司替戊醇(Diacomit)的用法用量,注意事项及药品价格？

The recommended oral dose of (Diacomit) is 50 mg/kg/day given in 2 or 3 divided doses (i.e. 16.67 mg/kg 3 times daily or 25 mg/kg Twice daily), depending on the patient's age and weight, cautions such as drowsiness, decreased appetite and weight loss, neutropenia and thrombocytopenia, withdrawal symptoms, suicidal behavior and ideation are required when using stiripentol.

It is understood that the reference price of Diacomit (stiripentol oral suspension) in the specification of 250 mg*60 packets is about $3,300 per box, and the reference price in the specification of 500 mg*60 packets is about $6,000.

About stiripentol (Diacomit)

Stipentol, also known as dioxenanol, is an allosteric modulator of GABA receptors. The drug has a complex mechanism of action, including directly acting on GABA receptors, inhibiting the activity of cytochrome P450, increasing the blood concentration of other ASMs, and having neuroprotective effects. It is effective for convulsive seizures, focal seizures with disturbance of consciousness, myoclonic seizures, atypical absence seizures and prolonged seizures. In July 2022, the U.S. Food and Drug Administration (FDA) approved stiripentol as an add-on treatment for patients with Dravet syndrome (DS) who are 6 months old and older and weigh 7 kg or more and are also taking CLB.

Stipentol (Diacomit) Dosage

The recommended dose of DIACOMIT is 50 mg/kg/day, administered in 2 or 3 divided doses (i.e., 16.67 mg/kg 3 times daily or 25 mg/kg 2 times daily), depending on the patient's age and weight, as shown in Table 1. If the exact dose cannot be achieved given the available strengths, round to the nearest possible dose, usually within the range of 50 mg to 150 mg of the recommended dose of 50 mg/kg/day. A combination of two DIACOMIT strengths can be used to achieve this dose. The maximum recommended total dose is 3,000 mg/day.

Table 1: Recommended doses for Dravet syndrome patients 6 months and older weighing ≥7 kg

Precautions for stiripentol (Diacomit)

1. Drowsiness

DIACOMIT can cause drowsiness.

Treatment measures:

(1) Prescribers should monitor patients for drowsiness. If drowsiness occurs during concurrent administration with clobazam, consider a 25% reduction in the initial clobazam dose. If somnolence persists, a further reduction of clobazam by 25% should be considered and the dose of other concomitant anticonvulsants with sedating properties should be adjusted.

(2) Prescribers should remind patients not to engage in hazardous activities that require mental alertness, such as operating dangerous machinery or motor vehicles, until the effects of DIACOMIT on mental alertness are known.

2. Decreased appetite and weight loss

DIACOMIT can cause appetite and weight loss.

Action: Pediatric patients receiving DIACOMIT should be carefully monitored for growth. In some cases, reducing the concomitant valproate dose by 30%/week may reduce appetite and weight loss.

3. Neutropenia and Thrombocytopenia

DIACOMIT can cause a significant decrease in neutrophil count.

Action: Hematology tests should be performed before starting DIACOMIT therapy and then every 6 months.

4. Withdrawal symptoms

Like most anti-epileptic drugs, DIACOMIT should usually be discontinued gradually to minimize the risk of increased seizure frequency and status epilepticus.

Action: In situations where rapid discontinuation of DIACOMIT is required (e.g., in the case of serious adverse reactions), appropriate monitoring is recommended.

5. Suicidal Behavior and Ideation

AEDs (including DIACOMIT) increase the risk of suicidal thoughts or behaviors in patients taking these drugs for any indication.

Action: Patients receiving any AED for any indication should be monitored for the emergence or worsening of depression, suicidal thoughts or behaviors, and/or any unusual changes in mood or behavior.

The price of stiripentol (Diacomit)

It is understood that the reference price of Diacomit (stiripentol oral suspension) for 250 mg*60 packs is about $3,300 per box, and the reference price for 500 mg*60 packs is about $6,000.

Stipentol is currently on the market in China, but as of January 2024, the drug has not been included in the national medical insurance. Therefore, patients can choose to purchase it domestically, or they can choose to trust domestic and overseas medical service institutions to help them purchase it.