The Ups and Downs of Doose

This blog is long overdue for an update. Ethan continues to have several ups and downs. He'll be great for a period of time, then we'll find ourselves right back where we were with several daily seizures, and watching our child decline. We actually had a very good run. Once we got his dose up on the clobazam he was mostly seizure free for a while. He would have a bad day mixed in here and there, but it was a marked improvement. But slowly but surely we started to see the typical path that new medications always seem to take. Small jerks came back, then drops, then tonic clonics. We would always try to find cause, maybe it was his little cold, or maybe he just had a bad day. But they came more frequently, and eventually we were back to daily seizures, and multiple tonic clonics a week, sometimes more than one in a day. We called and spoke with his doctor and they upped the Clobazam even more, and we were informed he's over the maximum dose on both the medications he's on, and that would be the last increase we could do. We took it for what it was, and continued on hoping the increase would do it. Unfortunately it didn't. The seizures returned full force just a few days later, and we are now stuck here.

Ethan also had a new MRI a couple weeks ago to check to see if they could see a reason for his central sleep apnea, and also to just general check things and compare to his last one. His last MRI was completely clean, and I found myself in limbo between wanting another clean MRI, and wanting them to find something, almost hoping that if they found something they could somehow fix it. The procedure went well, and besides Ethan being angry and groggy after waking up he did fine as well. We got the results a few days later and it was in fact clean, with just some sinus swelling. So that was good/bad news I guess! It leaves him still with no physical reasons for his seizures, and no obvious reasons for his central sleep apnea. As has been the case the whole time, we're left with no reason for this all.

Waking up after the MRI

With his health once again declining his neurologist has been pressuring us to put him on yet another drug called Felbatol. It is a risky drug, and more or less used as a last resort. It has a high chance of side effects, and requires a whole battery of tests before starting it, and blood tests every other week while on it. It also statistically has the same chance of killing his as he has of having Doose in the first place. We are required to sign a waiver for him to even be put on it. We really don't want him on it. After 12 drugs and the ketogenic diet we kind of figure that magical dangerous drug number 13 isn't going to do it, but they have informed us that they will not pursue surgical options until this drug has been marked off the list, and it's either try it or we have no other options for you. So we are begrudgingly putting him on it. Of course there is a chance it will work, but after so many fails you become very skeptical, especially when there is such a risk to it.

Snuggling with his Rhino!

If all this wasn't bad enough, one of my worst nightmares became a reality this week. We have the typical kiddie pool set up in our back yard, a little pool with a foot and a half of water, and and a slide going in. I was laying in a chair about 15 feet away watching the kids play. Ethan was at the top of the slide about to go down when he went into a tonic clonic seizure. I watched him fall down the slide and end up face down in the water as I ran to him. When I got there he was face down and seizing. I grabbed him out by the arm with one hand, as I dialed 911 into my phone on the other, not actually making the call yet. I got him out and he continued to seize for another minute or so and when he came out of it he coughed up a ton of water. I took him inside to dry him off an watch him, and put in a call to his doctor to make sure everything was ok. They said since he did cough up so much water, and was acting fine that he probably got it all up, but to call back if we saw any changes. It's definitely one of those memories, just like his very first seizure, that is burned into my brain. Just him face down in the water flailing. I can't get it out of my mind that if I had my back turned, or was farther away, or had stepped inside for a minute that he could have drowned. It was horrifying, but I'm very happy it did all turn out fine!

Handsome boy!

Our big boy also turned 5 at the end of April! I can't believe he's already 5, who knows where the time goes! He started telling us months before that he wanted a birthday party at Chuck E Cheese, so off we went to every parents favorite place.... It didn't matter, he deserved the best day every, and we would have given him anything we could. But on his actual birthday he got to open his present from mom and dad, a firetruck! I love that we can get him gifts that can secretly be used for both him and Taylor:)

My yearly picture of him in bed in the morning!

So excited!

His firetruck with sissy riding shotgun, and Jet acting as the fire dog:)

The day of his party was 2 hours of chaos, but the smiles on his face were so worth it! He had a blast! Just hope he doesn't want to go back next year:)

Blowing out the candles on his Spongebob cake!

Having a blast! http://www.youtube.com/watch?v=WdQuO5LuKWE

And shortly there after, and much too soon for me, school was out. Ethan graduated preschool!

That's pretty much what has been going on with Ethan over the last couple months. We do have something big we're looking forward to though, we are going on vacation at the end of June, and Ethan is finally going to get his feet in the sand!!!! It has been his wish to go to the beach for years now, and thanks to Kate, and everyone that donated that is going to be a reality! We're not stopping there though, we're also hitting Disney, Legoland, and Seaworld while we're there! Ethan can't stop talking about going to Mickey's house, and Taylor won't stop mentioning Pooh Bear's house! They will have a blast! I can't wait to go to Legoland. Of course it will be fun for the kids, but I grew up in the town it's in, so it will be fun to go back "home" after being gone for so long, and see how things have changed:) It's going to be a blast, and we can not wait!!!!

In one last bit of news we have been working on Jet's training full force. Over the last few months he has gotten so significantly better! I'm amazed at some of the things he can do, and his behavior is fantastic! He even passed his public access test last week! I will eventually get a DVD of the test, and will share it here. Just for fun we tried to get him to open a handicap door, and he got it on his third try! I haven't trained him for any tasks like that, so I was impressed. So I'll leave you with some fun doggy training pictures:)

He really is a water dog!

Don't mess with Jet, his best friends are all German shepherds:)

 They're all having hot dogs thrown at them, none of them touched them:)

Pretending he's a shepherd at training.

Video of Jet working on scent training: http://www.youtube.com/watch?v=KwyquhEzjf0

Please take a second to check out Love My Canine. I don't think we would have gotten half as far as we have without their training and support!
http://sites.google.com/site/lovemycanine/Welcome
https://www.facebook.com/media/set/?set=a.460777783949077.122315.100000505770490&type=3#!/pages/Von-Alpenmac-German-Shepherds/144522108973685

Updates

It's been quite a while since I've updated things here. Once we got home and settled from the last few hospital visits we upped his clobazam up to higher dose, and slowly but surely Ethan seizures started to go away! The drug is working! This isn't to say he is having no seizures. We still feel occasional jerking, and without looking into his brain with EEG there is no way to know for sure. He has had 2 tonic clonic seizures in the last month. These are the typical seizure you think of, the ones you see on TV. His normal tonic clonics do not stop. They are violent and long, and last upward of 6 or 7 minutes on average. Since he's been on the Clobazam at the higher dose, he will go into one but once it gets to the bad point it just kind of fizzles out and he comes out of it. I'm obviously not a doctor, and don't know exactly what the drug is doing, but it seems that it is stopping them from taking their full course and getting out of control which is amazing! His quality of life has definitely improved. Our days don't revolve around seizures at this point, they revolve around Ethan being a normal (mostly) little boy, playing with his dog, playing outside (because it's nice out!), going to school and playing with his friends, and of course bickering with his little sister:) Two big seizures a month is no big deal when you've become accustomed to 100+ a day! We are also finally, after a year and a half, weaning him off of Depakote!!!! It's a nasty drug with bad side effects, and he's been on an ever increasing dose for over a year. It has never made any difference, but it's a front line epilepsy drug that often works well so they have been reluctant to take him off it. He's currenly at only 250mg a day, down from 750mg a day with no increase in his seizures. That leaves him on only 3 drugs so this is good news!

So that is the good news, and mostly the end of it. During our last neurology appointment we brought up to our doctor that when Ethan is sleeping he does this weird breathing thing. Basically he'll take a big breath or two, then not breath for maybe 15-45 seconds, then catch his breath and do it again. We've been noticing this for maybe the last couple of months. Also when we were in the hospital the last few times he would have several de-stats through the night. Doctors would always check him and say yes, he is dropping his stats, but seems stable. She agreed to order a sleep study basically just to shut us up and get us to stop talking about it... When we got the call to schedule it, it was booked all the way out to May! We had them put us on a cancellation list, and last Tuesday morning we were called and asked if we could come that night, of course we said yes even though it meant Ethan would miss one of his last 2 days of school before 2 weeks off for spring break... That's a LONG time for him to not be in school:)

Loving his doggy.

We got up to the sleep lab in the evening, and I saw this as a perfect opportunity to have Jet stay the night at the hospital for the first time. This was a planned short visit, just perfect for him to get his feet wet! We've been focusing much of our training with him on accepting strangers. It's typical for people not to walk up and pet a service dog, and Jet ignores everyone when we're out in public so it's not always seen, but he really doesn't like people. He is nothing like a lab who happily comes up and wags his tail to be petted, he stands away and ignores people, and if you approach him instead of him coming up to you he backs away. The trainer we work with has suggested that unlike most working dogs, we actually encourage people to come up and pet him and give him treats, so while in the hospital we were working on it. While in the waiting room of the sleep lab there was another little boy there, maybe 7 or 8 years old. I was beyond shocked when Jet got up and walked over to this little boy and set right in front of him and licked his face. This is so far away from his normal behavior. He cuddled with this boy and let him love all over him, not much different than he does with Ethan. It turns out that this boy has autism, and somehow Jet picked up on something that told him he was needed there. His mom told me he was completely non verbal until they got a dog that totally brought him out of his shell. It's really amazing the things dogs can do just with their presence!

Pics of Jet at training.

Once they got him back they hooked him all up. They use some EEG leads, then put leads all over his body and face, and tape oxygen tubing to his face. He's such a trooper. No child I've ever met his age would sit there and not complain for an hour while his entire body was wired up and wrapped up. He's such a good boy! Jet fell asleep on his bed, and eventually Ethan got to sleep as well. When he woke up in the morning the tech came to unhook him and told me he needed oxygen in the night. I asked why and he told me he really couldn't tell me, only the doctors can go over results, but "not to worry", and "it's often normal".... I should know better by now then to trust those words.

 Jet hanging out with Ethan!

Not 20 minutes after leaving the hospital I got a call from a nurse. She said they still couldn't give me any results, but it does seem that Ethan does need to be on oxygen and we would be getting a call from a health care company to set up a delivery. I was told he needed to be on it at night, or anytime he was sleeping... But no one wants to tell us why! The health care company came and delivered everything, one huge oxygen canister, and 4 smaller ones for travel and gave us a quick run down on how to use it. He actually had slightly more information than we had been given. He said all he knew is that his oxygen had gotten as low as 83% during the night. Anything under 93% is considered dangerous. So there we were. For the next week we called every single day, multiple times a day to try to get answers as to why we had him on oxygen, and no one could tell us anything because certain people have to sign off on results, but those people can't give results, yadda yadda yadda... We were trying to figure out how to stop him from strangling himself on the tubing. Things got really bad when Mike was woken up in the morning to him choking. I was out in the living room awake already. The tubing had slipped off his nose and around his neck and somehow his legs were pulling down on the tubing and he was choking. Thankfully Mike did wake up and was able to untangle him. It took about 15 minutes before he was breathing well again. That was the end of it, we weren't putting that thing back on him until we knew if there was more of a risk of him not breathing at night, or choking himself.

The next day we did get more answers, but still not all of them. His average oxygen level while sleeping was between 90%-91%, with drops down as low as the 83%. Also he was doing the breathing thing we had been seeing. He was doing this approximately 5% of the time, and in children normal is considered less than 1% of the time. The people in the sleep lab suggested he has a new MRI (his first was done after he had had maybe 5 seizures, and a year and a half later he's had over half a million so I imagine some damage could have been done), and we see a pulmonologist. So finally, 2 days later, our neurologist finally felt she could pick up the phone and call us back. She calls just to tell us there's nothing we can do and we need to call our pediatrician to go over the results. Mike asked her about repeating the MRI and she said even if it did show damage there's nothing we can do about it so why repeat it? Hmmmm, maybe it would be good to know? Obviously our frustration with her is very high right now.

Our amazing pediatrician got us in last minute yesterday. She told us if anything like this ever happens again, and for a week we are sitting with no answers we are to call her and she will take care of things. We're so lucky to have her for a doctor. She randomly calls out of the blue just to check on Ethan, and always makes time to see us even though she has a very busy schedule. She went over the rest of the results with me. Yes, his results were abnormal, and his O2 was very low during the night. Even in her office it was only at 93%. He also had irregular breathing quite frequently during the night. She described it much the same as the way newborns breath when they sleep, breathing fast, then slow, then pausing. This is not normal past the newborn stage. She also said there were frequent periods of apnea, and the type he was having was coming from the brain. She said he absolutely needs a new MRI, no questions asked, quite a difference from our neurologist! They need to see what changes may have happened in his brain that might be causing this. She is getting the MRI set up, and getting us into a pulmonologist. She kept apologizing for how we had been treated and said it was unacceptable! We just love her:)

So for now I guess that's where we are. He has to stay on the oxygen for an indefinite amount of time now. That's just his luck though I suppose. They say any seizure under 5 minutes is completely benign, but Ethan's aren't typically that short. He's had 10, 15, even up to almost 20 minute long tonic clonics. Not only that but he's been in status for days at a time before. I worry what damage it has actually caused, and I guess we will see soon with this new MRI.

And just for anyone that doesn't know, March 26th is epilepsy awareness day. Next Monday wear purple to honor those living with epilepsy, and spread awareness! And I leave you with an adorable squishy puppy pic, and one of spring fun in the warming days to brighten your day:)

One of Hugh and Angela's puppies!
(Hugh helps with Jet's training, and is a breeder of amazing working line German shepherds, check them out! https://www.facebook.com/#!/pages/Von-Alpenmac-German-Shepherds/144522108973685)

UPDATE 5:45PM -  Things quickly turned bad this afternoon. He had about a dozen violent drops, followed by a long and violent full out tonic clonic. He still isn't acting quite right. Let's hope this was just a fluke, and not a change:(

http://www.youtube.com/watch?v=K3NRrzqhLGc

Jet comforting Ethan before it got really bad.

Back to Square One...

Things have continued to go downhill the last couple of weeks. It started small, he would have small jerks here and there, full out myoclonic-astatic seizures maybe once or twice a day, and tonic clonics every week or two. Then they got more violent. His drops are knocking him to the floor, his myoclonics are quite literally throwing him back two feet, and tonic clonics are coming every couple of days, and sometimes multiple times a day. We have been waiting so long for Clobazam and we are finally on it, but in the almost two weeks he's been on it he's gotten worse every day. We ended up in the ER twice last week, and one of those trips found us being transferred via ambulance back up to Children's 70 miles away.

They were so sweet, they turned on the lights for him:)

We arrived in the ER and they put us in a room and basically ignored him. It wasn't until he started clustering that they were paying him a little attention. The ER doc came in and started to tell me how they were planning to watch him for a little while then send us home because there was nothing they could do. Just then Ethan went into a tonic clonic and in a flash the room was filled with about 3 doctors and 5 nurses. It's amazing that the seizures we deal with on a daily basis alone require half a dozen hospital staff and oxygen to deal with. The seizure lasted about 10 minutes and was quoted as being "impressive". Right when he started to come out a nurse picked him up and ran him down the hall to the front room where ambulances drop off the most critical cases. He was put on oxygen and cardio leads and once again, we were dropped and left alone. More than an hour later the same ER doc came in to tell us again that neuro wanted to send us home because there was nothing they could do. Mike called the neuro department and talked to them and magically 20 minutes later someone was coming down telling me they had a room for us upstairs.... Just to add, he had Ativan given just a few hours prior at the other ER, and they gave it to him again during his long seizure. He was pretty drugged up at this point, and it still wasn't stopping the seizures.

Waiting to go up.

We got up to our room and got settled in for the night which was pretty quiet, no real obvious seizures to me. The next morning the nurse told me he wet the bed and she changed him out in the middle of the night. This was somewhat worrying to me. He very rarely wets the bed, and when he does we sort of figure it was a seizure. But he had an apple juice before bed, so maybe it was just a fluke. I wasn't sure yet.... The seizures started coming back in the morning. Only now he was having a brand new one. It looked somewhat like an atypical absence seizures, but he would make a funny noise.... And wet himself which he has never ever done during a seizure. That day he had 4 of these episodes where he would wet himself. Neuro was still completely ignoring us. They told us they didn't want an EEG on him because they knew everything about his epilepsy... REALLY???? This was news to us. Sure wish they would share this info with us. Even with a brand new seizure they still said they knew all they needed and he wouldn't be getting an EEG even though in the last month his seizures have increased from a few a month to up to a hundred or more a day.

Still doesn't phase him!

Our frustrations were rising by this point. We were once again pretty much ignored for the rest of the day, and for the first time in years I had to put a diaper on my child to sleep in. The next morning he woke up and the seizures started and did not stop. He was having atonics, myoclonics, and absences back to back to back. He seized for 2 hours straight. I told every doctor on the floor, every nurse, everyone. I told them I needed someone from neurology down immediately and no one came. He quite literally had 200 seizures in 2 hours before an order was finally put in for Diazapam. Having an IV they brought in that form. Of course they go to give it and his IV isn't working. Great. So they ordered the rectal version that took another 20 minutes to be sent up. They gave it to him and he had 2 more seizures then they stopped. Nearly 2.5 hours later they did what I would have done instinctively hours ago. Someone finally came up from neurology and apologized. I was told his primary neurologist was out until Friday, and basically they wouldn't touch him without her telling them what to do. Well great, that's very helpful. Another pediatrician came in and apologized for how everything went. She said he wasn't continuously seizing for more than 5 minutes at a time and the order is to give diastat after 5 minutes... No, the order is 5 minutes straight or more than 6 in an hour. He was having 6 in 10 minutes. I was so fed up. I told them the diastat keeps his seizures away for about 72 hours and since they wouldn't do anything until his neuro came in in 2 more days I wanted to go home. There was no point staying there and keeping him trapped in a bed when we could watch him in the comfort of his home.

myoclonics and absences. They started small and not too frequently, and hour by hour got worse. Last night he was up all night seizing. It is very rare that he has seizures at night, and he was having clusters every time I woke up last night. He got up this morning and it's been non stop. If I told you my child had been up since 6:00 in the morning, and by 7:30 he had over 50 seizures you would probably think I was crazy. That's been our morning. We are living off rescue meds. It seems his regular 3 prescriptions he takes 3 times a day, 13 pills a day  are doing nothing. It's great that the rescue meds stop his seizures. The problem is eventually they won't because his body will get used to them so you're constantly questioning when he really needs them. To make matters worse we had a blizzard yesterday and we're literally snowed in. If I needed to get him to the ER I couldn't. I actually just said to Mike that I should probably hop in the shower and pick up the house in case I need to call an ambulance today. It's ridiculous to even have to think that way. The frustration, stress, and anxiety are very high right now. We're just hoping there is something positive laying in the near future for us.

The type of seizures he's having this morning. And yes, I give him cookies at 7:00 am!:
http://www.youtube.com/watch?feature=player_detailpage&v=39SAUg7JY_M

Rough Couple of Weeks

Ethan had been doing VERY well seizure wise. We actually went 4 weeks and a day between tonic clonics which is a new record! But after that he had 2 more within a week. Then we starting seeing the small jerks coming back. These are the same small jerks we starting seeing a little over a year ago. We had no idea what they were at the time as he was still only having tonic clonics then. At that time over the span of about a month those small jerks turned into clustering jerks, then clustering with loss of consciousness, then to almost non stop seizing all day. Unfortunately in the last weeks we have watched those small jerks come back, and now evolve into full myoclonic-astatic seizures with loss of consciousness. Basically they start with a drop seizure which has been knocking him to the ground which wasn't the case before, then the drops keep coming over and over, at this time it's about 10 in a row. Then it's followed by about a minute long absence seizure. He's unconscious the whole time. These have been happening several times a day now. The path things are taking is so similar to what happened this time last year, and I'm very fearful that we will end up where we were then. We were literally in the hospital every other week for a week at a time. We have an appointment on the 23rd with his epileptologist so I guess we'll see what the next move is then. It seems they always just want to throw more drugs at him. But these are the same drugs he's been on for a year+ that have never worked. I don't really know why they think increasing doses will do anything. I suppose they feel they have to do something, but it's very frustrating. There is a new drug that was recently FDA approved that we will be trying out though that's called Clobazam. It has actually showed promise in other kids with Doose, so we're hoping for the best!

 

Ethan has also completed his first 2 weeks of school! He loves it! He has a personal aid that says with him all day to keep him on track, and watch out for medical issues. It seems to be going really great so far! He shoves me out the door when I'm dropping him off because he can't wait to get in there and have fun! He seems to be adjusting well, and working on making new friends. He does have to wear a helmet when he's outside playing, but he doesn't seem to mind, and the other kids don't seems to notice. I guess that's a great thing about 4 year olds, they don't notice differences in kids yet. Taylor and I also have a great time when he's in school! We like to go out every day to play and run errands together. She misses so much one on one time because of all of Ethan's medical problems, so it's nice for her to have some time.

Ready for his first day!

 

So hopefully where the seizures are now does not get any worse, and we don't go down that road again. All we can do is try to stay positive, and realize that there will be ups and downs on this path!

Just because it's funny, can you see the squirrel he was trying to get??? He's going to have to be faster!

Happy Birthday Mr. Jet Dog!

*Warning, tons of Jet pictures to come:)

Jet is one today! We are so thankful to have him, and he's such a good boy! He has given so much to Ethan since we have had him. Above all else he's an amazing companion to him, and gives him someone who will always be there to play with him, and snuggle with him. Just within days of having him we saw a decrease in Ethan's seizures. As a 16 week old puppy he instinctively knew to be calm and relaxed around him. Ethan is prone to seizing when he cries because of hyperventilation. He would cry then have several drop seizures. Once Jet was here he would call out to Jet whenever he would start crying and he would come and lay with him settling him down and stopping the seizures.

His first week home.

From the beginning he showed himself as an extremely calm and gentle dog, not what you would imagine a puppy as at all. He never ran around crazy and jumped up on the kids. Instead he would would approach gently wagging his tail, and sit calmly to be petted. He caught on to training extremely fast, and had the basics (sit, stay, come, down, heel) down in a week. Looking at him you could see him thinking, and trying to problem solve, something I've never seen in a dog before. He would always keep an ever watchful eye on Ethan. Wherever Ethan went Jet followed. When you looked at him you saw he was a puppy, but it was quickly forgotten when you saw the way he acted.

Eventually we started public access training with him. I was shocked every time when that vest went on and his always calm attitude became even more calm and even better behaved. He did always what he was told when he was told. He would walk calmly through any crowd never blinking an eye and ignoring anything else around him.

One of his first trips out.

Around 6 or 7 months we began to notice after Ethan's seizures in his postictal stage Jet would lay with him, never leaving his side. He was mostly oblivious when the seizure was occurring, but somehow knew he was to stay with him afterward.

Where you find one you're likely to find the other. Jet is very in tune with Ethan, and knows when to keep an extra close watch on him, and when to give him his space but watch from a distance. Ethan has recently been moved into his own room right next to ours. Jet can choose between sleeping on our bed, in his bed in our room, or in Ethan's room, but most nights we find him asleep on Ethan's floor, the most uncomfortable option, but where he chooses to be.

Looking a bit too much like a poodle here....

Taylor loves him too:)

Over the months he has become more and more aware of Ethan's seizures, and more concerned about them. He usually reacts after they have begun. He will come and lay with him, often licking him and just being there, and always sleeping with him after. Today made 4 weeks and 1 day between tonic-clonic seizures for Ethan, the longest he's ever gone. We have been on edge for the last week knowing it would happen anytime. I was making Taylor's breakfast this morning when I heard ever so familiar noises coming from Ethan. I looked over and found him on the floor in the tonic phase with his best buddy laying over him:) What a way to show how special he is on his birthday!

This mornings seizure.

I can't wait to see what the future holds for him. It seems every day he does something that amazes me. He may or may not ever learn to detect his seizures before they happen, but I can only imagine he will continue to respond and react. We are currently working on him retrieving his rescue meds, and he's doing well with that. A treat or two is always good incentive! We hope to have him attending school with Ethan in the next year or two. This is more an issue of Ethan becoming a handler instead of me, and Jet taking commands from him and obeying. He has given so much to Ethan and the whole family in the last year, we can't imagine life without him!