Finnan’s going to be three years old in less than two weeks, which in rare genetic disease time is pretty far off. Using the future tense when referring to Finnan is not something I like to do. I’m just happy if he wakes up each day and carries on breathing. Just this week, one CDG child stopped breathing and was rushed off to the hospital. His mom commented on how one day he was so happy and smiley, and the next day he was hooked up to a machine which was breathing for him. So every day really is a blessing.
However, in real time, Finnan’s birthday is pretty close, looming even, and there are things to be done in preparation.
And not just the party. (Well, it’ll be more of a mini-celebration-that-keeps-to-Finnan’s-routine-and-favorite-people-so-as-not-to-freak-him-out party. God willing.)
No, it’s time to prepare for…
The late-infantile and childhood ataxia-intellectual disability stage, with onset between age three and ten years, is characterized by hypotonia, ataxia, severely delayed language and motor development, inability to walk, and IQ of 40 to 70; other findings include stroke-like episodes or transient unilateral loss of function, retinitis pigmentosa, joint contractures and skeletal deformities.
In other words, Phase II is going to be more of the same, plus the dreaded potentially fatal seizures. And there is plenty of evidence from my CDG friends on the web that these seizures are not friendly.
Bit scared. Don’t really want him to be three much now.
Anyway, time to stop sticking my head in the sand and get out the first aid manuals. I did do a first aid course which took three days, but hopefully my qualification has expired because all I can remember is bandaging up a rather delicious music teacher wearing a very stroke-able knitted tank top.
If you’ve got an eye injury and need a bandage, I’m your girl.
Fortunately, I took copious notes on the course, saved every scrap of first aid literature I’ve ever seen or cut out of a magazine, AND brought it all with me to Thailand. So being paranoid has finally paid off. (I knew it.)
So in teacher mode again, I have the whole family pretending to have seizures so that they know the symptoms. They scream, collapse, roll their eyes, foam at the mouth, go stiff and jerky, stop breathing and wee themselves.
OK they don’t wee themselves, but you get the image.
The kids are quite good but my husband’s acting skills need work. And my husband also points out that Finnan would already be on the floor on a soft blanket anyway, so he wouldn’t collapse.
Then, using teddybears, they 1. Clear the area 2. Loosen clothing around the neck and body 3. Put teddy on his side and 4. Put a pillow under the teddy’s hips and head.
Then my husband has a private lesson on resuscitation.
How I wish that was a euphemism.
Teddy has stopped breathing, but he still has a heart beat, so we give him 20 rescue breaths – one every three seconds. We pick up teddy and take him with us, while doing rescue breaths so we can get him in the car to go to the nearest hospital. Teddy’s breathing does not recover and his heartbeat stops. We find his nipples (OK, we guess where a teddy might have nipples) and put the heel of our hand two fingers’ width above them. We press his chest down five times singing the Bee Gees (ah ah ah ah staying alive, staying alive- yes it really should go at that speed according to my notes), then we do another rescue breath. We keep doing this until teddy’s heart and breathing recover.
Also, we are supposed to make a video of Finnan’s first seizure and my husband will have to do it alone and car-less if I am at work. There are no ambulances.
I’m starting to look a bit wild-eyed and panicky, so my husband has to give me a calm down talk. It helps– although I do keep springing first aid test questions on everyone at random moments. Maybe I should stop worrying and start focusing on which birthday cake Finnan would like on his special day 🙂
Interested in taking a first-aid course in your area? Check out listings here.