Treatment and Management of Dravet Syndrome

This is an exciting and hopeful time for Dravet Syndrome, with much ongoing research into new medicines and treatments. As treatments improve, along with understanding of the condition, researchers expect that long-term outcomes for people with Dravet Syndrome will improve.

Children and adults with Dravet Syndrome typically need a combination of anti-seizure medications (ASMs) to help control or reduce seizures. Currently, Dravet Syndrome remains one of the most treatment resistant medications and good seizure control is rare, with very few children/adults living seizure-free.

Because Dravet Syndrome is a spectrum condition, not all children/adults respond in the same way to treatments and what ‘good seizure control’ looks like, may vary from person to person.

Essential reading:

In recent years, there have been a number of updated guidelines, consensus statements and other publications outlining the appropriate treatment algorithm for Dravet Syndrome. These are listed below with links to the publications:

• NICE guidelines - Dravet Syndrome is section 6.1: National Institute for Health and Care Excellence, Epilepsies in children, young people and adults, 2022.
  
• The NICE Technology Appraisal for fenfluramine (Fintepla) was completed after the NICE guidelines publication: Fenfluramine for treating seizures associated with Dravet syndrome, 2022.
• In 2022, the ILAE updated the classification and definition of Dravet Syndrome: Sameer M. Zuberi et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions, Epilepsia 2022
• European consensus statement and roadmap: Elena Cardenal-Muñoz et al. 'Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe' Epilepsia, 2021.
• Treatment algorithm, including rescue medication: Helen J. Cross et al. 'Dravet syndrome: Treatment options and management of prolonged seizures' Epilepsia, 2020.
• North American consensus: Elaine C. Wirrell et al. International consensus on diagnosis and management of Dravet syndrome, Epilepsia 2022.

IMPORTANT: Medications to avoid when treating seizures

In children with Dravet Syndrome, sodium channel blockers should be avoided: including lamotrigine, phenytoin, carbamazepine, oxcarbazepine, lacosamide and rufinamide. 

This is because they may increase or prolong seizures and with long-term use, may have an adverse impact on intellectual development.  

In adults, sodium channel blockers should only be used in caution. 

In some cases, adults with Dravet Syndrome have responded to sodium channel blockers, but overall there is not enough evidence of their effect (and few randomised controlled trials have included adults). However, if a young person or adult receives a new diagnosis and is taking sodium channel blockers, there may be benefit in weaning this medication to see if it improves seizures or other comorbidities. 

Interdisciplinary management

Dravet Syndrome is more than seizures. Effective management of Dravet Syndrome involves multidisciplinary care in addition to drug treatment tailored to the specific needs of each patient. For example, in children, expressive and receptive language is often impaired; early intervention with speech therapy optimises potential. Ensure developmental assessments begin as early as possible and are repeated regularly. Adults also benefit from additional therapies; all too often these therapies can become difficult to access following transition from paediatric to adults services.

In their 2022 international consensus statement Cardenal-Muñoz et al, describe the ideal multidisciplinary team in charge of a Dravet Syndrome patient as being formed by a (child) neurologist, a nurse, a neuropsychologist, a physiotherapist, a speech therapist, a dental practitioner, and an occupational therapist, among others. In addition to this team, patient management should be supported by special education in regular or specialized centers, as well as by a dedicated group of patient advocates.