Usual Adult Dose for Dravet Syndrome

25 mg/kg orally twice a day or 16.67 mg/kg orally 3 times a day
Maximum dose: 3000 mg/day

Comments:

• No clinical data are available to support the use of this drug as monotherapy in Dravet syndrome.
• The total daily dose is 50 mg/kg/day.

Usual Pediatric Dose for Dravet Syndrome

6 months to less than 1 year:

• Weight at least 7 kg: 25 mg/kg orally twice a day

• Weight 7 to less than 10 kg: 25 mg/kg orally twice a day
• Weight at least 10 kg: 25 mg/kg orally twice a day or 16.67 mg/kg orally 3 times a day

• No clinical data are available to support the use of this drug as monotherapy in Dravet syndrome.
• The total daily dose is 50 mg/kg/day.
• For patients 6 months to less than 1 year: Dosing frequency should not exceed twice a day to limit free water administration and to avoid overexposures.
• For patients 1 year and older weighing 7 to less than 10 kg: Dosing frequency should not exceed twice a day to avoid overexposures.

Renal Dose Adjustments

Moderate or severe renal dysfunction: Not recommended

Liver Dose Adjustments

Moderate or severe liver dysfunction: Not recommended

Precautions

CONTRAINDICATIONS: None

Safety and efficacy have not been established in pediatric patients weighing less than 7 kg or younger than 6 months.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• Obtain hematologic testing before starting this drug.
• If the exact dosage is not achievable given the available strengths, round to the nearest possible dosage, which is usually within 50 to 150 mg of the recommended 50 mg/kg/day.
• A combination of the available strengths can be used to achieve this dosage.
• Administer 50 mg/kg/day by mouth in 2 or 3 equally divided doses, depending on patient's age and body weight.
• Swallow capsules whole with a glass of water during a meal; do not break or open capsules.
• Mix the powder for oral suspension in a glass of water (100 mL) and take immediately after mixing during a meal; to ensure no medicine is left in the glass, add a small amount of water (25 mL) to the drinking cup and drink the entire mixture.
• As for most antiepileptic drugs, if this drug is discontinued, it should be withdrawn gradually to minimize the risk of increased seizure frequency and status epilepticus; if rapid withdrawal is medically required, monitor appropriately.

• Store in a dry place at 20C to 25C (68C to 77F); excursions permitted to 15C to 30C (59F to 86F).
• Store in original package to protect from light.

• The powder for oral suspension contains phenylalanine; before prescribing this formulation to a patient with phenylketonuria, the combined daily amount of phenylalanine from all sources (including this product) should be considered.
• The capsules do not contain phenylalanine.

• Hematologic: Hematologic testing (before starting therapy and then every 6 months)
• Metabolism: Growth of pediatric patients
• Nervous System: For somnolence
• Psychiatric: For emergence/worsening of depression, suicidal thoughts/behavior, and/or any unusual changes in mood/behavior

• Read the US FDA-approved patient labeling (Medication Guide and Instructions for Use).
• Somnolence may occur and may require a decrease in the clobazam dose; do not consume alcohol during therapy. Until you know how this drug affects you, do not engage in hazardous activities requiring mental alertness (e.g., operating dangerous machinery or motor vehicles).
• Do not discontinue this drug without consulting health care provider.
• It is important to obtain hematologic testing before starting this drug and then every 6 months.
• It is important to be alert for the emergence or worsening of symptoms of depression, any unusual changes in mood or behavior, or the emergence of suicidal thoughts/behavior or thought of self-harm. Report behaviors of concern immediately to health care providers.
• Notify health care provider if you become pregnant or intend to become pregnant during therapy. If you become pregnant, you are encouraged to enroll in the North American Antiepileptic Drug Pregnancy Registry.
• Notify health care provider if you are breastfeeding or intend to breastfeed during therapy.
• Take a missed dose as soon as possible; if it is almost time for the next dose, do not take the missed dose but resume taking this drug with the next scheduled dose. Do not double doses.

Frequently asked questions

• What type of epilepsy is Diacomit used to treat?