How is Dravet syndrome treated?

How is Dravet syndrome treated?

The goal of treatment is to reduce the number and the severity of your child’s seizures. Because seizures range in type and length, no two children respond to treatment in the same way.

Medications

Medications approved specifically to treat seizures associated with Dravet syndrome are:

• Stiripentol (Diacomit®).
• Cannabidiol (Epidiolex®).
• Fenfluramine HCl (Fintepla®).

All three medications are approved for use in children two years of age or older.

In 2017, epilepsy experts called the North American Consensus Panel developed treatment recommendations, listing medications that should be tried first, second and third. These medications are shown in the table.

Antiseizure MedicationsFirst-line treatmentsClobazam (Onfi®, Frisium®, Urbanyl®) Valproic acid (Depakote®, Depakene®, Epilim®, Epival®)Second-line treatments (added to first-line medications)Sitripentol (Diacomit®), Topiramate (Topamax®)Third-line treatmentsClonazepam (Klonopin®, Rivotril®), Levetiracatam (Keppra®), Zonisamide (Zonegran®), Ethusuximide (Zarontin®)

Seizures seen in persons with Dravet syndrome are difficult to control. The goal of seizure treatment is to find the best combination of medications to treat your child’s long-term condition. Usually two or more medications are needed to treat the multiple seizure types seen with Dravet syndrome.

Some antiseizure medications can make seizures worse and shouldn’t be prescribed. These medications include phenytoin (Dilantin®), carbamazepine (Tegretol®), fosphenytoin (Cerebyx®), lamotrigine (Lamictal®), oxcarbazepine (Trileptal®), rufinamide (Banzel®) and vigabatrin (Sabril®, Sabrilan®, Sabrilex®). Talk with your child’s healthcare provider if their seizures worsen while taking any of these medications.

Other treatments

Your child’s healthcare provider may discuss the use of other treatments that have shown positive results in people with Dravet syndrome. These treatments include:

• Ketogenic diet. This diet is high in fat, adequate in protein and very low in carbohydrates. It’s a diet designed to help manage children with epilepsy. This treatment is typically considered a second-line treatment when medications haven’t helped manage your child’s seizures.
• Vagus nerve stimulation. This treatment uses an implantable device to stimulate your child’s vagus nerve with an electrical impulse. The impulse counters the abnormal electrical activity in their brain that’s causing seizures. This treatment is usually considered after first- and second-line therapies aren’t successful.
• IVIG (intravenous immunoglobulin). This is a solution of antibodies from healthy donors meant to bolster your child’s immune system. An impaired immune system may play a role in triggering epilepsy.

Rescue treatment

Work with your child’s healthcare provider to develop a seizure action plan for home or school. This plan may include rescue medications that are used as an emergency treatment for continuous seizures (status epilepticus). This medication is given to stop your child’s seizure. Rescue medications are usually benzodiazepines, which include:

• Clonazepam (Klonopin®).
• Diazepam (Diastat®, Valtoco®).
• Lorazepam (Ativan®).
• Midazolam (Versed®, Nayzilam®).

Other assessments and therapies

Before starting school, your child should have cognitive and developmental assessments. Based on the results, your child’s school can recommend an Individualized Education Plan (IEP) or your child may qualify for a 504 program (plans and programs that some schools develop to give children with disabilities support and proper accommodations).

Ask your child’s healthcare provider about physical, occupational, speech and social/play therapies for your child, too. Your child may benefit from orthotics for foot and ankle alignment issues or may require orthopedic surgery for bones of the leg or foot due to the physical changes that can occur with Dravet syndrome.