How Dravet Syndrome Is Treated

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Dravet syndrome is not curable, but treatments can improve symptoms and quality of life. The treatments used for Dravet syndrome are aimed at controlling seizures, as well as managing the other effects of the condition.

Medical management of Dravet syndrome can involve a team of healthcare professionals working with the family to help achieve the best possible outcome. 

Treatment of Dravet syndrome includes avoiding seizure triggers

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Home Remedies and Lifestyle

It is important to have a consistent lifestyle routine to prevent seizures and complications of this condition.

A number of triggers are known to provoke seizures in Dravet syndrome. It is vital to take prescribed anti-epileptic drugs (AEDs) as directed, but keeping triggers to a minimum is crucial. 

You can avoid factors that precipitate seizures by:

  • Controlling fevers: Fevers can provoke seizures in Dravet syndrome. Talk to your child’s doctor about what to do when a fever develops, such as an infection-associated fever. 
  • Regulating body temperature: Extreme temperatures can provoke a seizure in Dravet syndrome. Try to maintain regular temperatures with strategies you can plan—such as wearing layers in fluctuating weather, and hydration after physical exertion.
  • Avoiding flashing lights: Dravet syndrome is a type of photosensitive epilepsy. Rapidly flashing lights, such as from video games, entertainment, and emergency vehicles, can provoke a seizure. Avoid onscreen triggers and have your child close their eyes if flashing lights are encountered unexpectedly.
  • Getting enough sleep: Fatigue and sleep deprivation can provoke seizures for anyone who has epilepsy. Getting adequate rest can help prevent seizures.
  • Avoiding unexpected changes: Behavioral problems are often part of Dravet syndrome symptoms. Keeping daily activities stable with a predictable routine and explaining changes in advance can help prevent anxiety.

It is also important to maintain safety at home and when going places outside the home. Dravet syndrome causes physical and intellectual disabilities, which may increase the risk of accidental injuries. Planning to ensure that it is easy to get around can help prevent problems, such as falling. 

Over-the-Counter (OTC) Therapies 

Generally, OTC therapies can be used to manage mild symptoms of illness, especially a fever. 

Because your child will likely be taking one or more AEDs, and possibly other prescription medications as well, drug interactions can occur, even with OTC therapies. Be sure to check with your child’s doctor or with your pharmacist regarding which OTC medications would be best for your child to use. 

Prescriptions

Your child will likely be taking prescription AEDs to manage their seizures. They may also need other medications. Dravet syndrome can cause autonomic problems that affect the heart, breathing, or digestive system. Sometimes medications are needed to manage these issues. 

Anti-Epileptic Drugs (AEDs)

The seizures of Dravet syndrome can be especially difficult to control. The seizures tend to be more frequent and less responsive to medication during childhood. They can diminish in frequency and severity during the adult years, although seizures wouldn’t be expected to completely resolve. 

First-line AEDs cause few side effects. If seizures continue, second-line AEDs might be added or used instead.

First-line AEDs for treating epilepsy in Dravet syndrome: These include Depakote, Depakene (valproate), Frisium, and Onfi (clobazam).

Second- and third-line AEDs for treating epilepsy in Dravet syndrome: These include Diacomit (stiripentol), Topamax (topiramate), Klonopin (clonazepam), Keppra (levetiracetam), Zonegran (zonisamide), Zarontin (ethosuximide), Epidiolex (cannabidiol), and Fintepla (fenfluramine).

Rescue AEDs: With Dravet syndrome, it is not uncommon for a breakthrough seizure to occur, even when AEDs are taken as directed. Your child’s doctor might also prescribe an emergency AED that can be given at home if you can tell that a seizure is about to happen, and sometimes if a seizure has already started.

This will be a short-acting AED, possibly with a formulation for rectal administration since it isn’t possible to safely swallow medication during a seizure. Parents and other caregivers would be instructed on how to use a rescue seizure medication.

Certain AEDs can worsen seizures in Dravet syndrome. These include Tegretol (carbamazepine), Trileptal (oxcarbazepine), Lamictal (lamotrigine), Sabril (vigabatrin), Banzel (rufinamide), Dilantin (phenytoin), and Cerebyx and Prodilantin (fosphenytoin).

Systemic Effects 

Heart problems are associated with Dravet syndrome, especially during adolescence and adulthood. Some heart issues, such as arrhythmia (abnormal heart rhythm), may need to be treated with prescription medication.

Additionally, autonomic dysfunction, which affects systemic physical functions such as breathing and digestion, can cause symptoms that range in severity. Treatment would be tailored to the specific problem. For example, digestive issues might be treated with dietary modification combined with medication.

Surgeries and Specialist-Driven Procedures

Surgical interventions used in the treatment of Dravet syndrome may include epilepsy surgery or procedures that might be necessary for the treatment of complications.

Epilepsy surgery has been used as part of the treatment plan for seizures in Dravet syndrome. Implantation of a vagal nerve stimulator (VNS) or corpus callosotomy (cutting the corpus callosum that connects the left and right sides of the brain) have both resulted in good outcomes. However, epilepsy surgery is not a common choice in treating seizures of Dravet syndrome.

Other interventions may include orthopedic procedures, such as spine surgery to treat malformations that can result from the physical disabilities of Dravet syndrome.

Physical Therapy

Ongoing physical therapy can be beneficial in Dravet syndrome. When a child who has this condition gets older and physically grows, their physical capabilities will not advance like their same-age peers.

Therapy can help children, adolescents, and adults who have Dravet syndrome learn how to move their bodies more safely and with better control.

Cognitive and Behavioral Therapy

Techniques to help with learning and behavioral management can be part of the overall comprehensive treatment plan for Dravet syndrome. Children might attend school with a tailored learning plan, and speech therapy can be beneficial.

People who have Dravet syndrome might also meet with a counselor or therapist who can help with behavioral challenges. Identifying and recognizing emotions and learning how to manage feelings and interactions with others can be part of this type of therapy.  

Complementary and Alternative Medicine (CAM)

There are no CAM remedies that can cure Dravet syndrome or prevent it from worsening. However, the ketogenic diet is used as one of the strategies for controlling seizures.

Ketogenic Diet

Many people who have Dravet syndrome experience intractable epilepsy, in which the seizures are not well controlled with AEDs. The ketogenic diet is used as part of the treatment for some types of intractable seizures, including seizures caused by Dravet syndrome.

This diet is highly restrictive and alters the body’s metabolism through a specified combination of fat and protein intake, eliminating carbohydrates. It is difficult to follow a ketogenic diet, and it might be more practical for people who are completely dependent on caregivers for food supply.

 

A Word From Verywell

Dravet syndrome requires a comprehensive management plan. Seizure control is a primary focus of therapy, but other issues need to be managed as well.

Living with Dravet syndrome involves coordinating care with doctors, therapists, professional caregivers, and family members to ensure the best possible outcome. 

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