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
Sunday, December 9, 2012
Sunday, November 18, 2012
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.
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.
Friday, November 2, 2012
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.
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.
Monday, October 15, 2012
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.
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.
Tuesday, August 28, 2012
She's in there!
It's strange how epiphanies in Melanie's treatment seem to rush upon me like a flood. In February we discussed with our neurologist tapering drug no. 2 a couple months after finishing a taper of drug no. 1. The first taper was successful. Then we waited to make sure our new rescue med would work before tapering drug no. 2. Well, the rescue med definitely works much better than the old one. But we found that out right after seeing the neurologist in July... When we had discussed completely weaning the tiny bit of drug no. 3 she's on. Well, I waited to start any tapering or weaning. Follow me so far? (I promise I'm sparing you the complicated details.) Oh, and we feel comfortable that drug no. 4 is her wonder drug and pulls most of the weight in her cocktail of seizure meds.
Then we went to the Dravet conference, where I got new and better ideas for the next steps in Melanie's care. After a pharmacology talk, I thought "hmm, maybe we can keep this small dose of no. 3, now that I kind of understand its mechanism. And maybe we want to do that taper of no. 2." As this thought was stewing in my head the week after the conference, all of a sudden there was talk in the online support group of the nasty side effects of drug no. 2 and how great people's kids are when they taper or wean it. "It's like someone turned on a light bulb inside."
There's the flood. Why did I wait? This is the drug I have felt uneasy about for a long time. It's the one that affects her appetite, weight, speech, gait, comprehension, mood, motor planning... And it depletes her carnitine levels so she needs a supplement that makes her urine smell like the penguins at the zoo, and it damages her liver so that she needs an expensive milk thistle supplement. Who knows, maybe it's even the main culprit in giving her reflux? I know all this from experience- our own and other parents', as well as the official reported side effects. Why did I wait? Why did I wait?
Last Thursday, five days ago, I decreased drug no. 2, which by the way goes by the name depakote, or valproic acid, or in other countries epilim. She's now on 5/6 of what she was taking before, and this Thursday it'll go down to 2/3 of the original dose. UMMMM...... 6+ new words within four days, some ending consonants on some words(!), multiple-word phrases, better appetite, better mood, slightly better motor planning, and... There it is.... The light bulb. It's still not as bright as it can be, but I'm going after that dimmer switch!
Praise the Lord! And I pray we can continue to decrease this drug safely. Our real Melanie is coming out, and I want to keep her.
Then we went to the Dravet conference, where I got new and better ideas for the next steps in Melanie's care. After a pharmacology talk, I thought "hmm, maybe we can keep this small dose of no. 3, now that I kind of understand its mechanism. And maybe we want to do that taper of no. 2." As this thought was stewing in my head the week after the conference, all of a sudden there was talk in the online support group of the nasty side effects of drug no. 2 and how great people's kids are when they taper or wean it. "It's like someone turned on a light bulb inside."
There's the flood. Why did I wait? This is the drug I have felt uneasy about for a long time. It's the one that affects her appetite, weight, speech, gait, comprehension, mood, motor planning... And it depletes her carnitine levels so she needs a supplement that makes her urine smell like the penguins at the zoo, and it damages her liver so that she needs an expensive milk thistle supplement. Who knows, maybe it's even the main culprit in giving her reflux? I know all this from experience- our own and other parents', as well as the official reported side effects. Why did I wait? Why did I wait?
Last Thursday, five days ago, I decreased drug no. 2, which by the way goes by the name depakote, or valproic acid, or in other countries epilim. She's now on 5/6 of what she was taking before, and this Thursday it'll go down to 2/3 of the original dose. UMMMM...... 6+ new words within four days, some ending consonants on some words(!), multiple-word phrases, better appetite, better mood, slightly better motor planning, and... There it is.... The light bulb. It's still not as bright as it can be, but I'm going after that dimmer switch!
Praise the Lord! And I pray we can continue to decrease this drug safely. Our real Melanie is coming out, and I want to keep her.
Melanie and Mommy singing one of our favorites! (with backup)
Sunday, August 19, 2012
In all things God works for the good...
We just left our new family. We traveled up to Minneapolis for a three-day international Dravet conference, put on by Dravet.org. There were 200 families and many of the biggest names in Dravet there, including Dr. Dravet herself. I've never been with a group of people with whom I feel more connected. Every table of new faces we ate a meal with proved to be fruitful and an instant connection. Amazing... People from all over the world, different personalities, backgrounds, ages... All fiercely in love with their children and always searching for better things for their suffering, yet happy children.
It was heartbreaking to see the other Dravet kids there, especially the older ones. I feel hopeful for numerous reasons, but last night my heart ached so bad for my little girl that I curled up in bed with her... until she literally kicked me out. At this point, I'm actually feeling most concerned for her posture and gait, but that has also renewed my hope and passion for ABM. I told a lot of people about that method and saw many of them take a pen to their notebook, so hopefully more children will benefit from it. I also acted as a cheerleader for diamox, our wonder drug of the last year and a half.
We learned a lot about Dravet syndrome itself, and all things that go along with it, tools to deal with issues, financial and future planning.... All useful knowledge, but so densely packed into three days, that we are now physically, mentally and emotionally exhausted. Praying Nick will survive going back to work tomorrow and the rest of the week.
We made some great connections and new friends which I know will last and continue to be extremely valuable. I hope we can go to the next conference in two years.
Romans 8:28
deo gratia
It was heartbreaking to see the other Dravet kids there, especially the older ones. I feel hopeful for numerous reasons, but last night my heart ached so bad for my little girl that I curled up in bed with her... until she literally kicked me out. At this point, I'm actually feeling most concerned for her posture and gait, but that has also renewed my hope and passion for ABM. I told a lot of people about that method and saw many of them take a pen to their notebook, so hopefully more children will benefit from it. I also acted as a cheerleader for diamox, our wonder drug of the last year and a half.
We learned a lot about Dravet syndrome itself, and all things that go along with it, tools to deal with issues, financial and future planning.... All useful knowledge, but so densely packed into three days, that we are now physically, mentally and emotionally exhausted. Praying Nick will survive going back to work tomorrow and the rest of the week.
We made some great connections and new friends which I know will last and continue to be extremely valuable. I hope we can go to the next conference in two years.
Romans 8:28
deo gratia
 |
| With the family who live just 2 blocks from Nick's grandparents in smalltown, MN. |
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| Melanie and Luella. Melanie's got a fat lip and nose from having too much fun with Grandma earlier in the day... |
Sunday, July 29, 2012
Didn't want to see that sunrise
For the short version, see the "Recap" secton below.
We just had a successful trip to MN, to get a second opinion in orthopedics and for a neurology follow-up. While mom, dad and Melanie were at our first appointment, James was with Grandpa and got pretty sick, with fever. He was down for two days, and we even took him to a clinic for a strep test. Next morning he woke up, mysteriously, completely fine. That afternoon it looked like we'd have to take Melanie to the same clinic for a UTI. Thankfully, after some running around, that was averted with a home test strip and mysteriously vanishing symptoms.
We arrived home last night around dinner time, and all was uneventful. Mom had an inkling, so we gave Melanie a dose of ibuprofen in case she might be coming down with a fever. 3:45am I awake to alarming breathing sounds and rush up to find Melanie in a partial seizure and very hot. Since laying her down always encourages the seizure to generalize into a grand mal, I brought her downstairs and kept her upright. She was sitting on the couch, feverish and shaking while we got ibuprofen and tylenol in her, and some water. Then, it decided to come anyway. So I gave her her first-ever dose of our new rescue med, midazolam, which is sprayed up her nose. The convulsions stopped within a minute. Then I believe she was in a non-convulsive seizure, but ambiguously slipped into her sleepy post-ictal state after not too long. The midazolam has an extremely short half-life, so it was working out of her system pretty quickly-- unlike the 24+ hours of diastat. So we got to see how she shakes it off, without being drugged. Her eyes are always out of commission in this state, and her jaw is clenched and mouth doesn't work well. The rest of her body is limp, but all of this slowly improves (while she's still shaking from fever).
New this time, since she wasn't druggy, about 15 minutes into the post-ictal state we heard a tiny voice attempt a word. Then a few words... But her tongue and mouth weren't quite functioning yet. When Daddy stepped in the room, with her eyes still closed, she said "haa Daddy." As I watched, I sensed that 'coming out of anesthesia' feeling, when you just want to shake it off and get up and move around. She tried and tried: moving, talking and even singing it off. "ooooOOWEEEEEOOooooh!" If James had been awake, he would have been laughing at her. It was confusing, sad, cute and funny all at once. This started getting annoying the more she came-to, and I was almost lamenting the loss of the diastat stupor, because we used to be able to put her back to bed right away and let her sleep it off for a solid night and late morning. Wellll, she woke up around 7:30. So we all missed church and took turns sleeping... kinda. Still exhausted.
Recap: Took a family trip to see the grandparents in MN for the week, beginning with an orthopedic appointment, then neurology. Both appointments went well. James was sick Tuesday and Wednesday with a relentless fever, but woke up completely fine Thursday. We got home Saturday night, and it seems Melanie may have caught that bug, and began with a seizure this morning at 4am. Our first trial of the new rescue med, which sprays up the nose, seemed to go well. The whole seizure, including the ambiguous ending and coming-to was much quicker than usual. Since the new drug has a 30-min half-life, she's looking pretty good today, just more wobbley and fighting off an illness. Mom and Dad lost a lot of sleep and we had to miss church this morning. We got back in bed after the sun rose.
We made it nearly four months without a seizure!
We just had a successful trip to MN, to get a second opinion in orthopedics and for a neurology follow-up. While mom, dad and Melanie were at our first appointment, James was with Grandpa and got pretty sick, with fever. He was down for two days, and we even took him to a clinic for a strep test. Next morning he woke up, mysteriously, completely fine. That afternoon it looked like we'd have to take Melanie to the same clinic for a UTI. Thankfully, after some running around, that was averted with a home test strip and mysteriously vanishing symptoms.
We arrived home last night around dinner time, and all was uneventful. Mom had an inkling, so we gave Melanie a dose of ibuprofen in case she might be coming down with a fever. 3:45am I awake to alarming breathing sounds and rush up to find Melanie in a partial seizure and very hot. Since laying her down always encourages the seizure to generalize into a grand mal, I brought her downstairs and kept her upright. She was sitting on the couch, feverish and shaking while we got ibuprofen and tylenol in her, and some water. Then, it decided to come anyway. So I gave her her first-ever dose of our new rescue med, midazolam, which is sprayed up her nose. The convulsions stopped within a minute. Then I believe she was in a non-convulsive seizure, but ambiguously slipped into her sleepy post-ictal state after not too long. The midazolam has an extremely short half-life, so it was working out of her system pretty quickly-- unlike the 24+ hours of diastat. So we got to see how she shakes it off, without being drugged. Her eyes are always out of commission in this state, and her jaw is clenched and mouth doesn't work well. The rest of her body is limp, but all of this slowly improves (while she's still shaking from fever).
New this time, since she wasn't druggy, about 15 minutes into the post-ictal state we heard a tiny voice attempt a word. Then a few words... But her tongue and mouth weren't quite functioning yet. When Daddy stepped in the room, with her eyes still closed, she said "haa Daddy." As I watched, I sensed that 'coming out of anesthesia' feeling, when you just want to shake it off and get up and move around. She tried and tried: moving, talking and even singing it off. "ooooOOWEEEEEOOooooh!" If James had been awake, he would have been laughing at her. It was confusing, sad, cute and funny all at once. This started getting annoying the more she came-to, and I was almost lamenting the loss of the diastat stupor, because we used to be able to put her back to bed right away and let her sleep it off for a solid night and late morning. Wellll, she woke up around 7:30. So we all missed church and took turns sleeping... kinda. Still exhausted.
Recap: Took a family trip to see the grandparents in MN for the week, beginning with an orthopedic appointment, then neurology. Both appointments went well. James was sick Tuesday and Wednesday with a relentless fever, but woke up completely fine Thursday. We got home Saturday night, and it seems Melanie may have caught that bug, and began with a seizure this morning at 4am. Our first trial of the new rescue med, which sprays up the nose, seemed to go well. The whole seizure, including the ambiguous ending and coming-to was much quicker than usual. Since the new drug has a 30-min half-life, she's looking pretty good today, just more wobbley and fighting off an illness. Mom and Dad lost a lot of sleep and we had to miss church this morning. We got back in bed after the sun rose.
We made it nearly four months without a seizure!
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