Tag Archives: Dravet syndrome

International Dravet Syndrome Awareness Month

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Date: June 23, 2022

Epilepsy is the 4th most common neurological disorder in the United States. With children, around 400,000 have epilepsy and most are able to control their seizures and lead normal lives. Dravet Syndrome is a rare form, of epilepsy found in children. Symptoms include, developmental delays, sleeping conditions, and chronic infections. Here are 20 facts you shoud know about Davet Syndrome.

20facts.dravet

click here for a printed copy

  • Charlotte Dravet first described severe myoclonic epilepsy of infancy in France, 1978.
  • The name was later changed in 1989.
  • It is also called Severe Myoclonic Epilepsy of Infancy (SMEI)
  • It is a rare genetic disorder affecting 1 in every 20,000-40,000 children
  • It appears during the first year of life.
  • Developmental disabilities and abnormal EEG’s are often not evident until the second of third year of life.
  • Seizures are often fever-related.
  • It is rare to develop beyond the age of 5.
  • Children often experience poor development of language and motor skills
  • Is caused by defects in a gene in 79% of cases.
  • Affects 2-5% of children in North America
  • It is induced by prolonged exposure to warm weather
  • It is associated with sleep disorder including insomnia
  • Seizures are frequently resistant to treatment
  • There is a higher risk of Sudden Unexplained Death in Epilepsy (SUDEP) (15-20%)
  • The onset of signs and symptoms is around 6 months old
  • Children diagnosed with Dravet Syndrome have a 85% chance of survival.
  • Development is typically normal.
  • Episodes may be frequent and prolonged

To learn more, Download a free factsheet here:

For Caregivers of Dravet Patients, Emotional and Financial Impact High, Study Reports

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Published by: Dravet Syndrome News
Written by: Iqra Mumal

Families caring for patients with Dravet syndrome experience significant emotional, social, and financial impact, according to a multinational study.

This study also found that directs costs from non-seizure-related healthcare use are four times higher than costs directly linked to seizures. High seizure burden was associated with higher healthcare costs as well.

The study, “Caregiver impact and health service use in high and low severity Dravet syndrome: A multinational cohort study,” was published in Seizure: European Journal of Epilepsy.

Symptoms of Dravet syndrome typically appear in the first year of a child’s life. Along with epilepsy, it is associated with a range of developmental and cognitive issues, behavioral disorders, and mobility problems.

While the impact of Dravet on families has not been widely studied, a recent survey has reported that caregivers of Dravet patients contend with a variety of concerns other than seizures, such as the disease’s impact on siblings and issues related to patients’ cognitive impairment.

In 2016, researchers conducted a large multinational online survey called the DS caregiver survey (DISCUSS), which was designed to further understand the clinical, economic, and humanistic burden of Dravet syndrome.

The survey included 584 caregivers of Dravet patients (83% pediatric; 17% adult), of whom more than 90% lived in Europe. The patients’ quality of life was very low, and Dravet patients who had a high seizure frequency had more coexisting conditions reported more emergency treatments, and had a lower quality of life than those who experienced fewer seizures.

To further investigate the financial factors associated with Dravet syndrome, researchers used data from the DISCUSS study to estimate the mean annual direct costs per patient for seizure-related and non-seizure-related healthcare and caregiver out-of-pocket costs in five European countries including France, Germany, Italy, Spain and the U.K.

The annual direct per patient cost for Dravet in these five countries was $15,885 ($9,783 when excluding anti-epileptic medications). This figure consists of $7,957 for treatment of seizure-related symptoms ($1,854 when excluding anti-epileptic therapies) and $7,929 for treatment of non-seizure-related symptoms.

Drivers of total direct costs were anti-epileptic drugs (38%) and non-seizure-related therapies (50%). When researchers excluded costs associated with anti-epileptic drugs, treatment for non-seizure-related symptoms contributed to 81% of direct healthcare costs. As a comparison, in a general pediatric population with epilepsy, treatments for non-seizure-related symptoms make up only 9.1% of total direct costs.

These treatments included physiotherapy, speech and behavioral therapy, and therapies for learning difficulties,  autism/autistic-like symptoms, and attention deficit disorder.

Among these, physiotherapy and speech therapy made up most (79%) of the non-seizure-related costs. Click here to read the rest of the story.

What You Need To Know About Dravet Syndrome

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Epilepsy is the 4th most common neurological disorder in the United States. With children, around 400,000 have epilepsy and most are able to control their seizures and lead normal lives. Dravet Syndrome is a rare form, of epilepsy found in children. Symptoms include, developmental delays, sleeping conditions, and chronic infections. Here are 20 facts you shoud know about Davet Syndrome.

20facts.dravet

click here for a printed copy

  • Charlotte Dravet first described severe myoclonic epilepsy of infancy in France, 1978.
  • The name was later changed in 1989.
  • It is also called Severe Myoclonic Epilepsy of Infancy (SMEI)
  • It is a rare genetic disorder affecting 1 in every 20,000-40,000 children
  • It appears during the first year of life.
  • Developmental disabilities and abnormal EEG’s are often not evident until the second of third year of life.
  • Seizures are often fever-related.
  • It is rare to develop beyond the age of 5.
  • Children often experience poor development of language and motor skills
  • Is caused by defects in a gene in 79% of cases.
  • Affects 2-5% of children in North America
  • It is induced by prolonged exposure to warm weather
  • It is associated with sleep disorder including insomnia
  • Seizures are frequently resistant to treatment
  • There is a higher risk of Sudden Unexplained Death in Epilepsy (SUDEP) (15-20%)
  • The onset of signs and symptoms is around 6 months old
  • Children diagnosed with Dravet Syndrome have a 85% chance of survival.
  • Development is typically normal.
  • Episodes may be frequent and prolonged
  • Certain seizure medications are likely to worsen the seizures such as Dilantin, Cerebyx and Tegretol.

Resources

Dravet Syndrome Foundation
P.O. Box 16536
West Haven, CT
Tel. 203-392-1950

Dravet Syndrome UK
P.O. Box 756
Chesterfield
5439EB
Tel. 07874 866937

Epilepsy Foundation- Provides online resources on information about epilepsy and seizures, locating assistance, advocacy and public awareness.

ICE Epilepsy Alliance– Provides resources on refractory patients (anti-epileptic drug therapy which is ineffective). Site includes information on advocacy, childhood epilepsy, and Dravet syndrome.

NINDS Dravet Syndrome Information Page– Medical information page on Dravet syndrome, diagnosis, treatment, research and organizations.

WebMD– Medical website providing information on diagnosis, signs and symptoms, and treatment.