Keep the Champagne on Ice

I realized something this year that I hadn't thought of before and that is when times are tough, you know what to expect so you are always guarded and when times are good, you are always guarded because you are looking for the things that are familiar. You never get a break. Such is the case for kids with Dravet Syndrome and their families.

Damonito's surgery this summer saved his life. I have no doubt that if he hadn't had the corpus callosotomy he would have spiraled downward and ended up in status. There's a good chance he would not be with us right now. His recovery was hard but he came through it and has been doing much, much better. He seems to be learning, he's more engaged, there has been talk of reducing his medication, and maybe, just maybe everyone could relax.

Except you can never relax when you have a child with Dravet. The full blown grand mals have returned. We will have to do Christmas presents a couple at a time over a period of days. His recovery drugs are being amped up to keep him out of the hospital. The Nevada Santa brought him Elmo slippers. He also got some books. He's good at turning pages and he loves to look at letters and numbers. It's easy to get ahead of yourself when things are going well. For now, we're back to focusing on today.

Routine Post-Surgery Testing

Damonito is spending a few days at Phoenix Children's Hospital having a video EEG. This is a routine test for him, they want to see what is happening in his brain since the surgery this summer. From our perspective, he's doing very well. He has come back from the surgery better than ever, so much so there is discussion about lowering his phenobarbital dose and if that goes well, they will eventually ween him off phenobarb all together. It's a goal.

I would venture to guess that most of what we are seeing on the monitor is pretty normal brain activity. The doctors can tell us more but it almost doesn't matter because we are seeing such a marked improvement since the surgery that it brings us all so much hope. It's such a relief.

One and It's Done

 

Damonito's seizures are tremendously better since the surgery this summer. Nicole described them as "one and it's done" with the one lasting 30 seconds or so. This is great news since before the surgery he was having clusters of seizures that were sending him into status and only subsiding after mega doses of anti-seizure medicine had been administered. 

 

His memory seems to have returned to where he was before the surgery. Such a relief after so much worry about what he would retain. He even seems to have picked up some new things, too.

 

Yesterday hanging out, we were watching Elmo's World. When the door would show up in Elmo's World, Damonito would knock on the door on the front of the TV.

He's definitely doing a lot better.

Special Thanks!!!

THANK YOU to my amazing niece, Megan, for coordinating a donation effort to raise money for the Dravet Syndrome Foundation! You ROCK! And THANK YOU to the fantastic staff at Monfort Heights Elementary in Cincinnati, Ohio for donating $5 each to wear jeans last Friday. What a kind and generous thing to do! You guys are THE BEST!

Success

I feel I can say with some confidence that Damonito's corpus callosotomy was successful. The purpose of the surgery was to reduce the number of seizures and lessen the severity of the ones he does have. We can call it a success because both of those things have happened.
He's not by any means seizure free.

Damonito is still not able to go to school. Just too risky right now. If he gets over-heated or over-stimulated he'll have a seizure. He's happy hanging out at home watching Elmo. The other thing is he's still pretty wobbly when he stands and someone has to be with him all the time.
When I was over the other day, he was watching Elmo and some elephants came on the screen and he turned and said, "elllllll!" It seemed clear he was trying to say "elephant." It's exciting to think he has a new word. He's doing great with words, we weren't sure what he'd remember after his surgery but he seems to have retained more than we anticipated. He definitely remembers that Elmo is his buddy.

Socks the Puppet

Damonito loves Sesame Street. He loves Cookie Monster and the Count (he even mimics the Count, uh..uh..uh...) but he especially loves Elmo. He loves to put socks on his hands and play puppet. Seizures in kids with Dravet can happen because of over excitement, over exertion and stress. Socks the Puppet is a nice, calm activity Damonito can experience without fear of having a seizure.

Plus, he loves it. Anything to get a smile.

Don't Blink

I was set to post about a new drug Damonito is trying. Was trying. Sometimes they know right away that something isn't going to work. The side effects are everything and while the jury is still out on whether it helped Damonito's seizures, Verapamil caused his feet to swell, indicating poor circulation so they had to stop giving him the drug. Verapamil is actually a blood pressure/angina/arrhythmia medicine but it's a calcium channel blocker and apparently it can help kids with Dravet Syndrome for a short period of time. Usually 9-12 months.

He will likely have another chance to try Verapamil. They were hoping it would give him some reprieve while waiting for the CBD trials. Nothing has happened with the trial yet but we're hoping our politicians put people's health ahead of their petty squabbling.
In the meantime, Damonito is up and moving around more and more each day and he's standing to build towers from blocks. He still requires constant supervision but every day feels a bit better.

Two Steps Forward, One Step Back

Damonito took an ambulance ride to the hospital on Friday night. When having a seizure, he's always given some time to come out of it on his own. When that doesn't work, he's given rescue medication. And when that doesn't work, a call goes out to 911. If luck is on his side, the paramedics will be able to get the seizure under control and Damonito will take a ride to Phoenix Children's Hospital. If not, then it's the closest hospital, and eventually he'll be moved to Phoenix Children's Hospital. The problem with going anywhere else is they don't know his history, Nicole has a binder but often the medical staff isn't prepared for someone with Dravet Syndrome. This is not to say they aren't great, they are, but his condition is so rare and his treatment is so aggressive that simply absorbing everything that he is going through is a challenge.

Damonito is going to be ok. His medication has been adjusted. Again. It's a never ending battle to find the all elusive "right balance." And even then, the balance changes and the search continues.

Game Changers

Even though Damonito takes 7 (yes, count them, seven) anti-seizure medications, he has a VNS chip, and the corpus callosum between his 2 brain hemispheres has been cut, he still has seizures. Granted, since his surgery, his seizures are definitely less severe. But long term use of 7 anti-seizure medications will certainly take its toll. Even short term usage has its problems. Phenobarbital can cause headaches, dizziness, and nausea sort of a "hangover" the day after. Day after day usage just means you feel like that all the time. The side effects of Topomax are similar so more headache, dizziness, and tiredness, something like a cold. And then there's Zarontin: headache, dizziness, drowsiness, and general lack of balance but can also cause confusion and hallucinations. How would we even know if he's confused or hallucinating?
We are trying hard to reduce medication however, it's tough because one seizure, just one seizure can put him into status and can change the game.

We're Getting There

I got to spend some time with Damonito on Friday. It was a good day. He was the best I've seen him since his surgery. Definitely more like himself. He still has some obstacles to overcome but it's hard not to be excited when he's in a good mood.

We watched The Adventures of Elmo in Grouchland. I've seen it dozens of times but Damonito loves Elmo so we watched it again. We've been bringing out his birthday toys a little at a time and he was proud to spin wheels and open doors all on his own. Clapping every time he finished. He even used his left hand to hold his strawberry milk (it's actually a nutritional supplement).

Nicole brought him a cup of water that he drank with a straw. I had never seen him drink water before, he'd always gotten it through his G-tube but Nicole said he'd been checked out to actually drink water so last week he got to have some. Damonito is 12 years old and never drank water. Nicole said he really likes it.

Stalled CBD trials

This fall, Damonito was to be part of a medical trial to determine the effects of CBD (or Cannabidiol, a compound found in marijuana) on epilepsy. CBD doesn't get you high, it's not that part of the plant. With all the dispensaries around the county experimenting with CBD oil and hospitals in other states doing trials, there is reason to believe it helps with seizures. It's been well documented.

 The trial Damonito was to be part of has been stalled while our Arizona Congress figures out if CBD should be changed from a Schedule 1 to a Schedule 2 drug. A Schedule 1 drug is considered to have no medical value. Dispensaries around the country produce CBD oil but the thing about having the pharmaceutical companies involved is that you know what you are getting. You know that the pill you take today is exactly the same as the pill you take tomorrow, as the pill you took a year ago. Damonito needs that assurance. We simply can't leave things to chance.

Damonito currently takes 7 anti-seizure medications. He has a VNS chip (kind of a pacemaker for the brain), he has had a full corpus callosomotomy and he still has seizures everyday. CBD could help him. There is hope. Write your Arizona Congressperson and ask that they work to change CBD from a Schedule 1 drug to a Schedule 2 drug so research on the potential medical benefits can be ascertained. It could make a difference in this young man's life.

The Family Visits

Over the weekend Damonito's Aunt Harmonie and family came to visit. It's great to see his face light up with recognition. It's something we're hopeful about but not sure how much he has retained. He seems to remember more than we anticipated.

Damonito had a birthday but postponing the party until he was feeling better seemed like the best option for him right now. He's still weak and struggling from the surgery plus he's still having seizures. Not as bad as before but seizures. Right now anything can be a trigger.

The Sykes brought some presents in plain brown paper wrapping. Counting games, he loves counting. He absolutely remembered to tear open the paper to reveal his gift. He even knew he needed help to get it started.  

And then there were the thank you kisses.

A good time was had by all.

Never Become Hopeless

There was an article in the July 2015 issue of Wired Magazine about a boy with epilepsy. Sam has a different type of epilepsy than Damonito but there are some similarities between them. Sam's epilepsy benefited from cannabis and if all goes well, Damonito will be part of a study involving cannabis in the fall. The thing about the article that really resonated with me was the very last paragraph.

His dad, Fred Vogelstein writer of the article, wrote that years ago an epileptologist had made a point of saying that, as hard as you might find it sometimes, the one thing you should never do is become hopeless about the situation.


Sometimes I wonder how long he can go on this way but it's just my own preconceived idea because I know the possibilities. Damonito keeps pressing on because he doesn't know any different. There are good days and not so good days but each day has to be accepted for what it is. Most importantly, never give up. There is always hope.

Happy Birthday, Damonito!

Happy Birthday, Sweet Pea. You are the light of our lives.

Smiles all around

Yesterday was a good day. It was a good day in a string of just ok days but when a little progress is made it's hard to be unhappy. Damonito is still having seizures but not with the intensity as before the surgery. That said, yesterday was a good day.

Yesterday was a day of lots of love. Hugs and kisses for everyone. He's such a sweet boy. We played tickle, hearing him laugh is music to my ears. There is no better sound.

Kids with severe epilepsy often have behavioral problems, not this kid. He's about as sweet as they come.

Number 8

Damonito is fascinated with numbers. 8 is his favorite, 2 is a close second. Damonito has really never been able to talk. He has a voice and about 5 words, eight and two are among them. And silly. He says, "silly, silly, silly." I dropped by to hang out for a bit yesterday and he gave me a big hug and a smile. When he hugged me he patted my back and said, "awww."

It's hard to know what has been affected by the surgery. He's not saying numbers, although he likes to look at them and points. I would say his personality hasn't really been affected, he loves to give hugs and kisses, always has. He moves very slowly and deliberately. He's shakey and weak, and tires quickly. But I do see progress.

Beautiful

One of the first things people notice upon meeting Damonito is his incredibly long eyelashes. Everyone comments about them. He has these gorgeous deep blue eyes and these long lashes to go with them. He's quite handsome. I'm not just saying that because he's my grandson but because he is beautiful. Those eyelashes have something to do with it. For 11 years I have tried to capture the beauty of his long lashes. And I keep trying.

There's No Place Like Home

Damonito is home. A hospital bed was delivered along with some medical monitoring equipment and he is resting comfortably. He gets worn out pretty easily but it feels like we've turned some corners. It's good to have him home.

Giant Leap

I wear an epilepsy awareness wrist band with Damonito's name on it. We have this game where he takes it off my wrist and plays with it, tossing it around. I handed him my wrist band and he moved it from his left to his right hand. Sounds simple but for him, huge.

So huge, in fact, he was so proud, he gave me a little smile.

Teeny Tiny Baby Steps

We know this will be a long road to recovery. There will be physical therapy, occupational therapy and speech therapy. After a surgery such as his, sometimes it takes time for the right and the left to figure things out. We look for signs in everything.

Elmo is Damonito's trusty side-kick. Elmo found his way to his shoulder. Damonito is in there, he's slowly making his way to the surface.

He's in there...

 I'd like to say we were seeing progress every day but we really weren't. He wasn't having seizures, at least that we could see. There was that. He would open his eyes a little but he just stared into space. No eye contact.

Late afternoon on Friday, Nicole decided to climb in bed with him. Damonito opened his eyes slightly. We were talking to him trying to get some recognition from him when Damon said something from behind me and Damonito's eyes moved toward his dad's voice. When his dad leaned in for a kiss, Damonito slowly reached his arm up and rubbed his dad's head. This was a game they played daily, Damon would put his head down and Damonito would rub his hair. The tears were flowing, he's IN there. He is in there.

Post Surgery

There was no way this could be a slam dunk. That would have been the miracle of miracles. But he's a fighter. The day after surgery was not a good day. Damonito definitely had some swelling, the side of his head was bulging. His face was puffy. We expected his eyes to swell shut. They did the last time. What we didn't expect was a 12 minute grand mal seizure first thing in the morning. Followed by seizures we'd never seen before, jerking as though startled followed by stiffness. Every 5 minutes. Clearly a tonic seizure but not one familiar to us. After a massive dose of phenobarbital the seizures subsided.

He still wasn't breathing right. His blood pressure was all over the place. His temperature was climbing. In the afternoon, Damonito spiked a fever 104.4 and the seizures returned. More medication, clothing stripped, and then a cooling blanket introduced. They don't throw you in an ice bath any more, thank goodness, apparently that was too shocking to the system.

These are the moments you question everything you know. You hope you've done the right thing. You hope that you haven't fixed one thing at the price of something else. He sailed through his last surgery. This one wasn't going to be so easy. There were no words to describe how deflated we felt.