How Dravet Syndrome Affects Life Expectancy and Survival Rates
Key Takeaways
Dravet syndrome is a rare and severe form of epilepsy that carries a higher risk of early death compared to other forms of epilepsy, though many people with the condition now live longer than they did in the past.
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Dravet syndrome is a rare and severe form of epilepsy that can affect life expectancy. Many people with Dravet syndrome now live longer than they did in the past. Still, Dravet syndrome carries a higher risk of early death compared to other forms of epilepsy, such as Lennox-Gastaut syndrome.
Each person is different, and life expectancy can vary significantly. In this article, we’ll cover the statistics on life expectancy based on many cases of Dravet syndrome, risk factors that affect survival, and how people with Dravet syndrome are living longer than ever before with newer treatments.
Life Expectancy With Dravet Syndrome
Most children diagnosed with Dravet syndrome — as many as 80 percent to 85 percent — survive to reach adulthood. Unfortunately, between 15 percent and 20 percent of children diagnosed with Dravet syndrome die before they become adults. Among those who die at a young age, 73 percent of deaths happen before age 10, and 93 percent occur before age 20.
“As many as 80 percent to 85 percent of children diagnosed with Dravet syndrome survive to reach adulthood.”
With proper medical care and close monitoring, many people with Dravet syndrome live well into middle and late adulthood. For example, in the U.K., the oldest person with Dravet syndrome lived into his 70s.
Many factors can affect the life expectancy of an individual with Dravet syndrome. That’s why it’s so important to have safety plans and regular follow-ups with a neurologist experienced in treating Dravet syndrome.
Why Is There a Higher Risk of Death in Dravet Syndrome?
Compared to the general population and people with other forms of epilepsy, people with Dravet syndrome face a higher risk of early death. This is mainly because of the frequency and duration of seizures in Dravet syndrome, as well as complications and health issues discussed below.
Sudden Unexpected Death in Epilepsy
Sudden unexpected death in epilepsy (SUDEP) happens when a person with epilepsy dies suddenly without warning, and no other cause of death is found. SUDEP often occurs during sleep, and it’s more frequent in people who have had many tonic-clonic seizures — a type of generalized seizure with muscle stiffness followed by jerking movements.
“Complications such as sudden death in epilepsy, status epilepticus, and accidents including falls or drowning are often to blame for deaths in Dravet syndrome.”
For people with uncontrolled seizures, SUDEP is the most common cause of death. In Dravet syndrome, nearly half of all deaths are caused by SUDEP. Doctors don’t know exactly what causes SUDEP. However, changes in heart rate, breathing, and the autonomic nervous system (which controls basic, vital body functions) may play a role.
Gaining control over seizures with medication can lower the risk of SUDEP and other causes of death linked to Dravet syndrome. Families can also lower the risk by using nighttime seizure monitors, following medication plans closely, and working with a neurologist to manage care. SUDEP is rare, but because it can happen without warning, it’s important to discuss it early and often with your healthcare team.
Status Epilepticus
Status epilepticus is a seizure that lasts too long — usually more than five minutes. It can also describe a series of seizures where the person doesn’t become fully conscious in between. Status epilepticus is a medical emergency. If not treated quickly, it can lead to brain damage or death. In Dravet syndrome, complications from status epilepticus are the second leading cause of death.
Unfortunately, status epilepticus is common in Dravet syndrome, especially in babies and young children. It can be triggered by fever, illness, or missed doses of anti-seizure medications. Since Dravet syndrome often begins in a child’s first year of life, many children experience prolonged seizures early on.
Having a rescue medication plan at home can help stop seizures before they become life-threatening. A class of anti-seizure medications known as benzodiazepines can be used to treat seizures lasting longer than five minutes. Other medicines may be helpful to have on hand for emergencies, too. Parents and caregivers should seek training on how and when to give emergency treatment. Talk to your healthcare team or a pediatric neurologist to make sure you have a plan in place.
Accidental Injury and Drowning
Seizures in Dravet syndrome can happen at any time and often come without warning. This makes accidents more likely to happen, especially among active young children and teenagers. Some types of seizures, like myoclonic (quick jerks) or atonic (sudden loss of muscle strength) seizures, can cause a person to take a hard fall or lose control of their body.
Injuries from falls, burns, choking, or head trauma are the most common. Drowning is also a major concern. Seizures that happen in water — including bathtubs, pools, and lakes — can lead to death in a matter of seconds. People with Dravet syndrome must never be left alone near water, even for a short time.
“As children with Dravet syndrome become adolescents and adults, the pattern of risk begins to shift. Seizures may become less frequent for some.”
Simple safety precautions — like using helmets, padding furniture, and setting up seizure alarms — may minimize risk. With the right safety measures, you can significantly reduce the risk of accidents at home or in your community.
Age-Specific Risks in Children and Adults With Dravet Syndrome
In infants and young children, the biggest threats often come from long or repeated seizures. These are more likely in the early years, especially during illness or high fevers. The risk of status epilepticus is also highest in this age group. Some children may stop breathing during seizures, making these events even more dangerous. This is part of the reason why so many deaths in Dravet syndrome happen before age 10.
As children become adolescents and adults, the pattern of risk begins to shift. Seizures may become less frequent for some, while others continue to have daily episodes. Long-term effects like cognitive impairment (thinking problems) or ataxia (balance problems) can add to the risk of injury. Feeding issues may also lead to other serious health issues, such as weight loss, pneumonia, or choking.
What Causes Higher Risks in Dravet Syndrome?
Dravet syndrome is most often caused by a mutation (change) in the SCN1A gene, which plays a big role in how neurons (brain cells) send signals to each other. This gene affects sodium channels — a major player in controlling levels of excitation in the brain. When SCN1A doesn’t work correctly, the brain becomes too excitable. The result is frequent seizures, including some types of seizures that are hard to control or that last a long time.
Sodium channels affected by SCN1A mutations also play a role in other parts of the nervous system. This includes the autonomic nervous system, which controls basic bodily functions like heart rate, breathing, and temperature. When this system isn’t working properly, it can raise the risk of serious complications — most commonly, SUDEP, breathing issues, or overheating during fevers.
Neurological impairment caused by repeated seizures can also lead to developmental delays. Many people with Dravet syndrome also have comorbidities (other health conditions), such as sleep problems, weak muscles, or poor bone strength.
Improvements in Life Expectancy
The good news is that there are advances in science that may help people with Dravet syndrome live longer than before. New seizure medications may help people with Dravet syndrome have fewer seizures. Having fewer seizures can also lower the risk of death.
Other treatments for Dravet syndrome, like the ketogenic diet and vagus nerve stimulation, may also help. Newer therapies from clinical trials may offer even more options in the future. As awareness grows and care improves, more people with Dravet syndrome are reaching adulthood and experiencing better long-term quality of life.
Talk With Others Who Understand
On MyEpilepsyTeam, people share their experiences with epilepsy, get advice, and find support from others who understand.
What measures do you take to protect yourself or your child with Dravet syndrome? Where do you find support as you face questions about life expectancy? Let others know in the comments below.
Investigating Risk Factors for SUDEP in Dravet Syndrome — American Epilepsy Foundation
Dravet Syndrome (DS): Life Expectancy — Rare Disease Advisor
Sudden Unexpected Death in Epilepsy (SUDEP) — Epilepsy Society
A Practical Guide to the Treatment of Dravet Syndrome With Anti-Seizure Medication — CNS Drugs
Efficacy of the Ketogenic Diet in Patients With Dravet Syndrome: A Meta-Analysis — Seizure