Treatment Selection and Treatment Adherence in 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

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

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

Treatment Goals and Medication Selection Approach

Clinicians emphasize that seizure freedom should remain the primary treatment goal for patients with Dravet syndrome, despite the rarity of achieving complete seizure control in this population. The initial treatment approach involves immediately discontinuing any contraindicated sodium channel medications, then selecting appropriate therapies based on predominant seizure types. For patients experiencing frequent convulsive seizures or status epilepticus, clinicians prioritize stiripentol and cannabidiol due to their proven efficacy in preventing prolonged seizures. When absence seizures predominate, treatment focuses on optimizing valproate dosing or considering ethosuximide. Drug selection must also consider patient age, as not all medications are approved for children younger than 2 years, along with individual adverse effect profiles and monitoring requirements such as periodic echocardiograms for fenfluramine.

Strategic Drug Combinations and Interactions

Experienced clinicians recognize that certain medication combinations can provide synergistic benefits when managed appropriately. The combination of clobazam with cannabidiol creates beneficial drug interactions where elevated metabolites from both medications enhance antiseizure efficacy, though this requires careful dose adjustments to prevent toxicity. Similarly, stiripentol pairs well with fenfluramine, potentially improving efficacy while minimizing adverse effects. However, these combinations require expert management: Stiripentol necessitates reducing valproate and clobazam doses, while fenfluramine dosing must be lowered when combined with stiripentol. Additionally, stiripentol and cannabidiol demonstrate optimal absorption when administered with food, a detail that can significantly impact treatment effectiveness.

Adherence Strategies and Family-Centered Planning

Given the complexity of multidrug regimens often required in Dravet syndrome, clinicians prioritize simplification strategies to improve adherence. This includes preferring twice-daily dosing schedules, using round numbers for dosing measurements, and timing medications with regular activities such as meals. Most importantly, treatment plans must be tailored to individual family circumstances and lifestyles. Clinicians emphasize the importance of asking families whether proposed regimens are realistically manageable, then adjusting plans based on family feedback regarding work schedules, caregiving responsibilities, and daily routines to ensure optimal medication adherence.