Closing the year on a high note

We've made some treatment changes since our last appointment in November. First on my list was weaning the benzodiazepine. She was on a tiny dose because she suffered horrible side effects when we tried bringing it higher, and I doubted that it ever did anything productive. It's supposed to be one of the "magic three" for Dravet. It's also a very dangerous drug with horrid withdrawal symptoms, calling for a very slow weaning schedule. It took us about a month to wean her tiny dose, and she was a bit of a monster at times and had a few seizures because of it. I could tell she felt awful.
Well, about five days after her final dose, she was alert, sweet, obedient and exploding with language. Suddenly, I see her recognizing that her words don't sound quite right and she doesn't use the right grammar, and she is making a careful effort to fix that. She's also getting better at the phonics game we play ("let's think of words that start with mmm...").
We also began a daily dose of N-Acetylcysteine (NAC). This is a vitamin which has been discussed quite a bit recently in the Dravet support group. A number of kids have done well with it, improving mood and seizure control. Even though we're supposed to make one change at a time, I was anxious to start it and hopefully give her a little extra protection while weaning the benzo. I'm wondering how much of her recent "awakening" is because of NAC, and also if it has been boosting her immune system. She may be fighting something right now, but so far she's been healthy all season, avoiding many bugs around us.
We've been having a great Christmas break, spending time with both sides of the family. Melanie got some presents which will help us a lot in school, as we learn to read and write. I'm super excited about the felt boards made by Grandma Johnson!
The Lord has blessed us richly in 2013, and I know that whatever 2014 holds is in his sovereign plan and is for the good of us who love him, who have been called according to his purpose.
Happy new year, everyone, and thanks for following Melanie's progress! I know many have been praying for our little Rose. Please remember in your prayers the families of the 13 children who lost their lives to Dravet in 2013.

Productive Appointment

Whenever we get packed up and hit the road for our 6-hour+ car trip for a neurology checkup, I always question, "is this really worth it? Can't we find a suitable doctor closer to home?" I mean, I know she's a Dravet expert and I really trust and like her, but dude- this is a big trip....
Then we go to our appointment.... Yes. It is worth it. This doctor is worth it; this facility is worth it. (Besides, we get to stay with grandma and grandpa and see lots of other friends and family.)

Yesterday we had a check-up with the neurologist. She is pleased with Melanie's continued progress. We discussed her recent increase in seizure frequency, and how mood and behavior issues have ramped up along with that. I know some Dravet families accept some breakthrough seizures with lowered medication, in order not to interfere with development, cognition, etc. Unfortunately, seizure activity itself messes with Melanie, so we need to increase meds to keep her moving forward. Fortunately, she doesn't currently need toxic doses to control most of those seizures. She has been gaining weight, while not increasing- or at times even decreasing all her meds. According to body weight, she is on 1/2, 1/3, or less of the doses she was on a year or two ago. This is great news for her body! Fewer dangerous pharmaceuticals= healthier, freer body and brain.

That said, we will hopefully be increasing her (virtually no-side-effect) Wonder Drug, if her labs say it's safe. We discussed other options as well. We plan to add a supplement which has improved seizure control for many other Dravet kids recently. And we have started to wean her newest drug, which can be a dangerous one and probably hasn't done much to help her anyway.

I asked the doctor for her thoughts on medical cannabis, and she gave a very honest answer. Essentially, she admitted the lack of controlled studies, but did not discount the mountains of anecdotal evidences. She said if it were on the ballot in her state, she would vote yes and would support any family who would want to try it. She said she'd rather take mmj than depakote, but she'd rather take Keppra than mmj. Personally, I'd rather take mmj than anything sold by Big Pharma, but I'm coming from a different perspective and experience.

The doctor once again commented on Melanie's hyper-flexibility and her pronating feet and in-toeing. Basically, at age 5, she's still saying, "We'll have to watch that."

Overall, it was a very good and productive appointment. We have many plans for next steps and possible next steps, and I feel good about them. I am hopeful that we can help Melanie with some safer, natural supplements, rather than going on to new drugs or more drugs. I pray she continues to respond well to these less extreme treatments! This is not the norm for Dravet kids. Praise God.

DS stands for Don't Settle (down)!

When Melanie was diagnosed with Dravet Syndrome, we were all diagnosed with a lifetime of excitement and change! Can't get too comfortable for too long. One of Daddy's catch phrases, since I've known him has been, "If you're not scared, you're not living." Well heck, I'm livin' every day!

So, what's the change? Well, it's layered. Obviously we're out of our two-year stint of no non-febrile generalized clonic seizures (which began when we added WonderDrug, Diamox). Wwwhhhaat? Sorry, my vocabulary has changed since becoming Melanie's mom. This is what that sentence meant:
Feb. 2011: Dr. H put Melanie on diamox, an unorthodox choice, as it is an old diuretic, mostly used for mountain sickness. He thought it might help with seizures and ataxia.
*cha-ching* jackpot struck
Feb. 2011 - June 2013: Melanie only ever had a (big) seizure with illness: usually, but not always coinciding with the initial immune response (i.e. before symptoms).
June 2013 - Present: Five seizures not triggered by illness.

I'm also noticing poor behaviors in the days leading up to a seizure. But today I gave this some serious "momtuition" thought. If bad behaviors coincide with seizure activity, and she has actually been exhibiting these behaviors non-stop (or slowly worsening) for at least a few weeks, and seizures are coming more and more randomly.... Ok, when I list it all out like that, I feel dumb for not seeing it before. She needs a med change. But wait! I'm not so dumb after all: All of her seizures (now and in years past) happen in the late afternoon/evening. Annnd, she is always chipper in the morning, but becomes increasingly squealy and ill-behaved as the day wears on. *lightbulb* She needs something midday. So, since I've been successful at it in the past, I'm going to play neurologist again tomorrow and go ahead and add a 1/4 pill of diamox at lunch tomorrow.

Will let you know how our cute little ginea pig fares.

P.S. Melanie had her first day of horseback therapy today!  She had so much fun, and we are so excited to keep doing this. Praying for better strength, muscle tone and posture so she can suck in that low-tone little belly!

All the horses wore costumes today. Melanie's was a hula dancer.

Sympathy Candy

I knew there was a reason for Melanie's poor behavior all week: she was gearing up for a seizure. Many Dravet kids have that in common, and it looks like Melanie is joining that trend.

Just a little before getting ready to go out trick-or-treating tonight, while eating supper she began seizing. We have a neighborhood block party sort of night each year, and we had a couple families over for a party. We were eating in the garage before starting a fire and going out for ToT. It looked like Melanie was going to miss out, after an entire day of anticipation, but she heard the kids playing outside and mumbled, "I-ahh-trick-a-treat!" She begged and I was not about to deny her. So, SO thankful for the back carrier made by a Dravet grandma! We made it all the way up and down our street, three blocks. I was honest when people asked or commented on our arrangement, and we got some extra sympathy candy. :-) So, there's something.

I'm going to go take a hot shower now and rest with the heating pad...

Had no idea her costume would be so fitting tonight!

She was pretty weak, but very happy to go out in the fun.

Back to our once-a-month pattern??

Melanie had another seizure last Friday. She is not sick, so I'm not sure what the trigger was. Perhaps it was the excitement of Daddy pulling into the driveway. She and James were outside playing: she was not too hot or cold and she had her sunglasses on. In fact, she had asked if she could go outside and I said, "Yeah, hold on," while I was bustling around the house. Before I knew it, she had her shoes and glasses on and had let herself out. My big girl! I stayed inside to do some things, keeping a close ear out. Thankfully, Melanie talks and exclaims constantly while she plays so I don't really have to have an eye on her at all times. At some point, I heard Nick pull in, but I did not hear the usual, "Oh! Hi Daddy!! Daddy, you home! Aw, hi Daddy!" Just silence. Then, "James, go get mommy." He found her sitting on a trike, looking upward.

I tried some potent essential oil under her nose, as many parents report that can wake up their kid's brain from a partial seizure. But at minute 2 or 3 we saw it move into her hand, so I sprayed the rescue med. The last minute of this one looked odd and frightening. Nick grabbed the phone and turned it on, but I told him to wait. Then it ended. I believe she is spared the memory of anything unpleasant.

James always tells her to say something, and "Melanie, why aren't you talking?" during a seizure. Well, it took a while for her to come-to. Still not sure if that unresponsive sleepy state is non-convulsive status epilepticus, or if it's just her post-ictal brain-recovery state. I need some better answers from our neurologist. (Or any seizure parents reading this with some insight?)

Good news is that she recovered pretty well from this one. She looked more "seizurey" for about 3 days afterward and her endurance was definitely diminished, but we haven't seen the intense behaviors and refusal to listen too much this time. All of those after-effects tend to linger for a couple weeks, until her light clicks back on suddenly and we see our sparkley little Rose.

In the last roughly three months, our online Dravet community has mourned the intense struggle and loss of three young children who suffered "that one seizure" which landed them in the PICU in a coma, as their organs shut down one by one. Today it was a family across the world, but a wound close to home.

The Ugly Thief

...has carved another hole into another family, leaving empty arms where a darling son and little brother should be.

We've had at least five different Dravet kids go into crisis, in ICU, all around the same time here. Some coming around, others not at all... This syndrome is an ugly, nasty thief. It comes in the sunshine. A child has been seizure-free for months, even years: so healthy in so many ways. Then WHAM! The seizure that takes it all away. Or it slips them away in their sleep for no known reason.

I hate that the word "hope" has become a happy, touchy-feely empty word thrown around when hurting people are searching for something, anything to cling to. It is such a deep, real human need, yet the answer so many latch on to is this carved out shell of a truly BEAUTIFUL word! When Hope is rooted in the Creator of the universe, our loving Father in Heaven, there is nothing more beautiful and fulfilling. And what an answer to pain! What an answer to suffering and death! God the Son has conquered death: what could be better than putting your hope in Him? the conqueror of death?! There is no other way to survive the pain of this world and remain whole. There is no other way to escape death yourself.

I have hope that these children who have suffered greatly in their earthly lives, being robbed of the mental capacity to understand these profound spiritual truths, are welcomed into the gates to join all the saints before the Lord to sing his praises for eternity. No more pain, seizures, tears, suffering... I believe this because I know the loving God whom we serve. There is much pain and death in this world broken by sin. Not one drop of it is allowed in the presence of God.

Please watch this and pass it on

This is the documentary called WEED, by Dr. Sanjay Gupta, which aired Sunday night, August 11 on CNN. Please excuse the angry title attached to this YouTube video, but it is the full 42-minute show.
Dr. Gupta highlights one of the pioneering families for using cannabis (medical marijuana) to treat Dravet: the Figis. The beloved Stanley brothers in CO have developed a strain named after the daughter, called Charlotte's Web. It's extremely low in THC (psychoactive) and very high in CBD (medicinal), and every family wants it! Including us! Please watch with an open mind. I believe Dr. Gupta did a great job covering this controversial subject in a fair way. And remember that Charlotte's story could easily be Melanie's, as they do have the same syndrome.

http://www.youtube.com/watch?v=Vdiu-samktQ&nomobile=1&gl=US&guid&hl=en&client=mv-google

And here is a good article outlining this doctor's change of heart on medical marijuana and his research-based reasoning:

http://www.cnn.com/2013/08/08/health/gupta-changed-mind-marijuana

Please check it out, and pass it on, and contact your representatives. The pharmaceuticals are harming Melanie and won't work forever. We need this to become a compassionate state, legalizing medical marijuana. I don't care one bit about recreational use: I just want this completely natural, effective and safe medicine for my daughter. She would not get high, and she would take it as a tincture or a pill. This plant is not merely used to ease pain, as I used to think: it literally cures most seizures in many, many patients (not to mention cancer and a host of other diseases). Cannabis has been used as a highly effective medicine throughout history, and there are no side effects (when using a low-THC strain).

Try on Grandma's Shoes

So, imagine that you and your husband plan a big, exciting trip to a child's paradise, to enjoy an exciting vacation with your precious grandchildren. In the late afternoon of your first full day there, you are alone with your little "princess," as the Disney cast members call her, and the two of you enjoy multiple trips on her two favorite rides. When she goes limp as you walk off your third carousel ride, the magic comes crashing down. You've seen her have a seizure once before, in her own home, with her parents there to take action, and it ends quickly. That was painful to watch.

Now it's all on you. You're in a crowd of strangers and have to run 10 feet to the stroller parked in front of the cute flying elephants. You have your son's coat to lay on the ground, you have the rescue med with instructions tucked somewhere in your brain and on a card, which suddenly seem to be written in Chinese. You know she needs that right away, but her parents need to be contacted right away. So which - when - how - wait, first - no, maybe - where - how - breathe --- Suddenly you hear two women's voices: "Do you need help?" "Yes! Take this phone and call Nick." Phew, that's taken care of. Now, how do you spray this stuff up her nose? Pull? No, twist. Why is it shaking so much? Oh, that's your hands. Ok, breathe. You get it in, but you're not sure if you pushed it fast enough, or if it just dripped in there. Then an angel in the form of an ER doctor shows up. He gives her another dose, making sure it's sprayed and absorbed. He assures you she is ok, even though she is turning blue. He continually checks her pulse and asks if you have a cloth to catch the spit up he correctly predicts will come. He brings a confident calmness to the situation. A woman is holding her hand. The parents arrive. It's no longer all on you, but how do you feel now??

I sincerely hope you feel not an ounce of guilt for any part of any of it. As far as a trigger: it was a culmination of everything loaded on her senses over the last two days: sleep deprivation, travel, hunger, heat, excitement, noise, movement, newness... And as far as the medicine: you should have seen my first time with the atomizer! And that was in the quiet of my own home. I think Gramma deserves a trophy for the way she handled this nightmare, but I don't want to give her anything that will remind her of this horrid scene. So I invite everyone to leave a comment on this post, giving kudos to grandma for handling this frightening situation so well.

And by the way, that doctor just "happened" to be spending the day at Disneyland with his family, when his wife alerted him to what was going on. How could anyone not see God's providence in that? He even knew what Dravet was. And he stopped by the first aid station later to ask how she was doing.

All of the Disney workers/cast members took care of us so well. They even cleared out all the beds when we arrived to first aid. They offered us stickers and front-of-the-line passes for our lost fun time. They brought in Pluto for our own private meeting, lighting up Melanie's face and healing her with giggles.

While we were recovering at First Aid, I got a call from Michele, a mom whose beautifully long-lashed son :-) also has Dravet. They live very close to Disneyland and visit regularly. We were hoping to meet up for dinner that night, but instead when I told her what happened, she told me which hospital we would want to go to, what doctors to ask for, and to call her if we wound up going there-- even in the middle of the night. Imagine the comfort that brought me! A friend, protector, advocate so close by! We were able to meet Tuesday morning over breakfast, and we all got in a couple rides together, before Melanie headed back for a nap and James, mom and dad went on the Star Wars ride again. I wished we'd had more time to talk, but I'm so glad we met!

I will be writing thank you notes to all of these people for their help, and if anyone would like to send me some thoughts and thanks to add, I'll be happy to include them. I only wish I knew who the two women were who helped in the first moments and held Melanie's hand. I hope they know we're all grateful. Praise God for His protection.

"for you have been my help, and in the shadow of your wings I will sing for joy.
My soul clings to you; your right hand upholds me."
Psalm 63:7,8

Random Kid at Disney Schooled by Tiger Mom

At Disneyland, a 5 year old girl and her grandmother were walking away from the Carousel ride in the Fantasyland portion of the park, when the little girl began to have a seizure. The girl showed no signs of overheating or overstimulation before the incident, and the parents, brother and grandfather of the girl were not with them at the time. Instead, they were waiting for the pair in front of the infamous "It's a Small World" ride (also in Fantasyland), after enjoying their time in "Star Tours" in the Tomorrlowland portion of the park.
Close to 6pm, the father received a cell phone call from a stranger using his mother's phone, telling him to come quickly to the Dumbo ride, as his daughter was having a seizure. The mother took off, dashing through crowds and jumping over stroller wheels. After circling around the Dumbo ride, she and the father finally spotted a group of people bending over the ground and shouldered their way through the gawkers.
After kneeling at her daughter's side, a man she'd barely noticed informed the mother that he was a doctor at a local hospital and that he had administered the midazolam. The seizure had ended by this time, and the girl was in the post-ictal state, which is the state of recovery immediately following a seizure, when the brain is trying to calm down and return to its normal state. A little while later, the mother noticed an unidentified woman holding her daughter's hand, and at unknown times and intervals, security men and nurses from First Aid arrived. The girl was put on oxygen, as she had turned blue for a few minutes during the seizure. After a short while, the mother was seated in a wheelchair, holding the girl- with oxygen mask- to be transported to the First Aid station. The bumpy ride woke the girl only slightly from her post-ictal unconsciousness, as the mother averted the stares from passers by. The security men walked in front of the group, yelling to everyone to step aside, as if alerting guests to a parade coming through. The mother related to her daughter that she should open her eyes to see the royal treatment she was receiving.
The girl was able to recover and awake on a bed in the First Aid station. After a time, as the family were about to leave, with the girl comfortable in her stroller, a nurse informed them that they should wait for a visiter who wished to see her. The girl was at this time awake but groggy and not in a happy mood. Moments later, in walked Pluto, and the girl's face lit up with groggy giggles. She most loved the tickley whiskers in her face. She got many high-fives, blown kisses and nose pets. Her brother also enjoyed the personal visit. The family posed for a picture with the orange character and thanked him for the visit.
The three females of the group headed back to the hotel so the girl could finish recovering in her sleep, while the three males stayed in the park to visit more rides.
Just after exiting the park, the women saw a young boy of 11 or 12 years crash into a trash can and fall to the ground. The boy's friends began laughing as the boy lay on the ground and began shaking, faking a seizure. The boy got up to walk on, but he had no idea there was a Tiger Mom looking on whose protective instincts were especially heightened. The mother of the girl walked swiftly over to the boy, held him gently by the shoulder, looked him in the eye and said, "My daughter just had a REAL seizure on the ground, and it was NOT funny. Please remember that." The boy clearly got the message and said, "ok." Then the boy's aggravated father walked up and said, "You wanna let go of him now?" The mother let go and said the same thing to the boy's father. The man changed his tone only slightly and said, "Well, I'm sorry about that." Then the girl's mother turned and walked away before the situation could continue any further. As they walked, the mother said to her mother-in-law, "Well, he won't forget that."
Thus, a boy, his friends and his family have now been schooled by a fierce Tiger Mom, protecting the dignity of her sweet Tiger Cub and all others who suffer from seizures.

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

Minnesota Nice

We had a successful trip to MN, with a happy report. I traveled with both kids and my dad up to the Twin Cities, for a neurology appointment and then an overnight EEG at Gillette Children's.
The neurologist was so pleased with how well Melanie is doing. She's growing and learning and developing, and she's only had four seizures since our last appointment, in July. She seemed quite interested in my report that after our last decrease in Yucky-Drug, before Christmas, I started to hear frequent pauses in her speech, along with lots of crankiness. She agreed with my hypothesis that she was having bursts of spikes in her brain (short seizures), and that those frequent blips would make her cranky. Well, what she found interesting was that those pauses improved when I added an extra 1/4 pill of Wonder-Drug in the mornings, and then they seemed to disappear when I bumped that up to an extra 1/2 pill. (I had made that change on my own, since she was out of town for the holidays.) So that tells us that Wonder-Drug has the capability to do the work of Yucky-Drug, without ANY apparent side effects. Yucky-Drug is yucky because it poisons just about everything: liver, cognition, appetite, growth, motor development, speech, mood.... did I miss anything? Yeah, it's pretty nasty. Oh, and too much of Wonder-Drug can make her blood too acidic, but her bicarb levels taken during our stay were almost better than normal, so we're safe there-- with room to go up if needed.

Well, the main reason I wanted this overnight EEG, besides the fact that she hasn't had even a short EEG in over a year and a half, was because I've been worried about nocturnal seizures. Most Dravet kids develop almost exclusively nocturnals at some point-- many develop them by this age. And I hear Melanie wake somewhat frequently through the night... Well, Dr. Wical came to talk with me in the morning, after reading her EEG (I think she's Superwoman- how did she read that whole thing so fast?). She walked in and said, "Her EEG looks rrrreeeally good!" She listed the few abnormalities/spikey bursts she saw, which were almost minuscule for a Dravet patient. And her nighttime waking is just that: plain old waking, not from seizures. Yay! That was the biggest comfort.

So Dr. W was definitely happy and ready to taper Yucky-Drug some more. We had already cut it down to half the dose she was on for so long, and now we have a schedule to cut it in half again over the next 6 weeks. Oh, I hope this is a fruitful and easy taper. I can live with a very low dose of Yucky-Drug if she seems to really need it, though I'd be thrilled to chuck it out the window. She's also on a teeny-tiny dose of Could-Be-Dangerous-Drug, and a smallish dose of Not-Sure-It's-Needed-Drug (which I continue to skimp on more each week or so-- trying to slowly nudge it out of the cocktail).

Dr. Wical is so impressed with how Wonder-Drug has been working for Melanie, that she said it will be her next Dravet treatment study. All because of Melanie! And she's ready to give Dr. Hecox in Milwaukee a huge pat on the back for his unconventional choice for Melanie two years ago. Wonder-Drug, by the way is acetezolamide (Diamox). Yucky-Drug is divalproex/valproic acid/epilim/Depakote.

Besides the drug taper, the plan is to add zinc and selenium to her daily supplements, and to continue with our trial of gluten-free. The jury's still out on that, but it takes a while for the gut to heal from gluten, so we're sticking with it for a while yet.

If there were a utopian hospital, I think it would be Gillette Children's in St. Paul. The people, amenities, everything... just superb. Almost hotel-like! And to top everything off, our morning nurse, Lance, did awesome Elmo and Tigger voices! Everyone loves to make Melanie smile and giggle.



Rawr! The EEG Lion!

 

Hello Dairy

Melanie went without dairy for about four weeks. The first three of those weeks were hard to decipher because she got a cold. That always knocks her back at least 2.5 weeks. Her lightbulb clicked back on last Tuesday, and she's been super chatty and a bucket of bubbles since then. I also choose to believe that her seizure in the wee hours on Friday, January 25 helped hit the "reset" button, so we've seen the usual language explosion after she recovered.
Combing through all the swings she's gone through in those weeks, we never really saw a measurable difference in her when she was casein free. Since casein should be completely out of the system in at least three weeks (probably more like some days), I figured we were seeing a pretty pure picture, once she had recovered from the cold. We gave her a "challenge" with a serving of cheese last Saturday, and waited and watched for three days. No changes whatsoever. So yay!! She can have dairy again! Whew-- I was getting sick of that. Milk is surprisingly hard to avoid on ingredient lists. Plus, I never really knew what exactly I was feeding her with the few dairy substitutions we tried. I may still try avoiding too much dairy in the evening. She might have been sleeping better when we first weaned it in the evenings.

She has been gluten free for a few weeks now, and we're still unsure of any changes with that. Gluten can take much longer to completely leave the system, so this trial might take a bit longer. It really has not been very hard at all. Thanks to Karen for sharing her all-purpose flour mix with me: it really was the kick-start I needed. Melanie loves Karen's bread recipe, the chicken nuggets I made were delicious!, and it's pretty easy to find suitable GF snacks. I've purchased a few boxed/bagged snacks, but mostly it's forcing us to go more simple, natural and healthy. I'm making popcorn regularly, she loves Beanitos chips, guacamole, nuts, seeds, carrots (she could eat a whole bag in one sitting), and now she can have the occasional string cheese. GF pasta is super easy, tacos and tonight's turkey burgers are easy- she never ate them in tortillas or buns anyway. I'm going to try a GF pizza crust some night... So many people are gluten free now, that it's not so hard to do at all. Labeling is convenient, finding entire aisles devoted to GF is common, and I wonder if some companies have just decided it's more profitable to remove any traces of gluten their products uses to have, because enough people would stop buying them otherwise. If we need to keep gluten out of Melanie's diet (and maybe mom will try it too?), I think it will be very doable. I know birthdays and such may be an issue, but I already have a plan for those.

I'm looking forward to any answers we may get in a couple weeks when we travel to MN for a neurology appointment and then overnight EEG. I'm suspicious of possible subclinical and nocturnal things happening. But if I'm right, please pray that we won't let that scare us into bumping up her meds more and losing the great strides she's been making lately in her development. If anything, we need to lower them more so she can come alive even more!

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.