Monitoring and Switching Therapies in Patients With Dravet Syndrome

EP: 1.Symptoms and Disease Progression in Pediatric Patients With Dravet Syndrome

EP: 2.Role of SCN1A Genetic Testing in Diagnosis of Dravet Syndrome

EP: 3.Risk Factors and Risk Mitigation Strategies of SUPED for Patients With Dravet Syndrome

EP: 4.Standard of Care and Dravet Syndrome–Specific ASMs: Mechanisms and Risk Management

EP: 5.Treatment Selection and Treatment Adherence in Dravet Syndrome

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EP: 6.Monitoring and Switching Therapies in Patients With Dravet Syndrome

Evolution of Traditional Medication Use

Clinicians have shifted their approach to traditional Dravet syndrome medications such as valproate and clobazam based on experience with newer therapies. While valproate was previously favored for young children to address febrile status epilepticus, many specialists now find stiripentol more effective for this indication. The historical concern about valproate-related hepatic failure in children younger than 2 years primarily affected patients with POLG variants, which genetic testing for SCN1A typically rules out. Consequently, clinicians increasingly prefer starting with newer medications and clobazam in younger patients, reserving valproate for specific situations such as rescue therapy after status epilepticus or when absence seizures become prominent later in the course of the disease.

Treatment Assessment Timeline and Monitoring

Effective therapy evaluation follows a structured timeline with clear expectations communicated to families up front. Most newer medications require 4 to 6 weeks to titrate to reasonable maintenance doses, with follow-up appointments scheduled 6 to 8 weeks after initiation to assess effectiveness. Clinicians emphasize that while some medications provide dramatic "home run" responses with complete seizure cessation, even 75% improvement warrants continued optimization efforts. The goal remains pushing treatment effectiveness beyond "pretty good" control, as patients experiencing 1 to 2 seizures weekly can often achieve better outcomes with current therapeutic options. Regular communication between appointments is encouraged to address problems promptly rather than waiting months to make necessary adjustments.

Optimization Strategies and Communication

Treatment optimization involves ongoing assessment of whether medication adjustments, dose increases, or discontinuation of less effective drugs can improve seizure control. When patients achieve excellent seizure control or seizure freedom, clinicians consider reducing unnecessary medications to simplify regimens. However, the process requires active communication, as clinicians cannot effectively treat patients who experience months of suboptimal seizure control without reporting problems. Most medications demonstrate their effectiveness within 2 months, making extended observation periods without contact inappropriate and potentially harmful to patient outcomes.