How DIACOMIT can help you on your treatment journey

We know that caring for a child with Dravet syndrome can be a challenging path to walk. Explore this site to find answers to questions you may have about Dravet syndrome, from diagnosis and treatment to lifestyle management.

About DIACOMIT

DIACOMIT is a trusted Dravet syndrome treatment that comes in two convenient dosing forms
DIACOMIT is a trusted Dravet syndrome treatment that comes in two convenient dosing forms

Easy treatment access

The most common side effects are sleepiness, decreased appetite, agitation, impaired coordination, decreased weight, decreased muscle tone, nausea, tremor, slurred speech, and trouble sleeping.

DIACOMIT is indicated for the treatment of seizures associated with Dravet syndrome in patients 2 years of age and older taking clobazam. There are no clinical data to support the use of DIACOMIT by itself in Dravet syndrome.

Talk to your child’s doctor about DIACOMIT.

All about Dravet syndrome

Learning the lay of the land

When your child with Dravet syndrome graduates from being a toddler to a child, life can feel uncertain and full of new roadblocks. Enter this section to learn more about Dravet syndrome progression and get an overview of the challenges you may face as your child gets older.

Not actual patients

What is Dravet syndrome?

Dravet (pronounced “drah-VAY”) syndrome is a lifelong disease that usually appears in the first year of life, when an otherwise normally developing child begins having frequent seizures. This can be frightening and disorienting, with the child experiencing long seizures often triggered by fever or changes in temperature.

Seizures may cause lasting damage, which over time can lead to cognitive and physical developmental delays. The primary goal of Dravet syndrome treatment is to reduce the frequency and length of seizures.

Unfortunately, seizures associated with Dravet syndrome can be resistant to treatment—multiple therapies and frequent regimen adjustments are often needed to control seizures.

Dravet syndrome:

  • Is a rare and treatment-resistant form of epilepsy that often develops in the first year of life
  • Occurs in 1 in 15,700 births in the United States
  • Is most often caused by a genetic mutation that usually occurs “de novo” (or “new”)—neither parent carries the mutation

Dravet syndrome diagnosis

For children suffering from seizures, understanding the underlying cause can help guide more precise treatment. Earlier treatment could potentially contribute to better long-term outcomes.

Many children with Dravet syndrome are initially misdiagnosed. The patient’s initial brain scan may appear normal or their symptoms might lead doctors to diagnose a different form of epilepsy. Though not 100% conclusive, genetic testing is highly recommended.

Genetic testing is available at no cost for children up to the age of 8 through the Behind the Seizure® program at Invitae.com.

Genetic causes of Dravet syndrome:

  • Dravet syndrome is most often caused by an abnormality in a gene called SCN1A
  • The SCN1A gene is involved in transmitting signals between nerve cells
  • The SCN1A mutation is seen in at least 75% of all Dravet syndrome patients
  • Some patients will have a mutation in the gene and not have seizures; other patients without the gene mutation might have seizures that look like Dravet syndrome
  • Around 5%-10% of cases are inherited, meaning that one of the parents will test positive for the same SCN1A gene mutation, but will not have the disease themself
  • Other genetic abnormalities have also been associated with Dravet syndrome. Genes not yet identified may be involved as well

What to expect with Dravet syndrome

While each child will have a different experience, children with Dravet syndrome will have seizures and developmental delays ranging from mild to severe. Once your child reaches preschool or school age, you may notice that their seizures occur more frequently during sleep than while awake. Seizures often become shorter in duration, but they may happen in clusters. The types of seizures experienced by your child may also change.

Slowing of development usually becomes noticeable at preschool age. Behavior and attention problems may also begin, and your child may start showing signs of hyperactivity. Coordination issues may also appear around age 5.

Your primary source of care and information will be your child’s neurologist. Do not hesitate to discuss any concerns you may have with them.

Seizures and developmental issues in children

For many children, seizures reach their highest frequency during these years, and may be accompanied by new developmental issues.

Generalized tonic-clonic seizures

  • Seizures involving the entire body
  • Generalized stiffening followed by rhythmic jerking

Myoclonic seizures

  • Sudden, very brief twitching movements, sometimes repeated, generally of moderate intensity

Focal impaired awareness seizures

  • Unresponsive staring or apparent daydreaming
  • Fiddling hands
  • Chewing or other mouth motions
  • Sleepiness

Atypical absence seizures

  • Brief periods of staring
  • Decreased responsiveness

Complications

  • Slowing of developmental progression
  • Delayed language development
  • Poor appetite
  • Decreased muscle tone
  • Inattention
  • Hyperactive behavior
  • Sleep difficulties
  • Poor coordination
  • Crouched gait
  • Sudden Unexpected Death in Epilepsy (SUDEP)

Triggers differ from one child to the next. Possible triggers include:

  • Lack of sleep
  • Fever and/or illness

  • Intense exercise
  • Loud music

  • Flashing lights
  • Stress or emotional upset

Navigating Life with Dravet Syndrome information booklet available to download

Download our Navigating Life with Dravet Syndrome information booklet for answers to questions you may have after your child’s Dravet syndrome diagnosis and later down the road.

Learn more about lifestyle decisions you can make to support your child.

Be sure to consult your doctor before making dietary or other changes to your child’s treatment plan.

How DIACOMIT can help

Effective seizure relief

DIACOMIT offers a chance to significantly decrease the number of seizures in patients with Dravet syndrome. Learn more about the efficacy and safety of DIACOMIT and see if it could be the right next step for your child.

DIACOMIT—designed specifically for Dravet syndrome

DIACOMIT is an FDA-approved antiepileptic treatment that is used along with clobazam, another type of antiepileptic drug, to help reduce the number of seizures in patients with Dravet syndrome. There are no clinical data to support the use of DIACOMIT by itself in Dravet syndrome.

20 YEARS OF REAL-WORLD USE

DIACOMIT was designated as an orphan drug for use in Europe in 2001 and granted approval by the FDA in August 2018 for the treatment of Dravet syndrome seizures.

Proven seizure reduction in clinical studies

In 2 placebo-controlled clinical studies, patients were treated with either DIACOMIT plus clobazam or placebo plus clobazam.

While the exact mechanism of action is unknown, DIACOMIT is believed to help reduce seizures through:

  • Direct effects that are caused through the gamma aminobutyric acid (GABA)A receptors in the brain
  • Indirect effects that inhibit enzyme activity, which leads to increased blood levels of clobazam
DIACOMIT reduced the frequency of seizures by at least half in 7 out of 10 patients
DIACOMIT reduced the frequency of seizures by at least half in 7 out of 10 patients

How to take DIACOMIT

Please read, understand, and follow the Instructions for Use before preparing your child’s first dose of DIACOMIT and each time you get a refill. Download the instructions here.

DIACOMIT is available in two convenient dosing forms: capsules and a fruit-flavored powder for oral suspension to be mixed in water.

DIACOMIT is available in two strengths: 250 mg and 500 mg. Your doctor will tell you the appropriate dose of DIACOMIT for your child and how many capsules/packets are needed for each dose.

DIACOMIT can be stored at room temperature, so you can take it with you on day trips or longer vacations, for example.

Common side effects

The most common side effects with DIACOMIT are sleepiness, decreased appetite, agitation, impaired coordination, decreased weight, decreased muscle tone, nausea, tremor, slurred speech, and trouble sleeping.

Talk to your child’s doctor about DIACOMIT.

Daily life with Dravet syndrome

Working together as a family

Every person will have a different experience with Dravet syndrome, so it’s important to become familiar with your child’s individual needs and to design a lifestyle around them. Building routines and helping reduce your child’s risk of seizures are top priorities.

Not actual patients

Meeting the challenges of Dravet syndrome

Dravet syndrome and its management have an impact on almost every aspect of life, complicating simple daily activities and making spontaneous plans difficult.

Every child will have a different experience with Dravet syndrome. It’s important to learn as much as you can about the disease, and track your child’s experience so that you can confidently plan ahead for every possibility and help decide what’s best at each step.

Caring for a child with Dravet syndrome may be a challenge, but planning and staying one step ahead can help relieve some of the stress.

Here are some things you can do to be prepared:

  • Track all seizures and triggers. The free, comprehensive tools at seizuretracker.com can help make this job a lot easier
  • Learn seizure first aid and teach it to your family and friends. Information and digital classes are available at no cost through the Epilepsy Foundation® at epilepsy.com
  • Have a written family emergency response plan created in partnership with your doctor
  • Have a rescue therapy on hand. Your doctor may prescribe a fast-acting anti-seizure drug for emergencies
  • Stay on top of doctor appointments and medication management
  • Consider your child’s diet. Your doctor may suggest a ketogenic diet for your child. This is a high-fat, low-carbohydrate diet that requires close monitoring by a doctor. Some common foods included in a ketogenic diet are:
Your doctor may suggest a ketogenic diet

  • Seafood
  • Poultry

Your doctor may suggest a ketogenic diet

  • Low-carb vegetables like cauliflower, cabbage, broccoli, or zucchini
  • Avocados

Your doctor may suggest a ketogenic diet

  • Dairy products like cheese, plain Greek yogurt, or cottage cheese
  • Eggs

Your doctor may suggest a ketogenic diet

  • Nuts and seeds
  • Coconut or olive oil

It’s not easy for a child to stay on such a strict diet. Exploring fun and creative ways to present food to your child—like cutting food into fun shapes and using plates and utensils that will spark their imagination—may help. You may want to keep foods he or she shouldn’t eat out of sight and out of reach, and educate your family about the ketogenic diet so they can help your child stick to it. If the entire family follows a high-fat/protein, low-carbohydrate diet, it can help keep mealtimes manageable.

Be sure to consult with your doctors before incorporating a ketogenic diet or other lifestyle changes into your child’s treatment plan. You may require input from various members of their healthcare team in order to address your child’s specific challenges.

Helping your child navigate school

Children with Dravet syndrome can attend school, which can play an important role in their development, both cognitively and socially.

Your child will likely need an individualized education program (IEP) to support their schooling. The IEP sets academic and cognitive goals designed specifically for your child. This plan may involve a behavioral therapist, along with occupational, physical, and speech therapy.

School personnel should be familiar with your child’s diagnosis and know basic seizure first aid. They should also be familiar with seizure triggers and be aware of how to limit exposure. Your child should have a seizure action plan available at the school, along with rescue medication if prescribed by their doctor.

Making room for play

Discovering new ways to keep your child happy and engaged can often be a struggle. Try to find challenging activities for your child that allow them to have fun and provide therapeutic benefits, like outdoor activities. There are also camps specifically for kids with seizure disorders that provide a safe place for them to interact with peers who understand their condition.

Always check with your doctor before starting any new activity to make sure they are comfortable with your child’s participation, and never allow unsupervised activities.

Guidance and support

Building your support system

Managing a rare disease like Dravet syndrome can cause caregivers and families to feel lonely and isolated. Seeking support and community from friends, family, or other caregivers can help when times are difficult or when you need a reminder of your successes.

Resources to help you and your child steer through challenges

Take advantage of our expanding collection of resources. You can read about Dravet syndrome, discover new ways of helping your child navigate life, or find a support group to connect with other families like yours.

Get involved through these Dravet syndrome resources:

INDICATION and IMPORTANT SAFETY INFORMATION

INDICATION

DIACOMIT (stiripentol) capsules for oral use or powder for oral suspension are indicated for the treatment of seizures associated with Dravet syndrome in patients 2 years of age and older taking clobazam.

There are no clinical data to support the use of DIACOMIT as monotherapy in Dravet syndrome.

IMPORTANT SAFETY INFORMATION

CONTRAINDICATIONS

No contraindications are listed.

WARNINGS & PRECAUTIONS

Somnolence

DIACOMIT can cause somnolence. Monitor patients for somnolence, particularly when DIACOMIT is used concomitantly with other CNS depressants or clobazam, which is also known to cause somnolence.

Decreased Appetite and Decreased Weight

DIACOMIT can cause decreases in appetite and weight. The growth and weight of pediatric patients treated with DIACOMIT should be carefully monitored.

Neutropenia and Thrombocytopenia

DIACOMIT can cause significant declines in neutrophil and platelet counts. Hematologic testing should be obtained prior to starting treatment with DIACOMIT and then every 6 months.

Withdrawal Symptoms

As with most antiepileptic drugs (AEDs), DIACOMIT should be gradually withdrawn to minimize the risk of increased seizure frequency and status epilepticus.

Risks in Patients with Phenylketonuria (PKU)

DIACOMIT powder for suspension contains phenylalanine, which can be harmful to patients with PKU. Before prescribing DIACOMIT powder for suspension to a patient with PKU, consider the total daily intake of phenylalanine from all sources, including DIACOMIT powder for suspension. DIACOMIT capsules do not contain phenylalanine.

Suicidal Behavior and Ideation

AEDs, including DIACOMIT, increase the risk of suicidal thoughts or behavior. Patients treated with any AED for any indication should be monitored for the emergence or worsening of depression, suicidal thoughts or behavior, and/or any unusual changes in mood or behavior.

ADVERSE REACTIONS

The most common adverse reactions that occurred in at least 10% of DIACOMIT-treated patients and more frequently than on placebo were somnolence, decreased appetite, agitation, ataxia, decreased weight, hypotonia, nausea, tremor, dysarthria, and insomnia.

PREGNANCY

There are no adequate data on the developmental risks associated with the use of DIACOMIT in pregnant women. Based on animal data, DIACOMIT may cause fetal harm.

There is a pregnancy exposure registry that monitors pregnancy outcomes in women exposed to AEDs, such as DIACOMIT, during pregnancy. Physicians are advised to recommend that pregnant patients taking DIACOMIT enroll in the North American Antiepileptic Drug (NAAED) Pregnancy Registry (information at www.aedpregnancyregistry.org). This can be done by calling the toll free number 1-888-233-2334, and must be done by patients themselves or their caregiver.

To report suspected adverse reactions, contact BIOCODEX at 1-866-330-3050 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Please see full prescribing information for DIACOMIT®.

INDICATION and IMPORTANT SAFETY INFORMATION

INDICATION

DIACOMIT (stiripentol) capsules for oral use or powder for oral suspension are indicated for the treatment of seizures associated with Dravet syndrome in patients 2 years of age and older taking clobazam.