Treating seizures and related conditions is unique to each child or adult diagnosed with a Dravet Spectrum Disorder. An accurate diagnosis is critical for best treatment results. Because these are rare diseases, many families find it is helpful to consult with an epileptologist, a neurologist who specializes in treating epilepsy. They diagnose and treat many others with rare forms of epilepsy.

• Learn about Dravet.org’s Clinical Research and Comprehensive Care Network.

Current treatments focus on reducing seizure severity and frequency. Associated conditions are a significant feature of Dravet syndrome, and some of the related disorders. Whole patient care is required.

Treatment Goals

Seizure-preventing medicines, also called antiepileptic drugs or anticonvulsant drugs, are prescribed for the various Dravet Spectrum Disorders. Doctors prescribe medicines with the goal of seizure-freedom with few or no side effects from therapy. Often, complete seizure control is not achieved. The best balance between seizure control, medication effects, and quality of life is sought. Medicines to stop seizures in progress (rescue medicines) are prescribed for those with a history of prolonged seizures or status epilepticus.

Although treatment does not cure the disease, seizure control helps improve quality of life for both patients and their families. Optimal control helps children learn and grow to their full potential.

Starting Treatment

Febrile seizures are usually the first to appear in Dravet Spectrum Disorders. Most children with “ordinary” febrile seizures do not require treatment with medication. Only a small portion of infants with one febrile seizure will develop epilepsy; a number of these, however, will turn out to have a Dravet Spectrum Disorder.

The decision to begin treatment and the choice of drug depends on many factors: a specific epilepsy syndrome diagnosis, the person’s seizure history, and other medical needs.

Doctors typically prefer treating with a single antiepileptic medicine (monotherapy). Recurring febrile seizures and GEFS+ may respond to a single medicine. Careful adjustments to establish the dosage that best controls seizures and observation for medication side effects requires a partnership between the doctor and the family. Some medications require blood testing to help assess the correct dosage; with other medications, levels are not very useful or not even routinely available. It is common to need multiple adjustments of seizure medication over time to achieve the best results. If the number of seizures or their severity is still not reduced, a different medication must be utilized.

Children and adults with ICE-GTC, SMEB and Dravet syndrome (SMEI) may have many different types of seizures that often do not respond well to treatment. Usually, more than one medicine in combination with others is needed to control seizures (polytherapy).

Taking medicines according to doctor’s orders is very important in getting control of seizures. If you have any questions or concerns, or notice any new symptoms or side effects, contact your doctor.

Antiepileptic Drugs

There are many seizure-preventing medicines in use worldwide. There is some difference in availability, (even between the United States and in European Union). Most antiepileptic drugs are taken by mouth. Some people experience side effects, others will not. Side effects generally are dose related; they are more likely to occur with increased dosage. Ask your child’s doctor about possible side effects. Also, ask about drug interactions with other medicines or foods.

Antiepileptic medicines that may help patients with Dravet Spectrum Disorders:

• clobazam (Frisium, Urbanyl)
• clonazepam (Klonipin, Rivotril)
• divalproex sodium and derivatives (Depakote, Depakene, Epilim, Epival, Micropakin)
• leviteracetam (Keppra)
• stiripentol (Diacomit)*
• topiramate (Topomax)

*available in the European Union only.

Antiepileptic medicines that may make seizures worse in patients with Dravet Spectrum Disorders:

• carbamazepine (Tegretol, Calepsin, Cargagen, Barbatrol, Epitol Finlepsin, Sirtal, Stazepine)
• fosphenytoin (Cerebyx, Prodilantin)
• lamotrigine (Lamictal)
• oxcarbazepine (Trileptal)
• phenytoin (Dilantin, Epanutin)
• vigabatrin (Sabril, Sabrilan, Sabrilex)

Rescue Medicines

Prolonged seizures, particularly if generalized tonic clonic or clonic in nature, can be a serious concern. Patients with a history of prolonged seizures or status epilepticus may be prescribed rescue medicines. These are usually in the benzodiazepine class such as, clonazepam (Klonopin), diazepam (Diastat, Valium), lorazepam (Ativan), or midazolam (Versed). These are medicines given to stop seizures in progress. The doctor provides specific instruction for their use according to individual’s needs.

Alternative Treatments

Ketogenic Diet (KD)

This is a diet high in fats and low in carbohydrates. It is prescribed under the careful supervision of a doctor who is familiar with its use; a team of doctors, dieticians, and nurses work together to manage the KD. It is very strict diet with potentially significant risks; it should not to be done on your own. Other dietary therapies such as the low glycemic index diet or other modified low carbohydrate diets are of use in some patients. They also require the advice of an experienced team to maximize benefit and minimize risks.

Vagus Nerve Stimulation (VNS)

This therapy involves sending small pulses of electricity to the brain through the vagus nerve, a large nerve in the neck. The VNS generator device is placed under the skin on the chest; a surgeon places attached wires between it and the vagus nerve.

Learn about secondary conditions and developmental issues

Whole Patient Care

Dravet spectrum disorders affect the whole person. Other conditions may include:

• Autism spectrum characteristics and communication impairments
• Cardiovascular problems
• Coordination, growth, and nutrition issues
• Developmental and cognitive impairments
• Dental health
• Dysautonomia (problems with automatic body functions: temperature regulation, decreased sweating, slow intestinal function, and sometimes rapid heart rate)
• Infections and immune dysregulation (often recurring upper respiratory and ear infections)
• Orthopedic conditions  (such as foot deformities, curvature of the spine (scoliosis) and walking difficulties)
• Sleep disorders

Developmental assessments should begin as early as possible. Developmental delays usually appear between one and four years of age. Discuss your questions and concerns with the doctor. Getting help early on from specialists in physical, occupational, speech and social therapies can make a difference in your child’s optimal growth and development.