Epileptic Encephalopathies: An Overview (Sonia Khan and Raidah Al Baradie, 2012) states that:
Epileptic encephalopathies are an epileptic condition characterized by brain abnormalities associated with progressive cerebral dysfunction. Eight epileptic encephalopathy syndromes are recognized, depending on the child's age. These syndromes include: early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, West syndrome and Dravet syndrome in infancy, myoclonic status in nonprogressive encephalopathies, and Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow wave sleep in childhood and adolescences. Epileptic encephalopathies are severe brain disorders of early age that manifest with (1) EEG activity that is often aggressive, (2) seizures that are usually multiform and intractable, (3) cognitive, behavioral and neurological deficits that may be relentless, and (4) sometimes early death. EEG demonstrates primarily burst-suppression patterns in the neonatal period, hypsarrhythmia in infancy, and slow generalized spike-wave discharges in early childhood.
Braxton had his first seizure in August 2013, at 3 months old. Most of Braxton's seizures are tonic, they make his whole body go stiff. They usually last about three minutes, although we have had a couple longer than ten minutes. Seizures lasting longer than five minutes become dangerous. We have tried Keppra, Tripetal, Clobazam, Nitrazepam, Topiramate, phenobarbital, Sabril....nothing is working to stop the seizures. We were originally given ativan as a rescue medication to administer when his seizures last longer than five minutes and it failed to stop his seizures. We now have midazolam as a rescue medication.
In December, at 7 months old, Braxton was diagnosed with infantile spasms. Like his seizures we are still struggling to control the spasms with medication. The spasms look like a startle, he has a tiny jump, just a second long. The problem is with infantile spasms is that his brain can be having seizures that we can't see on the outside though movement or stiffening, and so there is no rescue from too many or too long seizures. This type is also associated with regression in babies development, but luckily typically only last a few years. Between the spasms and sedating medications, Braxton is once again like a new born. He has lost the ability to eat, often struggles to breath, and has little motor control.
In January Braxton had his forth EEG, a test that monitors his brain waves. This time it was a 24 hour EEG. His EEGs show he has abnormal brain waves, most likely a symptom of dandy-walker malformation and other brain abnormalities. Since so many medications have not stopped his seizures there is little chance additional medications will stop them. Unfortunately we have few options, steroids or ketogenic diet were the only two paths presented to us by Neurology. We decided to go the route of the ketogenic diet (http://fortheloveofbubbe.weebly.com/1/post/2014/02/ketogenic-diet.html). Braxton has now been on the ketogenic diet for many months. It has been difficult to find the right fat ratio and amount of calories to out Braxton's body into a state of ketosis to decrease his seizures. Although the diet is not yet effect in reducing his seizures, decreasing the other sedating anti-convusant medications has had a positive effect on Braxton's development. He is more aware of his surroundings and able to respond to us. In time we also tried steroids for periods of time, which did help, but was not a long term solution.
In May 2014 Braxton started using medical marijuana and got to his target dose of CBD in August. In September we started monthly IVIG treatment. In November we began theraputic doses of Vitamin B6. So far Braxton's seizures have been intractable (not controlled).
We have had to give midazolam, his rescue medication, many times now, and it often does stop the seizures, but also turns our baby into a unresponsive ragdoll. The more times we use the rescue med the more his body gets used to it and it is less effective for future use. We sometimes are able to go months without needing rescue medications, but now when we need them one dose of midaz isn't enough. We often need two or more doses, or sometimes a loading dose of phenobarb or fosphenytoin as well. These meds have to be administered via intermuscular injection or IV and require him to be monitored in hospital as they really drop Braxton's breathing effort.
Epilepsy is a physical condition characterized by sudden, brief changes in how the brain works. It is a symptom of a neurological disorder - a disorder that affects the brain and shows itself in the form of seizures.
Epilepsy is a disorder, not a disease; it is not contagious.
Approximately 0.6% of the Canadian population has epilepsy. This includes those who take anticonvulsant drugs or who had a seizure within the past 5 years. Each year an average of 15,500 people learn they have epilepsy; 44% are diagnosed before the age of 5. In approximately 50% of cases of childhood epilepsy, seizures disappear completely. The major form of treatment is long-term drug therapy. Drugs are not a cure and can have numerous, sometimes severe, side effects.
Epilepsy still can be a very serious condition and individuals do die of it. Experts estimate that prolonged seizures (status epilepticus) are the cause of many deaths in Canada each year.
Events that may trigger seizures include:
- Poor nutrition
- Missed medication
- Flickering lights
- Skipping meals
- Illness, fever and allergies
- Lack of sleep
- Emotions such as anger, worry, fear and others
- Heat and/or humidity