Treatment Options

Unfortunately there is no cure for Dravet Syndrome yet, although we are hopeful for the future as clinical trials progress with new genetic therapies. At present Treatment options for Dravet syndrome focus on reducing seizure frequency and severity using anti-seizure medications. Other therapies include the ketogenic diet, Vagus nerve stimulation (VNS), rescue medications for prolonged seizures, physical, occupational, and speech therapies, and management of co-occurring conditions. Avoiding seizure triggers like fever and overheating, as well as certain seizure-worsening medications such as some sodium channel blockers, is crucial. 

Treatment options for Dravet syndrome (DS) focus on reducing seizure frequency and severity using anti-seizure medications (ASMs) like valproic acid, clobazam, and stiripentol, as well as newly approved medications such as cannabidiol and fenfluramine. Other therapies include the ketogenic diet, Vagus nerve stimulation (VNS), rescue medications for prolonged seizures, physical, occupational, and speech therapies, and management of co-occurring conditions. Avoiding seizure triggers like fever and overheating, as well as certain seizure-worsening medications such as some sodium channel blockers, is crucial. 

Anti-Seizure Medications (ASMs)

• First-line options:

  Valproic acid is often the first-line treatment, followed by clobazam and stiripentol, especially in combination. 

• Approved newer drugs:

  Cannabidiol (Epidiolex) and fenfluramine (Fintepla) are FDA-approved for people two years and older to help control seizures associated with DS. 

• Other options:

  Topiramate, levetiracetam, ethosuximide, clonazepam, and zonisamide may be introduced if seizures are not controlled. Bromides may also be used for resistant cases. 

• Medications to avoid:

  Certain ASMs, such as oxcarbazepine, carbamazepine, phenytoin, and lamotrigine, can make seizures worse in people with Dravet syndrome. 

Non-Medication Therapies

• Ketogenic Diet

  A high-fat, low-carbohydrate diet that can be effective in reducing seizures for some individuals with DS. 

• Vagus Nerve Stimulation (VNS)

  A device implanted to send electrical pulses to the vagus nerve to help control seizures. 

• Therapies

  Physical, occupational, and speech therapy can help with overall symptoms, gait problems, and quality of life. 

Supportive Care

• Rescue medication:

  Having prompt rescue treatment for prolonged seizures (status epilepticus) is an essential part of an emergency protocol. 

• Seizure triggers:

  Identifying and avoiding seizure precipitants, such as fevers, overheating, stress, and flashing lights, can help manage the condition. 

• Multidisciplinary team:

  Management should involve a multidisciplinary team to address the various health problems and developmental challenges associated with DS. 

Emerging Treatments 

• Genetic therapies: Clinical trials are underway for gene-based therapies and antisense oligonucleotides that target the underlying SCN1A genetic mutation responsible for Dravet syndrome.