Because Dravet syndrome is a spectrum disorder, the course of the disease is variable from child to child and there is no standard treatment protocol. Multiple and changing seizure types are common and vary from patient to patient. EEG monitoring and neuroimaging techniques are often used to help further confirm diagnosis.
Anticonvulsants that have been shown to be useful for chronic seizure management in Dravet syndrome include benzodiazepines, topirimate, and valproic acid. Stiripentol (Diacomit) has been show to have efficacy in some Dravet patients when used in conjuncture with other anticonvulsants, typically valproic acid and clobazam.
Anticonvulsants frequently used in treatment:
- clobazam (Onfi, Frisium, Urbanyl)
- clonazepam (Klonopin, Rivotril)
- leviteracetam (Keppra)
- stiripentol (Diacomit)*
- topiramate (Topamax)
- valproic acid (Depakote, Depakene, Epilim, Epival)
Many patients with Dravet syndrome experience prolonged seizures (status epilepticus) that require emergency intervention. For this reason, your neurologist may prescribe a rescue medication, typically a benzodiazepine, that is given during the seizure to help stop it.
Rescue medications include:
- clonazepam (Klonopin)
- diazepam (Diastat)
- lorazepam (Ativan)
- midazolam (Versed)
Avoid Contraindicated Medications
Care should be taken in avoiding anticonvulsants which are contraindicated in the treatment of Dravet syndrome, due to their interaction with the sodium ion channel. Anticonvulsants which should be avoided include carbamazepine, lamotrigine, and vigabatrin, which can cause or increase seizure activity. Phenytoin may be used acutely in managing status seizure episodes but is generally not helpful in chronic management.
Anticonvulsants that may worsen seizures:
- carbamazepine (Tegretol, Calepsin, Cargagen, Barbatrol, Epitol Finlepsin, Sirtal, Stazepine)
- fosphenytoin (Cerebyx, Prodilantin)
- lamotrigine (Lamictal)
- oxcarbazepine (Trileptal)
- phenytoin (Dilantin, Epanutin)
- vigabatrin (Sabril, Sabrilan, Sabrilex)
Other treatments and therapies have shown positive results in the overall care and management of Dravet syndrome in some patients, even though they have not been fully studied. These include IVIG (Intravenous Immunoglobulin) Therapy, VNS (Vagus Nerve Stimulation) Therapy, and the ketogenic diet. Families have also reported that the use of CBD (cannabidol) has been beneficial with both seizure management and cognition. Read DSF’s position statement on CBD here
Medical Procedures & Testing
A patient will have various medical tests performed to either diagnosis or monitor their condition. These procedures may include blood tests, neuroimaging tests (CT scans, EEGs, & MRIs), genetic testing, and metabolic testing. Most of these tests are minimally invasive and will help the medical team assess the best protocol and treatment for the patient.
Establish an Emergency Protocol
It is important to design and implement an emergency protocol with your neurologist, including a fast onset benzodiazepine such as Diastat, nasal versed, or lorazepam, for any convulsive seizure lasting longer than five minutes. It is helpful to have a written copy of this protocol with the child at all times, in case of emergency. This protocol should include instructions on seizure treatment and when to call for emergency services, as well as parent and physican contact information.
Monitor & Treat Secondary Health Conditions
Be aware of secondary health conditions common to the syndrome to make sure they are properly managed. These conditions vary from patient to patient and may include:
- cardiovascular conditions
- dental health concerns
- orthopedic & scoliosis issues
- sleep disturbances
- weakened immunity
Growth and weight should also be followed closely and parents should be aware of treatment options such as gastrostomy tubes (g-tubes) when appropriate.
Learn & Avoid Seizure Triggers
Avoid seizure triggers whenever possible. Common triggers for patients with Dravet syndrome include rapid changes in environmental and/or body temperature, illness, stress, overexcitement, patterns, and flickering lights. Fevers should always be treated aggressively with a plan established with your pediatrician and/or neurologist.
Children with DS often also face developmental challenges such as autism or autistic-like characteristics, cognitive and/or communication delays, social skills, and behavioral issues. Regular developmental assessments and early and aggressive therapies (speech, OT, PT, developmental, etc.) may help with the overall outcome for the child.
Children with Dravet syndrome typically need constant care and supervision, as well as help in avoiding seizure triggers. Equipment that has been found by families to be useful in the day-to-day management of Dravet syndrome includes video monitoring, protective helmets, cooling vests, pulse oximeters, seizure alarms, and glasses with colored lenses (for photosensitivity).
Coping as a Family
A child’s chronic illness will have both direct and indirect effects on family members and their relationships. It is not uncommon for family members to feel denial, anger, fear, shock, confusion, self-blame, and helplessness. Family and grief counseling can help families deal with having a child with a chronic illness.