Stiripentol

What is Stiripentol?

Stiripentol (DIACOMIT) used along with clobazam to treat seizures in people 2 years or older with Dravet syndrome.

What are the uses of this medicine?

• This medicine is used for the treatment of seizures associated with Dravet syndrome in patients 2 years of age and older taking clobazam.
• DIACOMIT has not been shown to be able to treat seizures associated with Dravet syndrome without the use of clobazam.

How does this medicine work?

• The mechanism by which DIACOMIT exerts its anticonvulsant effect in humans is unknown.
• Possible mechanisms of action include direct effects mediated through the gamma-aminobutyric acid (GABA)A receptor and indirect effects involving inhibition of cytochrome P450 activity with resulting increase in blood levels of clobazam and its active metabolite.

Who Should Not Use this medicine ?

• This medicine have no usage limitations.

What drug interactions can this medicine cause?

• DIACOMIT increases the plasma concentration of clobazam and its metabolite through metabolic inhibition of CYP3A4 and CYP2C19. Consider dose reduction of clobazam in case of adverse reactions.
• Because of potential drug-drug interactions, consider dose adjustment of CYP1A2 substrates (e.g., theophylline, caffeine), CYP2B6 substrates (e.g., sertraline, thiotepa), and CYP3A4 substrates (e.g., midazolam, triazolam, quinidine), as clinically appropriate, when administered concomitantly with DIACOMIT.
• Consider a reduction in dosage of substrates of CYP2C8, CYP2C19 (e.g., diazepam, clopidogrel), P-gp (e.g., carbamazepine), and BCRP (e.g., methotrexate, prazosin, glyburide), if adverse reactions are experienced when administered concomitantly with DIACOMIT.
• Concomitant use of strong CYP1A2, CYP3A4, or CYP2C19 inducer, such as rifampin, phenytoin, phenobarbital and carbamazepine, as these enzymes all metabolize stiripentol with DIACOMIT should be avoided, or dosage adjustments should be made.
• Concomitant use of DIACOMIT with other CNS depressants, including alcohol, may increase the risk of sedation and somnolence.

Is this medicine FDA approved?

• It was approved for use in the United States in 2018.

How should this medicine be used?

• Hematologic testing should be obtained prior to starting treatment with DIACOMIT.

Recommended Dosage

• The dosage of DIACOMIT is 50 mg/kg/day, administered by mouth in 2 or 3 divided doses.
• Reduce dose or discontinue dose gradually.

Administration

• Take DIACOMIT exactly as your healthcare provider tells you to take it.
• Your healthcare provider will tell you how much DIACOMIT to take and when to take it.
• DIACOMIT capsules should be swallowed whole with a glass of water during a meal. Do not break or open capsules.
• DIACOMIT powder for oral suspension should be mixed in a glass of water (100 mL) and should be taken right away after mixing during a meal. To be sure there is no medicine left in the glass, add a small amount of water (25 mL) to the drinking cup and drink all of the mixture. See the complete Instructions for Use on the right way to use DIACOMIT powder for oral suspension.
• Your healthcare provider may change your dose if needed. Do not change your dose of DIACOMIT without talking to your healthcare provider.
• If you miss a dose of DIACOMIT, take it as soon as you remember. If it is almost time for the next scheduled dose, skip the missed dose and take your next dose at your regular time. Do not take 2 doses of DIACOMIT at the same time.
• If you take too much DIACOMIT, call your healthcare provider or go to the nearest hospital emergency room right away.

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form:

• As capsule: 250 mg or 500 mg
• As powder for oral suspension: 250 mg or 500 mg

This medicine is available in fallowing brand namesː

• DIACOMIT

What side effects can this medication cause?

The most common side effects of this medicine include:

• agitation
• loss of control of body movements (ataxia)
• low muscle tone or muscle weakness (hypotonia)
• nausea
• tremor
• trouble saying words clearly (dysarthria)
• trouble sleeping (insomnia)

DIACOMIT may cause serious side effects, including:

• Sleepiness and drowsiness
• Loss of appetite and weight loss
• Low white blood cell counts (neutropenia) and low platelet counts (thrombocytopenia)

What special precautions should I follow?

• Do not drive, operate heavy machinery, or do other dangerous activities that need you to be alert until you know how DIACOMIT affects you.
• Do not drink alcohol or take other medicines that may make you sleepy or dizzy while taking DIACOMIT without first talking to your healthcare provider.
• DIACOMIT can cause somnolence. Monitor for somnolence, particularly when DIACOMIT is used concomitantly with other CNS depressants; If somnolence occurs during co-administration with clobazam, consider an initial reduction of clobazam by 25%.
• DIACOMIT can cause decreases in appetite and weight. The weight of patients and the growth rate of pediatric patients should be carefully monitored.
• DIACOMIT can cause a significant decline in neutrophil and platelet count.
• As with most antiepileptic drugs, DIACOMIT should generally be withdrawn gradually to minimize the risk of increased seizure frequency and status epilepticus. Blood counts should be obtained prior to starting treatment with DIACOMIT and then every 6 months.
• Phenylalanine can be harmful to patients with phenylketonuria (PKU). DIACOMIT Powder for Suspension contains phenylalanine, a component of aspartame. Consider total daily intake before prescribing to patients with PKU.
• AEDs, including DIACOMIT, increase the risk of suicidal thoughts or behavior in patients taking these drugs for any indication. Monitor for suicidal thoughts or behaviors.

What to do in case of emergency/overdose?

• There are no data concerning overdose in humans.

Management for overdosage:

• Treatment of an overdose should be supportive (symptomatic measures in intensive care units).
• For management of a suspected drug overdose, contact your regional Poison Control Center.

Can this medicine be used in pregnancy?

• There are no adequate data on the developmental risks associated with the use of DIACOMIT in pregnant women.
• There is a pregnancy exposure registry that monitors pregnancy outcomes in women exposed to AEDs, such as DIACOMIT, during pregnancy.
• This can be done by calling the toll free number 1-888-233-2334.

Can this medicine be used in children?

• The safety and effectiveness of DIACOMIT for the treatment of seizures associated with Dravet syndrome in patients taking clobazam have been established in patients 2 to 18 years of age.
• Safety and effectiveness in pediatric patients below the age of 2 years have not been established.

What are the active and inactive ingredients in this medicine?

• Active ingredient: stiripentol
• Capsule inactive ingredients: erythrosine (250 mg capsule only), gelatin, indigotine (250 mg capsule only), magnesium stearate, povidone, sodium starch glycolate, titanium dioxide.
• Powder for oral suspension inactive ingredients: aspartame, carmellose sodium, erythrosine, glucose, hydroxyethylcellulose, povidone, sodium starch glycolate, sorbitol, titanium dioxide, tutti frutti flavor.

Who manufactures and distributes this medicine?

Manufactured by:

• BIOCODEX, 1 avenue Blaise Pascal, 60000 BEAUVAIS – France

What should I know about storage and disposal of this medication?

• Store DIACOMIT at room temperature between 68°F to 77°F (20°C to 25°C).
• Store in a dry place in the original package.
• Protect from light.
• Keep DIACOMIT and all medicines out of the reach of children.

• Dailymed label info on Stiripentol
• FDA Stiripentol

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