Diacomit

About Stiripentol

• Stiripentol, available as Diacomit, is an anticonvulsant prescribed for the treatment of Dravet syndrome, a severe genetic brain disorder. 
• Indicated for managing seizures associated with Dravet syndrome in patients aged 2 years and older concurrently taking clobazam, Diacomit received FDA approval in August 2018 for this purpose. 
• It’s important to note that there is no clinical data supporting the use of Diacomit as monotherapy in Dravet syndrome.
• While the precise mechanism of action for Stiripentol remains incompletely understood, it is believed to encompass various pharmacological effects. 
• Stiripentol is recognized for its ability to enhance the activity of gamma-aminobutyric acid (GABA), an inhibitory neurotransmitter within the brain. 
• Additionally, Stiripentol influences the functioning of specific ion channels, including sodium and calcium channels, crucial in the initiation and transmission of electrical signals in the brain. 
• Through its impact on these neurotransmitter systems and ion channels, Stiripentol is theorized to exert anticonvulsant effects, rendering it effective in addressing seizures associated with Dravet syndrome.

Strength

• 250 mg or 500 mg capsules
• 250 mg or 500 mg powder for oral suspension

Recommended Dosage:

Before initiating Diacomit treatment, it is essential to conduct hematologic testing. Diacomit capsules must be ingested whole with a glass of water during a meal and should not be broken or opened. For Diacomit Powder for Oral Suspension, it should be mixed in a glass of water (100 mL) and consumed promptly after mixing, also during a meal. To ensure no medication is left in the glass, add a small amount of water (25 mL) to the drinking cup and consume the entire mixture.

• Diacomit is 50 mg/kg/day, administered through the mouth in 2 or 3 divided doses.

Important: 

If a dose is missed, it should be taken promptly. However, if the next scheduled dose is approaching, the missed dose should be omitted, and the regular dosing schedule resumed. It is important not to double doses. In the event of any adverse reactions arising from Stiripentol treatment, patients should promptly seek guidance from their attending physician.

Warnings & Precautions

• Monitor for somnolence, especially if Stiripentol is taken with other CNS depressants; if somnolence develops during co-administration with clobazam, consider a 25% reduction in clobazam at first.
• Patients’ weights and growth rates in pediatric patients should be closely examined. 
• Blood counts should be taken before beginning Diacomit treatment and then every 6 months thereafter (Neutropenia and Thrombocytopenia). 
• To reduce side effects, Stirpentol should be phased down gradually. The risk of increased seizure frequency and status epilepticus. 
• Before recommending Stiripentol powder for suspension to individuals with PKU, consider total daily consumption (Risks in Patients with Phenylketonuria [PKU]).
• Stiripentol treatment may cause suicidal ideas or acts in the patients (Suicidal Behavior and Ideation).

Common Stiripentol Side Effects

• Somnolence
• Reduce appetite
• Agitation
• Ataxia
• Reduced weight
• Hypotonia
• Nausea
• Tremors
• Dysarthria
• Insomnia

Use in specific population 

• Insufficient data are available regarding the developmental risks associated with the use of Diacomit in pregnant women. 
• The presence of stiripentol in human milk, its impact on the breastfed infant, and effects on milk production remain undocumented. 
• The safety and efficacy of Diacomit for treating seizures linked with Dravet syndrome in patients taking clobazam have been established in individuals aged 2 to 18 years. 
• However, the safety and efficacy have not been confirmed in pediatric patients under 2 years old. 
• Notably, clinical studies of Diacomit in Dravet syndrome did not encompass patients aged 65 years or older, precluding determination of potential age-related differences in treatment response. 
• Consideration should be given to age-associated hepatic and renal function abnormalities when employing Diacomit in patients aged 65 years or older.

Storage and Handling

To protect them from light, store at 20°C to 25ºC. Excursions are permitted between 15ºC to 30ºC