Dravet Syndrome in Adults

Transition Document

Characteristics of Adult Patients

Dravet syndrome is a rare, catastrophic, lifelong form of epilepsy that begins in the first year of life with frequent and/or prolonged seizures. Previously known as Severe Myoclonic Epilepsy of Infancy (SMEI), it affects 1:15,700 individuals, 80% of whom have a mutation in their SCN1A gene. Adult patients suffer multiple co-mordities in addition to the seizures, including but not limited to cognitive disabilities, behavioral challenges, decreased mobility, sleep issues, and gastrointestinal issues.

Characteristics of Dravet syndrome often change in adulthood. A different therapeutic approach may be needed as the patient ages and new or increased care may be required. Seizure types often change and may decrease in frequency, however the majority of adult patients still experience regular seizures. Studies on behavior problems are lacking for this population, but anecdotal reports from caregivers tell us that older patients may experience irritability, aggressiveness, and other behavioral problems that need to be addressed to keep the patient and caregiver safe. Current treatment options are limited and the constant care required for someone suffering from Dravet syndrome can severely impact the patient’s and the caregiver’s quality of life. Patients with Dravet syndrome face a 15-20% mortality rate due to SUDEP (Sudden Unexpected Death in Epilepsy), prolonged seizures, seizure-related accidents such as drowning, and infections.

Adults living with Dravet syndrome (DS) have complex medical needs and require comprehensive care and community support. Top community needs include:

  • A correct diagnosis, at any age
  • Knowledgeable adult neurologists who understand the complexities of care for older patients
  • Pediatric-to-Adult Transition Clinics
  • Comprehensive care for co-morbidities

DSF offers resources and information for families and healthcare professionals and seeks to better define and understand the adult patient experience and needs.

Connect with other caregivers of adults with Dravet syndrome

Survey Results

In October 2018, DSF conducted a survey of caregivers regarding needs and gaps in care for adult patients with Dravet syndrome.

Top results included:

“Dravet syndrome is a disease that dramatically and permanently impacts patients, and their families, at every stage of life. As patients age, there is an even greater need for medical and community supports and resources to ensure a safe and meaningful quality of life for patients and those who care for them.”      

– Barbara, mother to Jake, age 23 with DS

For Providers

For Families

Special thanks to our adult caregiver community, especially the members of the
DSF Caregivers of Adults with Dravet Syndrome Advisory Group:

Sally Gaer, Committee Chair
Michele Calahan-Williams
Sharon Canino
Guida Clozza
Leslie Moccia
Lisa Smith
Barbara Swoyer