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.

It's Been Awhile

It's been awhile since I've posted. Damonito is doing as well as can be expected of a kid who has Lennox-Gastaut. He's 11 years old now and while this past year has been a bit rough, he's happy. Damonito had another surgery to replace a dead battery in his VNS. When the VNS was originally placed at the base of his brain, the doctors said the battery would last 10 years. This is the third battery in under 6 years but they estimate it's because it's at the maximum output so it wears down sooner. The VNS is an important part of his seizure control.

The bad news about this past year is that D'ito has developed migraine headaches. It's not hard to determine when a kid, who is normally very active, is suffering. And then of course there was the vomiting. He's taking a migraine medication, amitriptyline, however it's made his grand mal seizures worse. It's tough. It's all about the side effects. Everything that is done for him centers around the side effects. So having a couple of grand mals each week is better than him suffering from debilitating migraines.

D'ito is finally off Kepra, something they've been trying to do for some time. He still takes Onfi, Topamax, Neurontin, Fycompa, and Lamictal. If it sounds like a lot, it is. His doctors think he's taking too much medication but it's what keeps him from daily seizures.

 

D'ito celebrated his 11th birthday in July. We had a party delay because he had a long grand mal seizure so we postponed the party to the next night when he was feeling better. You never know what will trigger a seizure, it could be the wrapping paper, it could be he missed a nap but it takes awhile to recover so it's best to let him rest. But he loves opening presents. It's such joy to see his face light up. And of course, there's that laugh. It's so great to hear him laugh.