Stiripentol Use in Children with Ds

Stiripentol Use in Children with Dravet Syndrome
Data Source: Dravet Syndrome Patient Registry July 2008
Number of completed surveys: 22
Number of children using stiripentol: 8
Michelle Welborn, PharmD
September 2008

This research was generated by the preliminary Dravet syndrome patient registry launched in 2008 and was submitted to be used for application of Orphan Drug Designation for stiripentol in the United States. Stiripentol was granted Orphan Drug Designation in November 2008.
Current age of child:

Race:

How long has your child been on this regimen?

Please describe how seizure control now compares to other times during your child’s life:  (select all that apply)

Please indicate which seizure types your child currently still experiences:  (select all that apply)

My child still has the following seizure types that last more than 30 minutes despite appropriate use of rescue medications:

Please indicate your native country:

Please select the option below that best describes your expectation of “good” seizure control in your child:

Please indicate the number of myoclonic jerks your child has currently in the time periods stated below:

How often is your child experiencing any seizure type except for myoclonic jerks or atypical absence in the form of eyelid flutters (blinks)?

How many episodes of status epilepticus (> 30 minutes with appropriate use of rescue medications) is has your child had in the last six months?

My child tested SCN1A positive:

My child has used clobazam as an anti-seizure medication:

Child’s neurologist had no knowledge of stiripentol until I educated him/her:

Child’s neurologist had no knowledge of stiripentol until I educated him/her:

Experience with stiripentol: Please select all that apply

Please select from the list below the targeted total daily starting dose of stiripentol (first established dose after increasing to this dose over a few days)

Example:  Elliot took 250 mg of stiripentol three times a day = total daily dose = 750 mg

Please select from the list below the final total daily dose of stiripentol that was effective and tolerated by the child

Example:  Elliot took 250 mg of stiripentol three times a day = total daily dose = 750 mg.  After a month, Elliot presented with a one hour episode of status epilepticus.  Neurologist increased the dose to 500 mg twice a day and 250 mg at bedtime over the next three months.  Total daily dose was then 1250 mg.

Seizure types that were improved by stiripentol (Diacomit):

Please select all that apply

I had to decrease my child’s prescribed dose of stiripentol due to the price increase effective October 2007:

Please select all that apply regarding the dose reduction of stiripentol due to price increase:

I could not afford stiripentol after the price increase effective October 2007 and our child had to stop taking this medication:

My child’s quality of life improved when he/she used stiripentol:

My family’s quality of life improved when my child used stiripentol:

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