Yes, I am familiar with Dravet's syndrome
I do know many kids with Dravet's from the Dutch forum I host. This 'seizure disorder' is called an 'epilepsy syndrome'
Dravet's (SMEI, Severe Myoclonic Epilesy in Infancy) is a maligne childhood epilepsy syndrome, maligne meaning it is a medicine resistant type of epilepsy with a disasterous influence on the cognitive, mental and physical development of children. Dravet's does not disappear when kids grow older and it is not a syndrome with only myoclonic seizures like the name SMEI suggests. The seizures sometimes get less frequent in puberty.
The first seizures (myoclonic jerks) in early childhood (usually betwen age 1-3) are triggered by fever. Dravet has a genetic cause, a mutation called the SCN1a gene (found in 70% of cases, 30% without this gene are called 'borderline SMEI'). Kids with Dravet are always (severe) mentally disabled. Some kids with Dravet's can stand and walk but they usually have an unstable 'drunk' type of walking and most of them do need a stroller or wheelchair. Some of them can speak, others can't. Kids with Dravets need to be cared for all their life.
Kids with Dravet have all types of seizures, in most cases myoclonic jerks and complex patials, but many have tonic-clonics and drop seizures when they grow older. Many kids with Dravet end up on a regular base in the hospital with status epilepticus or non convulsive status epilepticus (NSCE) Usually Diastat doesn't help and midazolam IV is needed to stop these seizures. Some kids with Dravet need to be monitored for saturation and need oxygen during a seizure.
Stiripentol (recent new drug) and Topamax, with as an add on clobazam, and the ketogenic diet are good choices to try for Dravet's syndrome to decrease frequency of seizures. Kids with Dravet who are a 100% seizure free, are exceptions. Lamictal tends to cause seizure increasement (recent research.)
A support group for parents is the former IDEA league
http://dravet.org/about-dravet/smei , yoú can read more about Dravet including testimonials there.
Other maligne childhood epilepsy syndromes are West syndrome (Infantile spasms), Lennox Gastaut syndrome, Doose syndrome (MAE), Othahara syndrome, Aicardi syndrome, Landau Kleffner syndrome and ESES.
