SUDEP

Cure SPG4 has added Sarah to their website with an “In Memory of” overlay, and with some words we wrote about her. If you make it over to the Foundation’s webpage, scroll down to the bottom to the “We Are SPG4” section and swipe through the kid’s little bios. Sarah is kid number 4, all the way to the right, and if you click on her she pops open into a full page where you can leave her a little “heart” (and if you feel like it leave hearts for the not-dead kids as well). It’s quite nice, I think. I won’t flog you about donations because there is a button right there if you are able and willing to do so. There is so much need these days, you have to decide where and if you can help.

I don’t see any other kids on the website with an “in memory of” overlay on their photos, so either no other parents have informed the Foundation about their kid’s death, or as I fear, we are the only known death so far from complications of Complex Early Onset SPG4 as it’s now called in high society circles since about 2020. Although, of course, Sarah also rides with the SUDEP kids (Sudden Unexpected Death In Epilepsy), the Cerebral Palsy decedents, and the plain old vanilla SUDC (Sudden Unexplained Death in Childhood) members. Not to mention the Asphyxia cohort? Okay, this is getting ghoulish. But what I mean is that perhaps there are some SPG4 kids who have died and simply are not attributed to complications from SPG4. They and their families may not have even known about the rare disorder. It takes a lot of time, sick leave, and money to pursue this level of medical care and diagnostic testing.

Sarah had her first seizure when she was 14. Max was telling her a bedtime story and I was in the living room and all of a sudden he shouted my name very frantically. I ran in and we saw her having a grand mal seizure which lasted about 20 seconds. She recovered well but was very sleepy. We talked to her neurogeneticist and he got us a referral to an epileptologist at Johns Hopkins.

I had always watched Sarah very carefully for any sign of seizures because I have taken medication to control seizures for many years. Like any illness, my epilepsy has waxed and waned. The past 20 years or so it’s been controlled very nicely by medication and I barely think about it at all. However, my pregnancy with Sarah was delicately managed for this complication and it’s one of the reasons I waited so long to have a child.

Sarah didn’t have a whole lot of seizures, maybe 4 or 5 in her whole life. They tended to occur when she was tired out, at the end of the day. They usually didn’t last long and we could give her rectal Valium to stop them if they lasted more than a minute or two. There was no evidence we were aware of that she was having seizures during her sleep, but we didn’t do an awake/asleep EEG and it was never suggested to us. I knew about epileptic bed death, as it’s sometimes called, but associated it with people who have severe, untreatable seizures — not my daughter and me.

I guess that’s really the point. I did not see death touching my family or coming for my family. I thought I had covered all my bases and checked-off all my to-do lists to keep my family safe. Now I see death everywhere, constantly, every time Max sleeps late or Polly coughs or my sister-in-law has surgery. There is no safe place anymore.