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.

Surgery

 

The thing about brain surgery is, it's your brain. It's fragile. No one really knows what the outcome will be for Damonito. We can hope, when all is said and done, the only thing that will be different will be his seizures. But there's no way to know. We have to be patient. 

The surgery went fine. The surgeon was confident. The first 24 hours we were to watch for stroke. When we asked what to look for, the doctor said, "left side weakness." Shoot, he's always had left side weakness, what now? We were told we'd know.

Corpus Callosotomy

 

When Damonito was 3, a partial corpus callosotomy was done to help control his seizures. A portion of the membrane between the hemispheres of the brain was severed with the idea that it might keep seizures from traveling from one side of the brain to the other. A partial is usually done first because it basically cuts your brain in half which can create all sorts of issues with learning. At 3, it made sense do only do a partial corpus callosotomy because there was no way to know where he would end up developmentally.  

At 11, we know he will never be able to read. We know he'll never ride a bike or drive a car. So  the decision to do this was an easy one. 

Status Epilepticus

 

Status epilepticus can occur when a seizure lasts more than 5 minutes or seizures happen in clusters. When there is no rest between seizures the brain can get stuck and there is no recovery. The longer a seizure lasts, the less likely it will respond to medication, the more increased risk of death. One of Damonito's doctors said that every time he has a seizure that doesn't stop on its own, he has a 20% chance of dying. It's scary.

This year has been particularly hard. His seizure activity has been increasing, there have been a number of hospital stays and then in May, he had a seizure that wouldn't stop. High power drugs were administered and eventually they got the seizure under control but it took days in ICU to recover.

After 4 trips to ICU in about a month, his doctors recommended a corpus collosotomy. Brain surgery. It really wasn't a tough decision because he was out of options. His rescue drugs were being used daily to control seizure activity. He already has a VNS and tried the keto-genic diet. He needed this.

Fighters

Despite everything Damonito has been through, the prognosis of his condition, and all the health issues that go along with it, he's in pretty good shape considering the outcomes for most kids with Dravet. Don't get me wrong, it's tough. He's had a rough time of it. It's been hard on everyone but I've learned I have a bunch of fighters in my family.

The past couple of months have been especially challenging because as Damonito goes through puberty, an increase in testosterone levels can cause seizures. Unfortunately, there is no way to decrease testosterone levels in developing boys. Also, there is no way to know what all that testosterone will do in the end. 1/3 of the patients get better, 1/3 stay the same, and 1/3 get worse. As his seizure activity increases and becomes uncontrollable, there is a greater risk of him going into status epilepticus and not recovering. The rescue drug literally becomes life saving. He's got that family fighter in him and he's fighting everyday. It's an amazing thing to see.

Dravet Syndrome

http://www.dravetfoundation.org 

According to the Dravet Syndrome website Dravet presents itself with:

•  Seizures of various types beginning in the first 12 months of life
• Seizures begin as febrile, but later also appear without a fever 
• Episodes of status epilepticus (prolonged seizures) 
• Seizures do not respond to standard anticonvulsant drugs 
• Initial development is normal, then slows or stagnates in the second year of life 
• Myoclonic seizures occur around the age 18 months of age 
• Seizures associated with vaccinations, hot baths, or warm temperatures

Seizures of various types beginning in the first 12 months of life Seizures begin as febrile, but later also appear without a fever Episodes of status epilepticus (prolonged seizures) Seizures do not respond to standard anticonvulsant drugs Initial development is normal, then slows or stagnates in the second year of life Myoclonic seizures occur around the age 18 months of age Seizures associated with vaccinations, hot baths, or warm temperatures - See more at: http://www.dravetfoundation.org/dravet-syndrome/medical-information/diagnosis-testing#sthash.IAImMpnE.dpuf

There are daily medications, then there are rescue medications. The rescue medications are given when seizures requires emergency intervention. Sometimes even those don't work and that means a ride in an ambulance. There are anti-seizure medications that shouldn't be given to someone with Dravet because it will actually cause seizures. 

There are also alternative treatments that have had some success, VNS (Vagus Nerve Stimulation), Ketogenic Diet, IVIG (Intravenous Immunoglobulin), and CBN (cannibidol).

 
There are also a whole list of secondary health issues that must be monitored that include: sleep disorders, dental health concerns, cardiovascular conditions, and weakened immunity. It's a daily balancing act.

Intravenous Immunoglobulin

Earmarks of the syndrome include:

• Seizures of various types beginning in the first 12 months of life
• Seizures begin as febrile, but later also appear without a fever
• Episodes of status epilepticus (prolonged seizures)
• Seizures do not respond to standard anticonvulsant drugs
• Initial development is normal, then slows or stagnates in the second year of life
• Myoclonic seizures occur around the age 18 months of age
• Seizures associated with vaccinations, hot baths, or warm temperatures

- See more at: http://www.dravetfoundation.org/dravet-syndrome/medical-information/diagnosis-testing#sthash.IAImMpnE.dpuf

New Diagnosis

Damonito tested positive for a gene mutation that causes Dravet Syndrome. His epilepsy is even more rare than we believed. Not that this changes his outcome, Damonito's epilepsy is severe, his seizures are polymorphic and intractable. Which means he has many types of seizures that are not controlled by medication. In other words, he has seizures every day.