Aiden's Journey

One Little Boy's Battle with Dravet Syndrome

Medical Updates

5/18/13: I didn't update the site last year because it was an awful year. Aiden's seizures skyrocketed and he averaged 200-400 tonic clonics per month, often coming in clusters where he would seize every 10-40 minutes for 36-48 hours, day and night. We were exhausted dealing with these clusters at least every week, often twice per week, and his development regressed. We added meds, changed meds, tried medical marijuana, weaned all meds, developed and kicked a nasty benzodiazepine addiction, and spent countless hours in the hospital and ER to no avail. We had a feeding tube inserted to keep him hydrated and nourished during these clusters, and we are grateful for its help. HOWEVER, a supplement called N-Acetyl Cysteine, from GNC has helped reduce his clusters and we are able to function again. He is averaging 60-80 tonic clonics per month and we are thrilled! Most occur at night, with several random daytime seizures. We have had an issue with another family not wanting his service dog at school after an incident, and unfortunately he has been having seizures at school without his dog. We are working on the best solution for everyone and are constantly fighting this battle to keep him alive (first and foremost), safe (secondly), and happy. We are doing the best we can!

 

7/26/11: Aiden's been gluten free for a few months now and we think we see marked improvement. The tough part is that when his seizures come (and they're often in clusters of 3-4 spread out over 2-3 days now), there's often something dietary we can pin it on. We have added in dairy-free now, too, after a number of seizures following a milk binge. We'll see... 

4/5/11: I've been bad about updating this section - I apologize! VNS was implaned 4/7/11, and was ramped up each week thereafter. In the past two weeks we've seen much better control, though it could be due to the gluten free diet we implemented two weeks ago, too. I know it's not scientific to make two changes at once, but when you're desperate, it doesn't matter what helps - only that something DOES help. We see the neurosurgeon again tomorrow, as his VNS seems to have migrated upward too far and is now bulging out over his collarbone. It is looking like another surgery will be necessary to move it back down and re-suture it in place (I wonder what could have caused it to come loose: Certainly not two older brother.) Will update when we know more. 

3/25/11: We had our last consult before his VNS surgery in a few weeks. We feel good about the decision to implant it, knowing we are using every tool in our toolbox to help his seizures without making him a zombie again. 

3/20/11: It's been a rough spell seizure wise with increased seizures of all types. We have upped the Depakote and clobazam to no avail. We've been adding fat via almond oil to his ridiculously lean diet to help feed his brain and it seems to be helping, but the seizures are still too frequent (1-2 tonic clonics/wk, 2-3 complex partials in sleep requiring midazolam, and 3 days of flutters per week or so.) Midazolam is our newest rescue medicine and is such an improvement over Diastat. We syringe it into his cheek and wait for it to absorb, at which point the seizure stops and he comes back to life. He much prefers it for the semi-aware seizures where he can't talk and is dazed and out of it, but knows he doesn't want a syringe inserted in his rectum! So do we.  

2/16/11: Yikes, it's been too long since I've updated this section.  The MAD diet (which ended up at a 4:1 keto ratio, but with too much protein and too many calories) didn't fare so well.  We stopped just shy of 3 weeks due to zero improvement and general crankiness/lethargy/fluttering.  Since then we've had to increase clobazam again as well as Depakote, and spread the stiripentol out over 3 doses instead of 2 in response to increased tonic clonic frequency.  He has developed a brand new seizure (I thought he had them all!) in which his hand jerks up to his face quickly but strongly for a second.  He then examines it for a confused second as if to say, "How the heck did that get there again?"  It doesn't seem to bother him, but he does it even as he's drifting off to sleep - not good.  We'll see what neuro says on Friday. 

11/30/10:  Instead of waiting for the ketogenic diet appt in January (and then waiting another month for admission), we (read:  I) decided to try the modified Atkins diet one more time.  True to form, he jumped right into moderate ketosis (it's a struggle keeping him from going too deep) on Day 1, and wolfed down his eggs, bacon, and cream for breakfast on Day 2.  We'll see if I've learned enough to keep his fluids up, his constipation at bay, and keep him out of acidosis.  Wish me luck - I'm scared based on our previous two attempts at this, but more scared to not give him the best chance at relative seizure freedom.

11/17/10:  Last week we decided to try oxygen at night since Aiden's oxygen levels have been low for a few months (not helped by the altitude up here, but they were low even before we moved).  He'd been alarming 5-6 times per night for below 90%, resulting in less than ideal sleep for the entire house.  Since starting the O2 last week, he's slept through the night each time!  It's so satisfying to see him back up at 97-100% where he should be!  I can't say it's helped his eye flutters or myoclonics, but we are on Day 12 of no GTCs, so I can't complain.  We restarted his iron supplements to help his blood carry the oxygen more efficiently, and we have a ketogenic diet appt in Jan.  We're just not interested in more meds right now, despite his less-than-ideal control. 

10/1/10:  Aiden is plugging along on the same Stiripentol/Depakote/Clobazam dosages he's been on for a while.  He has a tonic-clonic every 8 days, but it is self-limiting, even with slight fever, and we're pleased with his speech development.  He has flutters and myoclonics at some point each day, but I think we've got a good balance of seizures and learning for now.   

9/10/10:  We contemplated starting ethosuximide in August after 2 weeks of nearly constant eye flutters and atypical absenses, but decided to wait it out. Sure enough, they attenuated on their own.  We're holding steady on a low doses of Stiripentol, clobazam, and Depakote.  We're happy to deal with a few seizures and have our alert, learning boy back.   

7/12/10:  Noting that his sz were mostly in the late afternoon, we split his stiripentol up into 3 doses, and it seems to have warded off the big ones for the past couple of weeks.  Yay!  To get rid of the daytime atypical absenses and eyeflutters, we cut out all his juice (replaced with milk), cut out his vitamin and any gum stashes he was getting into (aspartame ridden), and things seem better so far.  Not perfect, but if we could keep it at this rate we wouldn't contemplate the ketogenic diet.  We'll see!

6/14/10: After 2 months of increased seizures with unknown cause, plus some days full of eye flutters, we were about to increase the stiripentol when we thought, "Hey, why not decrease the meds instead?  Things can't get much worse.."  So we did, with the neurologist's blessing, and the daytime flutters/zoniness has been better.  The GTCs are still every 7-10 days, but are still self-limiting, so we are comfortable.  His activity level has perked up and his speech is improving.

5/11/10:  He did end up with two self-limiting GTC's at 6am with that last illness.  He also had an unexplained GTC while eating the other day, making 4 in the past month, which is a little high but since 2 were with illness, they shouldn't really count.   His overall seizure management is pretty good! The smaller ones are not as pervasive as previously, and his speech is coming along nicely.  He's also not exhibiting as many crazy behaviors as he has in the past - his attention span and interest in activities is definitely longer than it was last year, or even this past winter. His walking has improved dramatically in the past 3 months, due to a combination of heavy PT/exercise, Clobazam reduction, and his orthotics.  He's able to walk across parking lots now and is actually bruise-free at the moment!  (He used to have permanent bruises on his forehead from stumbling or falling.) 

4/29/10:  We did bump up the Clobazam and have seen slight improvement.  We also increased his STP to 450mg morning, 400mg night.  He currently has a fever and has not had a big status GTC yet, though he was in NCS for a while last night before the fever spiked.   

4/6/10:  He's been doing well on lowered Clobazam, but we may need to bump it up slightly - he seems on the edge in regards to the atypical absense/myoclonics.  Many days are good, but he's having trouble with excitement.  He's not as tired during the day and has lately been attending all 2.5 hours of his school! (For the past year he's been exhausted after about 90 minutes and needed to go home). Appt with his neurologist on Monday may tell more.. 

3/16/10:  Aiden's doing well sz-wise, and we've lowered one drug to help with his exhaustion during the day.  Sleep continues to elude us... He's been spending lots more time outside, but we were reminded he is not out of the woods yet at an overstimulating, poorly fluorescently lit Pump It Up Sensory night tonight.  You can't win them all!  Or at least not all at once.   

3/1/10:  He's been wearing his orthotics for about a week and a half now and is much more stable in them for the most part.  He can stand still without falling down, but when he gets tired he sort of "gives up" and then the effort of walking in them becomes difficult.  He's had a lot more eye flutters and zoniness this past week for some unknown reason which we're trying to figure out.  Still pretty good overall though.

2/13/10:  No news is good news :)  He's doing extremely well seizure wise - we can't complain at all in that department.  He's having a lot of trouble sleeping due to the stiripentol, but it's still so wonderful to see so few seizures that we'll take it.  He was cast-molded for orthotics on Wednesday, and they will be ready this coming Wednesday. He picked blue plastic with cars on them and thinks he's a soccer player when he wears them.  He also started using an abdominal binder for better trunk control and he loves his "belt."  I haven't taken him out and about to see if it helps his balance yet.. time will tell!

 1/31/10:  Today was Aiden's first day without Keppra since he was 12 months old!  To celebrate, he went on a walk with Andy and the dogs.  He walked, on his own, without his glasses, for nearly 3 blocks, and was even in charge of Lexie.  He had a lot of eye flutters and zoniness yesterday, but today more than makes up for it.  He is doing well overall, and we could not be happier to be back down to 3 anticonvulsants, rid of one that has possibly plagued his development for 3 years!

1/22/10:  He's still doing well.  He had a GTC on Tuesday, but he had a fever that day, so it is understandable.  That was the first one in over 4 weeks!  Thanks to stiripentol, it was short (a minute or so) and he came out of it on his own.  Before STP, his seizures with illness were the ones that had status epilepticus potential.  He's having a little bit of a tough time recovering from either the seizure or the illness (we can't tell which), but is still doing well overall.  He started physical therapy again this week, a good sign that we are confident enough with his seizure control to start trying to chase the other beasts that come with this condition!  Since exertion and movement are seizure triggers, we don't even attempt PT while he's unstable.  He was certified disabled last month and is now on MN Medical Assistance, which should help cover more therpies and give us some respite/nursing assistance. 

 1/13/10:  He's doing really well!  We're halfway through the Keppra wean, and he has rough patches here and there, but overall we're very happy with Stiripentol so far.  We just feel so blessed to have been able to try this drug and succeed so far, and are so happy with the results.  We're even branching out a little more - Aiden spent an hour at school without his glasses on so that his aide could actually see his eyes on Tuesday.  Hopefully this will continue! 

1/3/10:  Aiden open mouth kissed or tried to eat a lit, hot lightbulb last night and wound up with 2nd degree burns in his mouth and on his lips last night!  Luckily, they don't think it's going to cause permanent scarring and whatever they put on it in the ER is helping already.  He is so busy these days, he's a hazard to himself.  It's wonderful to see him so active and curious, but he has absolutely no sense of self-preservation.    I honestly cannot keep him from getting into the bleach, the lightbulbs (?!?), or the other unknown dangers every second of the day...   On a good note, we are continuing to wean his Keppra, and he had a pretty good week overall last week!  A couple of eye fluttery patches, but busy, alert, and good for the most part.

12/28/09:  He's had a number of pretty good days in a row now.  He still has bad periods, but hasn't had entire bad days, so that's good.  We are beginning to wean his Keppra (we've been trying to get rid of this one for over 2 years!) 

12/20/09:  Aiden is still alternating between good and bad days, though the pattern is a little blurred.  He had two particularly bad days on Thursday and Friday, with near-constant eye flutters and atypical absenses that didn't stop despite multiple doses of Diastat on Thursday, culminating in a tonic-clonic on Friday at Alec's tennis lesson.  Bad mom that I am, I had used so much Diastat in the past 2 days that I forgot to replace it in my purse, and panicked with no rescue meds and called the paramedics at tennis.  Fortunately he came out of it on his own! Once that seizure was out of his system he seems to be doing better.  Who knows? 

12/12/09:  We don't understand it, but for the past 10 days Aiden's been alternating between good days and bad days.  It is like clockwork:  One day will be bad, the next good, the next bad..  No matter how we try to replicate the "good" days, we can't figure out why he's falling into this pattern.  He had a big tonic clonic seizure on Thursday night, but it was precipitated by a fall halfway down the stairs, so it was understandable, and was only 2 minutes long. 

12/2/09:  He's been GREAT seizure wise since Friday (6 days now), so I think it's safe to say he was just sick last week, and that's why his small seizures were bad.  They always are worse at the start of an illness.  And he was diagnosed with an ear infection last night - yes!!  This is his first ear infection without a tonic clonic seizure in over 18 months.  I was scared to post an update for fear of jinxing it, so if you have an extra minute a quick prayer for continued success on Stiripentol would be greatly appreciated.  I am absolutely floored by the difference it has made in his alertness, speech, and happiness.  He smiles!  And he's so darn busy again! 

11/29/09:  Well, after last week's update he took a nosedive and his atypical absense/myoclonics came back with a vengeance.  We were extremely disappointed and frustrated by full days of continuous seizure activity again.  But on Friday he seemed to snap out of it and has had 2 great days again.  He did have a runny nose and congestion this week, and his seizures are always much worse when he's sick, so we're hoping that's all it was. Who knows?  Maybe his new medicine regime kept any tonic clonics due to his illness at bay.    

 11/21/09:  Aiden has had a great week overall!  He is much more alert since the clobazam reduction, and his eye flutters/atypical absense/myoclonic seizures are still reduced by more than 50% (likely closer to 90%) since the introduction of stiripentol.  He has periods of them every now and then, but it is not all day every day.  In fact, yesterday he had none that I saw and we went to dinner as a family!  We may be in the "honeymoon" stage, where kids react well to new meds but the effectiveness wears off, but regardless I am grateful for its introduction.

 11/18/09:  Insurance has verbally agreed to reimburse us for the Stiripentol!  This isn't so much of a medical update as a blessing and victory..  I'll post a blog at some point about it, but wanted anyone interested to know the good news. 

11/16/09:  We met with Dr. Wical, Aiden's neurologist, today.  She agreed that he looks very sleepy and lethargic, and we are reducing his Clobazam from 15 mg to 10 mg.  Hopefully as he re-enters the land of the living his seizures will remain at bay.  Stiripentol has been working seizure wise until yesterday, when we pushed him a little too much with excitement and family.  If his seizures are still reduced after the Clobazam reduction, there may be a Keppra wean in our near future.  We've been trying to wean that one for nearly 2 years!  She also shared some sobering new statistics about SUDEP/unexplained autonomic issues and Dravet Syndrome, which places Aiden in a very high risk category based on his age and previous possible autonomic/disautonomic and cardiac events.  He had a recent normal EKG, but that test "barely scratches the surface" of heart testing :)  She has ordered a Holter Monitor (a continuous heart rhythm monitor with a number of leads stuck to his chest) for 30 days to see if he is having any events we are not detecting visually or with his HR/spO2 monitor at night.  He'll also be having another PT evaluation after his Clobazam reduction. 

11/14/09:  For those of you following the stiripentol trial, it is going well so far.  Today is his 5th day, and his seizure activity has decreased dramatically.  He is having only a few eye flutters a day, no atypical absenses, and no myoclonic jerking (except while sleeping.)  Unfortunately, he's drunk as a sailor!  He can barely walk, his speech is even more slurred than usual, and he is extremely floppy and lethargic.  (He slept through the night for the first time in months.)  We see his neurologist on Monday, and she may advise us to wait it out while he builds up a tolerance, decrease one of the other meds (stiripentol increases the blood serum level of several of the others, and even though we've cut those in preparation, they may need to be further cut), or decrease the STP.  It is always a balancing act, and we will keep tweaking this until we get it right.  I am extremely encouraged by the fact that his seizure activity is down so much, since most of the other meds have not touched his atypical absenses AND made him drunk :) 

11/11/09:  Aiden started Stiripentol yesterday.  There's nothing like learning the best way to administer a new type of med!  So far, so good..  He has been a little better yesterday and today, but it could be a fluke like the two good days last week.  We're hopeful though! 

 

11/3/09:  Aiden is having a rough time lately, and we're trying to help get him back on track.  His atypical absense/complex partial seizures are through the roof right now, and he's extremely light/movement sensitive.  For the last 5 months he's been getting progressively worse despite varying diets and a two trials of Felbatol.  (He's currently on Keppra, Depakote, Clobazam, and Felbatol).  We're switching back to the name brand of Depakote and are about to start a trial of Stiripentol.  Stiripentol (along with valproate (Depakote) and Clobazam) is the only treatment proven effective in helping reduce seizures from Dravet Syndrome.  We've held off this long because it is extremely expensive and only available from one manufacturer in France.

They are shipping it out today, so hopefully it will clear customs in the next two weeks and we can get started.  It is not a cure, and doesn't work for everyone, but we are hopeful!  Now if we could just get insurance to pay for it...