Nurses Compass
Drug Guide
Stiripentol
Classification
Therapeutic: Anticonvulsant
Pharmacological: GABAergic agent
FDA Approved Indications
- Treatment of seizures associated with Dravet syndrome in pediatric patients 2 years of age and older.
Mechanism of Action
Stiripentol enhances GABAergic transmission by inhibiting the enzymatic degradation of GABA, increasing GABA levels in the brain, and modulating neuronal excitability to reduce seizure activity.
Dosage and Administration
Adult: Not approved for adult use; dosing primarily for pediatric patients with Dravet syndrome.
Pediatric: Initial dose: 50 mg/kg/day divided into three doses. Dose may be titrated based on response and tolerability. Maximum dose: 100 mg/kg/day.
Geriatric: Limited data; use with caution and appropriate dose adjustments.
Renal Impairment: No specific dosage adjustment recommended; monitor closely.
Hepatic Impairment: Use with caution; monitor for adverse effects, as hepatic metabolism is involved.
Pharmacokinetics
Absorption: Rapid absorption after oral administration.
Distribution: Widely distributed; high plasma protein binding.
Metabolism: Extensively metabolized in the liver, primarily via glucuronidation.
Excretion: Primarily via urine as metabolites.
Half Life: Approximately 4-7 hours.
Contraindications
- Hypersensitivity to stiripentol or any component of the formulation.
Precautions
- Monitor liver function during therapy.
- Use with caution in patients taking medications that affect hepatic enzyme activity.
- Potential for anemia; monitor blood counts.
- Careful use in patients with concomitant medications that can cause CNS depression.
Adverse Reactions - Common
- Somnolence (Common)
- Decreased appetite (Common)
- Diarrhea (Common)
- Vomiting (Common)
Adverse Reactions - Serious
- Liver enzyme elevation (Serious; monitor liver function)
- Hematologic abnormalities (e.g., anemia) (Serious)
- Severe hypersensitivity reactions (Rare)
Drug-Drug Interactions
- CYP450 enzyme inducers or inhibitors may alter stiripentol levels.
- Other CNS depressants may have additive sedative effects.
Drug-Food Interactions
- No specific food interactions identified.
Drug-Herb Interactions
- Limited data; consult healthcare provider before use of herbal supplements.
Nursing Implications
Assessment: Monitor seizure frequency, liver function tests, blood counts, and signs of adverse effects.
Diagnoses:
- Risk for injury related to seizures or adverse effects.
- Impaired liver function.
Implementation: Administer with food to reduce gastrointestinal upset; educate caregivers about adherence and monitoring.
Evaluation: Assess seizure control, adverse effects, and laboratory parameters regularly.
Patient/Family Teaching
- Take medication exactly as prescribed; do not adjust dose without consulting healthcare provider.
- Report signs of liver dysfunction (jaundice, dark urine), unusual bleeding, or severe rash.
- Be aware of potential drowsiness; avoid activities requiring alertness until tolerated.
- Maintain regular follow-up appointments for laboratory testing.
Special Considerations
Black Box Warnings:
- Potential to cause increased liver enzymes and hematologic abnormalities.
Genetic Factors: No specific genetic factors identified affecting dosing.
Lab Test Interference: May elevate liver function tests; interpret with clinical context.
Overdose Management
Signs/Symptoms: Seizures, somnolence, gastrointestinal symptoms, hepatic dysfunction.
Treatment: Supportive care, activated charcoal if ingestion is recent, monitor vital signs, and provide symptomatic management. Dialysis is not effective for stiripentol elimination.
Storage and Handling
Storage: Store at room temperature away from moisture and light.
Stability: Stable for recommended shelf life when stored properly.