More About Treatment

Determining an effective treatment plan for children with Dravet syndrome can be very challenging due to the intractability of the seizures and the fact that these children can be quite variable in their response to different therapies.  Monotherapy is not usually adequate to control seizures.  Most of the time two or more treatments are combined to help children achieve their best seizure control.  These combinations of treatments may include one or more anticonvulsant medications (AEDs), the ketogenic diet, vagus nerve stimulation (VNS), intravenous immunoglobulin therapy (IVIg), or certain dietary supplements that may be prescribed.  Some treatments have been found generally more helpful for treating Dravet syndrome than others, and some are most often ineffective or may aggravate the condition.  

Some treatments that have been consistently found to be ineffective or to have an aggravating effect on patients with Dravet syndrome, and should be avoided include: carbamazepine (Tegretol) and oxcarbazepine (Trileptol), lamotrigine (Lamictal), vigabtrin (Sabril), phenytoin (Dilantin, Epanutin) or fosphenytoin (Cerebyx, Prodilantin, Biston, Calepsin, Carbatrol, Epitol, Equetro, Finlepsin, Sirtal, Stazepine, Telesmin, Timonil).  While not recommended for chronic management of Dravet syndrome, phenytoin or fosphenytoin have not been ruled out as useful for management of acute status episodes.  Epilepsy surgery is not considered effective treatment for Dravet syndrome at this time.

Medicines which have, based on scientific literature, been found to be useful for seizure management in Dravet syndrome include: topirimate (Topamax,) which may decrease status episodes, partial and generalized seizures; valproic acid (Depakote, Depakene, Convulex, MicopakineLP), shown to decrease status episodes, partial and generalizes seizures, possibly absence; clonazepam (Klonopin) and clobazam (Frisium, Urbanyl®,Mystan®), both of which can decrease myoclonic, generalized or partial seizures as well as absence seizures; and stiripentol (Diacomit) which decreases status episodes when used with valproic acid and clobazam or clonazepam by increasing therapeutic drug levels of these medications.

Not all of these medications are currently available in every country.  Obtaining stiripentol in the U.S. requires a letter of medical necessity and prescribing physicians and parents will need to work through Caligor Pharmacy in New York City at (212) 368-6000 or directly with the manufacturer, Biocodex at
webar@biocodex.fr

Treatments that may be helpful in Dravet syndrome, but which are in need of further study include: ethosuximide (Zarontin), which may help with absence seizures; leviteracetam (Keppra), which has data pending publication; zonisimide (Zonegran, Excegran); Vitamin B6; intravenous immunoglobulin (IVIg); the ketogenic diet and vagus nerve stimulation (VNS).

As a key part of their treatment plan, it is recommended that all children with Dravet syndrome have an emergency protocol in place.  This protocol should be determined by the neurologist in consultation with the parents and according the specific needs of the child.  It should include the ‘at home’ administration of drugs to treat seizures lasting longer than 5 minutes.  In general these drugs would be rectal valium (Diastat) or intra-nasal or buccal midazalam (Versed).  Other medications, such as paraldehyde (where available) may be used for specific situations.  It should also discuss any need for oxygenation, and at what point to call for medical assistance or go to emergency department. 

Parents should be aware of the importance of avoiding seizure triggers.  Fevers should be treated aggressively and overheating of any kind should be avoided.  Chills and shivering, as well as sudden temperature changes may also be an issue.  Some children with Dravet syndrome are photosensitive and/or pattern sensitive.  Parents and caregivers should carefully observe what is happening with their child and avoid situations that may be aggravating to their condition.

Children diagnosed with Dravet syndrome should have regular developmental assessments by qualified personnel and should receive therapies in the areas of speech, physical, occupational and play as early in the course of the disease as possible in order to help them achieve their highest potential.

Note: The above is intended to describe the “fundamentals” regarding the treatment of children with Dravet syndrome based on scientific literature and the clinical experience of practitioners treating this type of epilepsy.  This should not replace the professional judgment of the treating neurologist, but rather provide information about current best practice for the management of Dravet syndrome.