Sadness in Our Hearts

It is with great sadness that I write this. Damonito passed away early Friday September 2, 2016. He had been moved into at home Hospice Care in June because it was felt that he had moved into the next phase of Dravet Syndrome. His brain was no longer regulating the things we take for granted, temperature, muscle coordination, sleep, and swallowing. He had been getting worse but he was such a fighter and would often surprise us by following a really bad day with a string of really good days. In fact, when I spent time with him last Sunday, he was up, moving around, playful and happy. We had a great day together. Thursday Nicole noticed a decline she hadn't seen before. I have to say that Nicole has superpowers. She has the ability to see things that are invisible even to the doctors. She called me Thursday to say I needed to come right away and of course, I did. No one, at that point, could have anticipated the outcome. He had become comatose but at one point, he did open his eyes and gave everyone a hug, Nicole knew it was his goodbye. But you know, he had come back from the edge so many times I think we all thought he would find his way back. By midnight, he was struggling to breathe and we decided to move him to the Ryan House in downtown Phoenix. Transport came to get him and Nicole rode with him. He took his last breath around 4am.
I can't begin to tell you how tremendously sad we are. He was the light of our lives. He made us all better people. He was our prince, our beautiful boy, our knight in shining armor. And we will miss him every single day.

Familiarity

Damonito has had some really bad days. This is nothing new. He's also had some really good days, again, nothing new. It's easy to get drawn into thinking it's all going to be ok because it feels familiar. Just a string of good and bad days.

There are some definite issues that need daily attention. Swallowing is a real problem and will only get worse. The suction/aspirator machine has been tremendously helpful. And there's morphine for those moments when his body takes over and anxiety sets in because when you can't swallow sometimes you can't breathe. It's frightening. Damonito experiences panic attacks just like anyone else.

Damonito loves to turn on and off the light switch in his room. He watches the lights and the fan. He's discouraged from turning everything off because the fan in the room helps keep it cool. Also, he's struggling to walk making getting to the light switch difficult. So I bought him his very own light switch that lights up when he flips it on. He loved it. There is the possibility it would cause seizures so he'll have to be closely supervised when playing with it. But so far, so good.

Happy Birthday, Damonito.

Damonito had a birthday on Wednesday. He's 13 now. It's pretty amazing, actually. He's a teenager. It's a big deal.

DNR

Signing a DNR for a child is much different than signing a DNR for an adult. Especially someone who is elderly. It's a lot more clear when someone has lived a long life, you understand when they are sick and there is no hope for recovery. It's not so clear with a child. You hold on to hope.

When you sign a DNR for someone, you are letting medical personnel know that you don't want that person to be resuscitated by chest compressions or intubation. In other words, you don't want the person to have their heart restarted if it stops or a tube down their throat if they stop breathing. You are letting this person die a natural death. You are simply going to let them go.

If you don't sign a DNR, medical personnel is obligated to do whatever measures are necessary to keep someone alive. They have to by law. If they don't, they could put their own jobs and licenses on the line. It's the deal. It is only after all measures have been taken with no success that a person be allowed to die.

How is it even possible to make that decision for your own child? For a kid? As a parent, how can you see the situation rationally even when you know what is best? When you know your child has been through so much and managed to hang on but you are now facing the end of the road? How do you sign off on that?

Do Not Resuscitate. You doubt everything you know.

When Every Moment Counts

Damonito went to see a palliative/hospice pediatric doctor this week. His epileptologist recommended this new doctor because he feels that Damonito has moved on to the next phase of Dravet Syndrome. No one really knows. It's all pretty uncharted territory with Damonito steering this ship. We are no longer leading but following him now.

One of the things this new doctor told us is through her career in hospice, she's had five other Dravet Syndrome kids. Damonito is the oldest. That's the good and the bad news. Good because he's beaten the odds so many times he's setting a precedent. He has always found his way back. Bad because we don't know how much time he has left but it doesn't feel like much.

Damonito will get a new bed and other equipment as he needs it. We will take each day as it comes and see it for the blessing it is. He is interactive and happy and that's what is important. We will make every moment count because the moments are limited.

No News is Good News

 

I got to spend a little time with Damonito on Sunday, he's doing really well. Compared to last year at this time, he's fantastic. 

Damonito has been using Klonopin as a rescue drug since the onset of this epilepsy. Klonopin is used for a number of issues but it was developed as an anti-seizure medication and it works well. However, it has a number of undesirable side effects so long term use is not recommended (although it is used long term for many because it works). 

The point of a rescue drug is just that, for rescue and unfortunately since last June Damonito has been taking Klonopin daily. Damonito needs a reliable rescue drug so when all else fails and he is having uncontrollable seizures, he has something that will bring him out of it. The surgery was intended to ease his seizures so he could be taken off the regular dose of Klonopin and bring it back only when he needs a rescue.

It's a slow process but it's working. He's better than he has been in awhile.

Happy New Year

With a new year there is always new hope, Damonito is doing ok. He's still having grand mals but he is way better in January of 2016 than he was in January of 2015. He has a seizure disorder and that's not going away but he's better and we'll take what we can get. 

 

He's up and moving around a lot more. He's still not very steady and too much activity can mean a difficult night with seizures but it's hard to keep him down. He repeats movement like someone with obsessive compulsive behaviors. According to the Epilepsy Foundation website, "OCD is uncommon (some would say rare) in people with epilepsy, but there does seem to be a connection. OCD is seen most often in those with complex partial seizures originating in the temporal or frontal lobe, or seizures originating in the anterior cingulate gyrus, near the corpus callosum." 

Damonito has generalized seizures which means his seizures happen in the entire brain. I'm not sure how that fits into the OCD model but observing Damonito for just a few minutes, you would realize he has obsessive compulsive disorder. Or some version of it. 

For now, he's ok. We're looking forward to the new year. 

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.