Too much fun

Last Friday we had a sudden, heavy downpour while out for a walk. We came home and sat on the front porch to watch it, but the kids wound up running around, playing in the rain. Pretty soon there was a river gushing down the street gutter and Melanie, James and best bud across the street were running through the river-- having a blast! After a while, the boys were still in the gutter, I was standing in the neighbors' driveway, and Melanie was smiling up at the neighbor... Then I hear "uh oh, uh oh!" I turn around to see Melanie slowly crash her face into their fender on her way down. I scooped her up, kicked off my sandals and ran across the street. Nick was inside and saw me coming, so he had the midazolam ready when we got inside.

She was fine by the next morning. There's always a little more sass and a little less fine motor ability the next day, but she's ok.

She's not ill. This was triggered by the extremely exciting fun in the pouring rain. We'll just have to be more careful next time.

"Tell Me Something Good"

As I drove Melanie to preschool this morning, I tuned in to Dave and Carole on KLH (classic rock station) and heard the end of their "Tell me something good" segment. Listeners can call in or email with some piece of good personal news, for a happy contrast to most of the stories in the news these days. I wished so badly that I'd had a chance to call in this morning!

I realized a few days ago that Melanie's inflection-filled, unintelligble babble is.... gone!! When did this happen? Every word she says is a real word. Not always clear, sometimes not understandable, but no more "fluent aphasia," as Dr. H called it. Some phrases don't use quite the right words, such as "Mommy, where's a you name?" but who wouldn't understand exactly what she means? She's using whole phrases and forming entire thoughts with complete sentences, people! C'mon, say Melanie's newest favorite word with me: "BOOYAH!" And a few more: "Ha-wa-what?!" and "What you talk about?!"

She can repeat/copy any word- not perfectly- but hey, a year ago she couldn't even say two one-syllable words together with two different beginning consonants. ("Pink toe" was 'pih poh.') Now she can approximate "hippopotamus" and "upside down." A typical thought from Melanie's lips: "Jae, you sitdown here. You prayer, a brush-a teeth, a go nigh-night. Jae, come-a here!"

She can sing her ABC's! On pitch!!! (She's got better pitch than her brother!)  My absolute favorite sound in the world is Melanie's singing, along with her sweet voice playing in her room when she wakes up in the morning. I love to just lie in bed and listen to her through the monitor, talking to her baby, singing ABC's, "Baa Baa Sheep," reading her favorite books... That sound is like being wrapped in silky-smooth, freshly-washed cotton sheets-- only on my insides. Ahh, I just love it.

Thank you all for your prayers! You have no idea the magnitude of hardships Melanie and our family have been spared, considering her diagnosis. There is so much pain, suffering, sadness, frustration, tiredness and loneliness in these Dravet families. My heart breaks for my brother/sister-tiger parents, but at the same time rejoices over the wellness and great strides we continue to see in our little girl. Did you know that she is still in what are supposed to be the rockiest years for a Dravet kid? Between about 3 and 5, she's "supposed" to be having too many seizures and ER visits and ICU stays for respiratory infections and major regressions and GI issues and behavior problems and and and....

She's not following statistics now, and I hope she never does. No matter what, she'll always follow the course God has laid out for her. And no matter what, it will all be part of His good and perfect will.

Soli Deo Gloria

Minnesota Adventures

We had a long vacation in the beginning of June, when we spent 11 days in MN visiting family. Melanie brought lots of smiles and joy to her family, especially with her significant gains in speech and understanding. And her dancing, of course. :-)

The last few days of our trip were spent in SW Minnesota, with Great-Grandma and Grandpa DeVries. We went out to Great-gpa's farm, where Gr-Aunt Jan and Uncle Ajay now live, and the farmland is rented out. We were getting a tour of the acreage and the family garden on a warm, sunny, windy day. We passed by (down-wind) the extremely pungent cattle yard, saw the garden, and the kids walked through some tall grass. Uncle Ajay picked up Melanie to help her to some shorter grass, and when he set her down I saw her staring at the sky, blinking. Poor Ajay! I assured him it had nothing at all to do with him carrying her. Nick ran back to the van to fetch the midazolam, and I ran back with Melanie in my arms, causing a mini stampede in the cattle yard (which made James laugh). I got my workout for the day! She was already coming out of it when we got to the van, but I tried the first spritz anyway. She whined, so I stopped with that.

I sat with her in the van while everyone else toured the buildings and James got to climb on machinery and then got a ride with Great-gpa on his restored 1951 Allis Chalmers tractor. By that time, Melanie had gotten over the post-ictal screamy pain and sat in her seat with a pacifier while she watched the tractor.

We got back to the house and put Melanie to bed. She woke up happy and recovered, so in the afternoon we walked two blocks over to visit Luella, her twin Dravet sister! They live so close to Nick's family in this small farm town! Our six kids played together in the sandbox and backyard, and I got some precious photos of Melanie and Luella. They even have the same blonde curl in their ponytails. I'm so glad we got to see them.

At dinner the same day, Melanie was thrown into a full-on tonic-clonic (grand mal) at the table. I'm thankful that only Gr-gpa seemed to notice. I wouldn't want to leave Gr-gma with that mental image. We took her to the front room and used the midazolam, but I think this one would have ended itself. Nick and I took turns laying down with her on the sofa and then eating. At some point, she had another seizure, but this one was also quite short and less violent.

This breaks Melanie's pattern. For a couple years, she only seizes when she's sick, and we just see one seizure-- usually lasting at least 5 minutes, with a long, ambiguous post-ictal phase. Well, last week she was not sick, but had numerous triggers on the farm, and I believe I mispacked her meds and she was a bit short on wonder-drug. Also, 3 in one day is unusual. She may be evolving to seizure clusters. But I'm wondering if that might be healthier for her ultimately. If her airway is blocked, I'd rather that happen during a very short seizure. She also recovered much more quickly from these. Normally it takes 2-2.5 weeks for her to fully recover. This time, she was "off" for only one week. Her light clicked back on yesterday, and now her walking is back to her norm, her brightness is back, cognition is back (and progressing), and she's eating again.

This tractor was built in West Allis, WI, almost in our neighborhood!

(You can see Melanie in the van, watching)

Melanie (R) and Luella (L) in Luella's back yard. They so enjoyed each other!

Gluten-free-free

Just as we had suspected, we've seen no changes in Melanie since our gluten challenge. We waited a few days, then gave her a little more, and now we're basically just done with the GF diet. She never really showed many signs of gluten intolerance to begin with, but we wanted to see if we might see improvement in mental clarity and cognition. She seems to be just as happy and forward-moving in her development, as she was during and before the diet.
It was worth the try, and I'm thinking of doing a little GF trial for myself, now that I know how to do it. I could do with less fatigue and brain fog.... Besides, somebody's got to eat all this GF pasta and such in our pantry.
What will remain from this GFCF trial is our avoidance of artificial colors, sweeteners, etc. Do you know how much of that stuff is banned in Europe? Those things are poison. Also, I believe our family's gluten intake will remain lower than it used to be. I also have a better understanding of how to watch for food sensitivities and how to feed our family healthier, more whole foods. This experience exposed me to a lot of reading, discussing and learning, for which I am grateful. Can't wait for our CSA to start!

The Gluten Challenge

Tonight was our first gluten "challenge" for Melanie. It can take about three months for gluten to leave the system and for any needed gut healing to be completed. We removed gluten in January, but about a month into it there was a woopsey which kind of hit the reset button on the whole experiment. So tonight was the night I decided to give it a try. She had about one serving of my sister's whole wheat sourdough bread. I wanted the challenge to be a quality, whole grain food. But she did also have about half of the bakery cookie that grandma bought her yesterday (sorry mom, I ate half of it-- it was for Melanie's good...).
Please pray that we see clearly in the next couple days if she has any negative reactions to the gluten. We doubt that we have seen any improvement attributable to going GF, but hopefully this challenge will make it clear to us.

James was excited about the fun plastic wine cups I just bought, so he decided it would be fun to have a "Cup Day." All of our food today was served in cups: Dinner was eggs, chicken sausage and French toast sticks. Lunch was James's sandwich cut in strips, with cups of berries and veggies. Mom and Melanie shared guacamole. I don't think this was our last Cup Day. :-)

Nighttime Scares and Laughs

Melanie came in from playing outside yesterday afternoon, acting a little subdued. She had a temperature around 100. I gave her ibuprofen and let her chill in the rocking chair in front of the tv while she slowly ate some supper, asking for more "apposhauce" and home-made GF "shicken nuggets." She had a bath and seemed fine, but I guess we should have given her more fever-reducers before bed.

At 2am, Nick and I awoke to the alarm on the pulse-ox. I looked at the monitor, said "uh-oh," and Nick shot out of bed quick as a flash. I came upstairs with the midazolam and sprayed it right away. The convulsions stopped within a couple minutes. Then there was a long period of ambiguity, as her eyes were fixed, pupils constricted, and she shivered and shook violently, seemingly from fever. She was not responsive enough to swallow ibuprofen or water from a syringe through her fixed jaw.

In those questionable moments, a number of thoughts run through my head. "Oh golly, I'm so tired and I can't keep my eyes open. I'm going to be dead tomorrow. What plans will need to be canceled now? How can we tell if this will settle down? What would the EMTs do for her? What would they accomplish in the ER? Do we call 911? Oh, that would wake up the neighbors and boy, we don't need that ambulance bill. Or do we wake up James so we can all drive to the ER, which would be 10x faster. Or could one of us drive her there- could she stay upright in her carseat? Oh Lord, please help my baby. Make this stop. Show us that she is coming out of whatever this is. Oh God, I just want to hug her and drink in her freshly-washed skin and make her giggle. Where is that smile she's always wearing?"  Yes, the thoughts come in that order: the selfish ones come first. Because this is routine for us. We're used to seeing her in this terrible state. We do our thing, I think about logistics... Then, as I wait, watching my baby suffer, not seeing the smiley lover-bug I tucked in to bed just hours ago, that's when it hurts and gets scary.

She started waking enough to slowly get some meds and water into her, and she really tried to talk, though no part of her body was functioning too well. As I held her, she kept mumbling "sorry" and a bunch of other stuff we couldn't make out. Perhaps she was trying to describe what she was feeling? Feeling another seizure come on.... She stopped talking, eyes started flickering upwards and she was thrown into another clonic seizure- this one looking more intense, oxygen dropping down into the 50's. I tried another small spritz of midazolam, since technically her dosage was recently increased. This one only lasted about a minute, I think. I hated that one. I told her, "Let's not get into the typical Dravet habit of clustering seizures, honey!" Thankfully that was the last one. But that darned fever never went down far enough. She did sleep safely through the rest of the night and woke up looking bleary but happy and chatty as usual.

Now, let's end with a smile. That cute little smiley, giggley Miss Personality was the first thing to emerge as she came out of all of this. Through her sluggish mouth with her lolling tongue, she was saying things like, "Ahwuuuh rea' book." And when I pointed out how silly it was that there was a pot of water in her bed (for dipping hands and feet), she made the cutest attempt at a laugh. When daddy started reading to her, she started whining and mumbling, "No daaee rea' book!" I asked if she wanted mommy to read instead, and she looked at me through tiny slits in her eyelids and said with her usual adultish inflection one of her most common phrases, "Oh! Sure!" She had us practically cracking up, at 3:45 in the morning.

She's the strongest person I know. A mountain; a fearless tiger cub; my superhero.

Carrying on per usual the next day.

This scene never would have happened in the days of diastat.

Once again thanking God for the safer, more convenient, and way more effective midazolam.

My Bad: God's Good.

Joseph's brothers held hate and malice in their hearts toward their favored brother. They threw him in a pit and planned to kill him. But just in time, some foreigners just "happened" to be passing by, and their greedy hearts led them to make money off of their brother instead, so they sold him as a slave. Thus, Joseph was thrown into a dreadful fate over which he had no control. Through a series of events (many of which looked like coincidences), Joseph became second in command under the king of Egypt. He saved the country from death and utter ruin in extreme famine. Ultimately, he saved his family- his very brothers who hated him and sinned against him in the worst way- from death and ruin. And because God put Joseph in this place of power, with a righteous heart full of love and forgiveness, his family was brought to Egypt to ride out the remainder of the famine and father the nation of Israel.

This is my favorite piece of bible history. There are so many lessons and reminders and promises woven throughout the story. The most obvious and comforting of these is that God will work all things to the good of those who love him. Joseph's brothers acted in malice and sin: God turned even that to an enormous blessing for Joseph and even his wicked brothers, and ultimately the nation of Israel, God's chosen people. It is an awesome lesson, reminder, and promise from God. I'm always blown away when I think of it.

Today I was reminded that the Lord does these works in even what seem like the minutia of our lives. Two days ago I utterly forgot to give Melanie her morning meds. It completely slipped out of my brain for the entire day, until the evening when I was cleaning and dealing with a wailing and limp Melanie in post-ictal pain. I told Nick to throw me in the bad-mommy dungeon. Of course he didn't, and instead comforted me, but I threw myself in there anyway. It was my bad.

This morning I called the neurologist's office to discuss a couple things, and I said in my message, "In the past, Melanie has been able to miss a dose of meds and be fine- no seizures. The fact that this caused her to seize tells me that her current med doses are near their minimum: right at the door of her seizure threshold. Maybe we could increase [wonder drug] before tapering [yucky drug] any more." We were due to taper yucky drug another step yesterday... Of course I have held off on that.

What does this have to do with Joseph?? Well, I realized this morning that God brought some good out of my forgetting M's meds on Wednesday- a rare oversight- so that I could have a clear sign that she was teetering pretty close to the bottom of therapeutic in her med levels. So now we can approach tapers and increases with more wisdom and knowledge. Even as I write this, it seems almost petty. We could have seen the same thing if we had just continued on our taper schedule... but, without going into the details and subtleties of what we know of Melanie and her seizurey brain... trust me, this was a much more definite, clear, and perhaps even less painful answer. God's good.

She is fine now. The seizure wasn't even discovered until after it was over- so it was short and self-ending. Nick didn't even recognize that she was post-ictal when she was hot, limp and wailing, with wet pants. She woke up chipper, sassy and defiant the next day. Her preschool teacher barely recognized her and even had to put her in a chair after too many No's. This is normal day-after behavior, so she was given much grace (but not too much!). I'm seeing more seizureyness lately, so I'm waiting to hear back from the neurology nurse, expecting her to tell us to increase diamox before a further taper of depakote. ...I am totally a Dravet M.D. (Mommy Doctor).

deo gratias