Does its job

Well, the pulse-ox machine works for its intended purpose. Nick and I were repeatedly awoken by the alarm in the wee hours Friday. So, Nick went up a few times to untangle or fiddle with it to make it stop false-alarming. (that's his job, since I can't fall right back asleep after running upstairs) Well, it kept going off, so I went up to try it and to offer her water, since her sinuses are still draining from this cold. She was warm and trembling. Sight fever, so I gave ibuprofen and kept asking if she was ok. "Yes." do you feel sick? "no." I went down and got back in bed, and not two minutes later the alarm goes off again, I look on the monitor and see her arm outstretched and shaking... 
Got the midazolam in quick and convulsions were soon over. Her eyes were goofy for a while, and she was barely responsive, so I gave the rest of the syringe at 8 minutes from the start. There was a whimpered moan with that, so I think she was coming out of it.
She didn't wake up to try and shake it off right away- just slipped into (finally) a deep, restful sleep. I lay down to sleep with her, but after an hour I realized I would never be able to sleep. I roused her and she responded, recovered, so I went back to my bed.
She's been ok since then. Unlike most Dravet parents, we get to ask "why?" Most DS kids have random seizures all the time, but for two years Melanie has only had them with sickness. She'd been through the worst of the cold already, so why now? Well, she had a fever so I thought it might be sinusitis, but there are no signs of that. In the morning I discovered that she had missed a dose of meds, which I'm thinking, combined with still suffering from the lingering cold symptoms, brought her threshold down. The fever could have been a result of seizure activity. Maybe the restlessness and trembling before the "big show" was like an aura, which is actually seizure activity itself.
I'm anxious to see her get over this cold, so we can see if the diet is helping at all. She's been totally GFCF for over a week, but we see no change yet. Keep the germs away! We need a healthy girl!

New diet

We are fully into the GFCF diet now. We removed dairy a couple weeks ago, and planned to stick with just that for three weeks. Melanie got sick about a week into it, so it was hard to decipher if cutting out dairy has made any difference. The first week of it, she seemed a bit more moody and easy to tantrums- a possible withdrawal symptom. But then again, perhaps she was just starting to get sick. And of course, when she is ill, all of her skills and overall wellness take a step back for a couple weeks.
I decided that it wasn't really worth waiting to start removing gluten. So I believe Friday was her first completely gluten-free day. We haven't seen any changes, and she is still under the weather. Feeding her hasn't really been much of an issue. I haven't even purchased special flours or baked anything, I haven't stocked up on GF pretzels or any of those substitute foods. She's been snacking on nuts, seeds, veggies, veggie chips, guacamole, popcorn from the family farm... She still asks for string cheese and crackers, but it hasn't been too hard to redirect her so far. She likes almond milk (lots of calcium) and even the disgusting "cheese" I've melted on corn chips and put on her taco bowl tonight. Blech. I'm thankful she's not picky.
Once she is fully recovered from this cold, we should start seeing if the diet is making any sort of impact on her in any ways.

Jumping into a new diet, and Mommy plays doctor

We had a good holiday break. For a couple weeks before Christmas, Melanie had become quite screamy and moody, with frequent tantrums. I noticed that she would have frequent pauses mid-word while she was talking. She talks a lot, so I noticed it a lot. The only possible explanation I can think of is increased seizure spikes in her brain. This could explain the change in her mood and behavior: Imagine having a brain blip every 10 seconds or so throughout your day, every day. When someone is talking to you, you would miss a word or two each time that happened. If you're moving or doing a task, somehow your hand just made it from your mouth all the way down to the table in a split second- but you don't remember it actually traveling down. You know that was what you intended to do, but how did it just suddenly get there? When I know that sort of thing is going on, I try to cut down on sudden movements, which might confuse her, and I give her extra time to register things.

That kind of constant spike activity can really be a detriment to learning, as I recall hearing in a lecture at the Dravet conference. That would also explain why, after the initial boost in speech and cognition after our last depakote decrease, she seemed to be plateauing again. So, with the doctor out of town for the holidays, I decided to play neurologist myself, and added just an extra 1/4 pill of diamox (our favorite drug) in the mornings. Instant change! Part of it was the happiness and excitement of Christmas and traveling to MN which boosted her spirits, but it was clear that she was happier and calmer in her brain. The pauses decreased, and speech started exploding again! I think we could safely boost it to an extra 1/2 pill, to help even more. I'm sure this set-back happened because of the tapering of depakote, but I refuse to go back up on that awful drug....especially after seeing that diamox can effectively take its place.

Two big news items for Melanie, besides what you just read: On Thursday night (1/3/13), Melanie jumped, with both feet off the floor, for the first time ever! She seemed to reach both hands between her legs to pull herself up by the butt to do it-- it was cute. And she did it more than once, and more times the next morning. Of course it's not consistent, but she can do it!
The other news is her new diet. We're starting the GFCF diet for her, to see what sort of brain-clearing improvements we may be able to get from it. We're starting by cutting out casein (dairy) for at least three weeks, and then we'll cut out gluten for three weeks. Perhaps then we'll try adding back casein to figure out which, if any of those things actually makes a difference. I'm kind of thinking already that dairy has been affecting her sleep. She has slept a little better and woken up happier without dairy at night. She's still had a little during the past few days, as we sort of ease into it, but I've tried avoiding it in the evenings. I will be taking careful notes.

James and mom start school again tomorrow, so I have been saving Melanie's Christmas presents so that she has brand new activities to do during school. We all need routine again after the break.

More sleep, less poison, more words... no cheese

We recently acquired a new piece of equipment which has been such a blessing already in peace of mind. After a long, exhausting battle with the insurance company, we now have a pulse oximeter in Melanie's room. It's just a small machine attached to a glowing sensor wrapped around her toe whenever she sleeps, monitoring her oxygen saturation and heart rate. We have it set to alarm if her O2 drops below 88%, indicating a possible seizure. In my mind, I also think of the higher risk of SUDEP (sudden unexplained death in epilepsy) in Dravet kids. One of my biggest fears, besides not waking to the sounds of a seizure in the middle of the night, is waking up to find Melanie has slipped away while I slept. I know this pulse ox won't save her life if an organ decides to shut down, but I can't even explain the peace it brings me to know that she will never simply slip away without our knowing it. Of course the main reason for having this machine is to be alerted to nocturnal seizures. The few times I have awoken to the seizure breathing sounds it he middle of the night, I have run up to her room to find her face-down and blue. Now with this machine, I don't have to worry that I might not wake up to those faint sounds next time. I can sleep so much better now!

Melanie's last seizure was the Saturday before Thanksgiving. As usual, she regressed in speech, mood, behavior, etc. after the seizure. It usually takes at least two weeks for her to get back to baseline. This was the shortest tonic clonic (grand mal) ever, because of the wonder-rescue-med, but it took two and a half weeks for her to recover. On Wednesday of last week, I saw something click in her, and she became brighter, happier and better. During that post-seizure slump she may not lose a whole lot of words or anything too severe, but her forward progress definitely halts for a time. When she snapped out of it this week, we instantly started hearing new words and speech sounds, and her cognition/understanding started improving again. I'm not sure how much this has to do with it, but at that same time we tapered her ickiest medication. We've been slowly bringing down her depakote, since it messes with... everything: liver, appetite, metabolism, behavior, cognition, speech.... It's poison. Every time I request a decrease, it makes our neurologist "nervous," but of course she always leaves it up to us. It doesn't make mom nervous, though. I would just love to cut this one out of her cocktail completely! I see Melanie emerge more and more with each decrease.

We are planning on trying a special diet in the new year. It's gluten-free, casein(dairy)-free, and it is touted mainly for treating autism and ADHD. We're hoping to see improvement in Melanie's cognition and sleep and overall wellness. I'm doing research right now, trying to get as much planning as possible done before we get into it. It will be hard, but I know we can do it. If it helps her, it will be more than worth the work.
Sometimes I just can't believe how great Melanie's seizure control is right now. We continue to lower med doses (I've even been skimping on her keppra more and more each week), and there is no visible increase in seizure activity. We only see positive improvements in everything else. Only one answer for that: God's amazing protection and the many and constant prayers of those who love us.
Deo gratia

Quickest Ever

Melanie's infrequent seizures have followed a couple of patterns for at least a year: Either we hear and find her face-down in bed, full-blown tonic-clonic (grand mal), or if she's awake it would start slowly with the eyes and then generalize at some point. Well, Dravet doesn't like you to get too comfortable with any kind of pattern for too long.
Last night, as I was preparing dinner, Melanie was passing back and forth through the kitchen shaking maracas. Grandma Johnson and I were right near her, smiling at her as she passed through. Suddenly she fell back into a full tonic-clonic, thankfully with a bag of clothing right behind her and not a door frame. Nick laid her down, while I got the midazolam sprayed up her nose within a minute. The convulsions stopped within a minute, and she fought off the remnants of the seizure for a few more minutes.
Dad sat with her in front of a football game for over half an hour, while she rested. Within 10 minutes she was opening her eyes a bit, and within 20 minutes she was exclaiming at the game, "oh no! Woah!" She needed help with dinner, but boy oh boy, I've never seen her recover so fast! Today she was not showing significant lapses in motor, and her speech was just a bit sub par. Her nose is getting congested and she's cranky and edgy, so I'm thankful it was not a "random" seizure. Haven't had one without illness since February 2011. I'm still nervous that it may be the stomach bug I came down with Thursday night, but so far we're just seeing stuffiness. I'll take a head cold over a stomach thing any day! For myself of course, but especially for my little girl who needs to keep her meds in her body and who has never thrown up before...
It's like night and day, comparing this new rescue med to the worthless diastat we were using for years! No doubt, if we were still using that stuff, it would have lasted at least 20 minutes, we may have ended up in the ER, and she would be unable to even walk today.

Make November purple

October was breast cancer awareness month, and everything turned pink. Did you know that, on average, more people die in the US each year from epilepsy than from breast cancer? I've never been a really big "awareness" kind of person, but when you see your child and consequently your entire family suffer from such a terrible and unpredictable disease, it's hard not to be changed by that. I am saddened and angered by the lack of awareness of seizures and seizure disorders, but even more so by the lack of research and funding. There is so much more to be understood, and our loved ones need better treatment. It's out there, ready to be found, researched and utilized. We need to start with that ambiguous idea of "awareness," with all its oppressive email forwards, Facebook posts, colored apparel and accessories, bumper stickers, petitions, news articles, brochures, walk/runs... and for us, personal stories. And when enough of the public make enough noise -and raise many funds in the process- then we can start getting a lot more accomplished. For us, of course, we care most about Dravet syndrome, and that is why you will find me bugging you, at least twice each year, to donate to the fundraisers in which we participate for the Dravet Syndrome Foundation.
Segue...
You have one more day to shop Maddie's Mall at www.maddietischer.net. And while you're thinking of her, praise God for Maddie's birth five years ago on November 3rd. Though her light was stifled far too soon, her legacy is already beautiful and prosperous.

Remember to wear your purple-- put a button or ribbon on your coat, wear your dravetfoundation.org purple silicone bracelet... tell people about it. I told our service man and then our waitress today. Caught them off guard, but that's ok. :-) I'll be searching for good factoids, buttons, etc. and share them as I find them.

Start with this:  http://cureepilepsy.org/aboutepilepsy/facts.asp  about epilepsy in general. And of course the two Dravet sites at the top of this blog are amazing resources.

Holy powerful nose spray, Batman!

...This stuff really works! That's the good news. Bad news is, we had to use the new seizure rescue med for the second time last night. Praise God we haven't used it more than that. We've had this new stuff for a few months now, and it works almost immediately to stop the seizure. The rectal stuff we used for years never really did anything, which is how we kept having 20-80 minute seizures.... I wonder how much time and brain health lost could have been saved if we'd made the switch sooner.

Melanie started coming down with James voice-stealing chest cold Saturday afternoon. I was encouraged to see that she started an illness, even had a low fever, without a seizure. Usually the seizure comes first, out of the blue. But since she had symptoms first, we knew to keep our ears and eyes open and to start the antipyretics right away. Still got hot and seized around 10 last night, thankfully before we were asleep in bed. Not much noise to warn us... I REALLY need to work on getting our insurance to cover a pulse oximeter to put on her during the night to operate as a seizure monitor.

She's doing ok today, all things considered. She's full of gunk and without much of a voice, but she's in ok spirits. This sickness lasted a whole week for James, but I'm hoping Melanie might be spared such a long period of suffering. And of course praying she doesn't seize again. Though the rescue med works so quickly, she does suffer a long and miserable post-ictal period, when her faculties don't really work, she's uncomfortable- shivery and feverish, eyes are completely absent and she can't talk or swallow for a while. And today her motor skills are sub par. But I'm always thankful when she wakes up after a seizure and still has her speech and other skills. So many Dravet kids have lost so much to "that one seizure." Praise God she has always bounced back! And today she said the most amazingly clear 7-word sentence when she sweetly said, "Please James, I want to sit down." Sometimes she comes out even better on the other side, like the seizure hit the 'reset' button.