My heart really goes out to you, dav - what a terrible thing to happen to a baby. Yes, a feeling of helplessness is awful; I'm sure your supportiveness for Eva is making a difference. Regarding the walker - you could try using a "johnny jump-up" as part of it. (it's a baby thing, check ebay) Can he not learn to walk because of strength in his legs? Have you tried an exersaucer (also known as supersaucer)? That will help in building strength and holding himself upright. I think with a walker a child may learn to propel themself around but it doesn't do much to strenghten their legs. I guess it's all in what the purpose is. Hmm, I may have an old walker buried under the junk in my garage - want me to dig it out and mail it to you? If you are looking for walkers to experiment on, you can usually get some at yard sales for just a few dollars. You can sometimes get exersaucers and jump-ups too, but the exercausers usually run around $15 - $20 at yard sales. Safety 1st makes a good walker with a high back seat and wide sides, so Kai could probably use that without worrying about him hitting his head (although you may have to move the toys on the front part of the tray). I was looking at some of the websites for the LG syndrome, and a couple listed a Ketogenic Diet for children - is he on that yet? The sites said it was a last resort I think.

[Dav]

His legs aren't terribly weak (but they've never been strong either). The concern is that he won't learn to walk because it's too dangerous for him. He won't get enough practice because he can only try it when someone is holding him and his head up for safety right now. In fact crawling can be dangerous with the atonic seizures.

I'm just thinking that with a walker he can at least have some sort of safe mobility that also encourages him to get used to some sort of upright self propelled movement. I've had two offers for walkers now, so I'll try to pick up a couple of them this week and start experimenting.

The Johnny Jump-Up thing looks like it might have some possibility, but what he really needs is something that can hold his upper buddy upright also (which is why I'm thinking of a torso harness). Eva had bought him a baby life preserver sort of thing hoping that the padding would help protect him during the collapses but it didn't work very well. However I'm thinking I can turn that into the harness.