The effects of Dravet syndrome can be severe and affect the quality and length of one’s life. However, most people with this condition live to adulthood and may not need to live in a nursing home.
Dravet syndrome is a brain disorder. It causes epilepsy and physical and cognitive impairment due to the effects seizures have on brain development. Typically, it begins to appear within the first year of life before a child reaches 12 months of age. The first seizure is usually prolonged and sometimes occurs during a fever. The type of seizures can change with time.
Dravet syndrome often leads to physical and mental developmental delays, such as difficulty walking, speech delays, slower growth than their peers, and nutritional issues. The disease can disrupt the autonomic nervous system, making temperature regulation difficult.
Having Dravet syndrome can affect a person’s life expectancy, but it doesn’t always. Keep reading to learn more about this complex condition.
Of all the epileptic conditions, Dravet syndrome is believed to have a higher mortality rate. Current statistics suggest that roughly
However, this also means that up to roughly 80% of people with Dravet do reach adulthood. Usually, people with Dravet syndrome will need some caregiver support throughout their lives, but the amount of support needed will vary widely, as there’s a range of symptoms that can be experienced.
Some people have mild Dravet syndrome, meanwhile for others, the condition causes impaired motor and brain function to the point that a continued high level of care is needed to manage basic tasks.
Dravet syndrome in infancy
Between 80% and 90% of people with Dravet syndrome have the SCN1A gene mutation. However, only 4% to 10% of those mutations are documented as inherited from one or both parents. And in most cases, signs and symptoms will present early, within the first 12 months of life.
Some common signs that suggest a baby might have Dravet syndrome include:
- seizures triggered by a warm water bath
- seizures that occur on just one side of the body
- seizures that are triggered by illness, hot weather, or exposure to bright or flashing lights
- seizures that last longer than 10 minutes
Dravet syndrome in toddlers and preschool-age children
However, some children won’t exhibit symptoms until toddlerhood. Usually, for these children, the seizures are unprovoked or don’t have obvious triggers. But, they’ll occur often and be prolonged, typically longer than 5 minutes.
But as the seizures continue, other developmental side effects are possible and may begin to present between the ages of 2 and 5 years. These can include:
- learning difficulties
- behavioral problems
- developmental delays
- poor coordination
- low muscular tone
- attention deficit hyperactivity disorder (ADHD)
- problems with movement and balance
- autonomic nervous system issues (trouble regulating body temperature, blood pressure, and heart rate)
- sleep problems
- poor gait (walking)
Who’s the oldest living person with Dravet syndrome?
According to a 2011 study that reviewed patient data of 22 people with Dravet syndrome, the oldest living person was 60 years old. However, the median age of the participants was 39.
While multiple seizures continue to be a hallmark symptom, adults with Dravet syndrome also showed neurological decline, specifically for motor and cognitive deterioration that requires more caregiving support. And dysphagia (difficulty swallowing) was more likely to occur, which can increase the risk of complications.
Having a life-limiting illness can be overwhelming on an emotional level as well as a physical one. This means that anxiety, as well as depression, are possible conditions that can develop.
Depending on the severity of their developmental delays, people with Dravet syndrome may experience:
- a reduced desire to socialize
- an unwillingness to talk about their feelings
- irritability, especially as they lose the ability to engage in activities they once enjoyed
It’s incredibly important to make sure that mental healthcare is a priority for people with Dravet syndrome, as well as for those that love them.
While this care may look different than typical talk therapy, early access to therapeutic strategies can shape the way children cope with their emotions throughout life. Some techniques, like sand tray therapy, use the way children play to help them express themselves.
Mental health services for people with epilepsy and Dravet syndrome
Depending on the severity of the condition, epilepsy and Dravet syndrome can be diseases that can significantly reduce a person’s quality of life.
Seeking mental health support is critical to ensure a person with Dravat syndrome and their loved ones have the tools they need to manage the condition beyond medical treatments.
Know that you’re not alone, and there are lots of resources out there to support you:
- The Dravet Syndrome Foundation provides resources for adults living with Dravet syndrome as well as caregivers of children who have received the diagnosis. They also have a Facebook group.
- Dravet Urgency creates infographics and other resources for people with Dravet syndrome and their loved ones.
- The Epilepsy Foundation also offers a range of support groups — both virtual and in-person — that are often categorized by age to help people and caregivers avoid feeling isolated during this time.
- The Child Neurology Foundation provides a peer contact line for those in need of support.
- Finding a family therapist can also help you work through the intense emotions that come with either having a child with a life-limiting illness or having one yourself.
How to get involved in clinical trials
Research into new therapies and treatments to better manage Dravet syndrome seizures and symptoms is ongoing.
Parents or people with the condition who are interested in applying to participate in a trial should visit ClinicalTrials.gov, a resource compiled by the National Library of Medicine (NLM), to look for trials that are actively recruiting participants. They include both public and privately funded studies.
But keep in mind that the studies haven’t been evaluated by the U.S. federal government. So, always talk with your physician before participating.
Additionally, the Dravet Syndrome Foundation has also compiled a list of clinical trials addressing the disease.
Unlike with cancer or other conditions where life expectancy can be easily quantified, a Dravet syndrome diagnosis doesn’t always offer that insight. So, rather than end-of-life care, caregivers and loved ones may need to consider palliative care services.
While end-of-life discussions can be a part of palliative care, the purpose is to provide a comprehensive network of medical care and emotional support for people with Dravet syndrome while improving their general quality of life.
Providers can include religious leaders, nurses, therapists, social workers, and other medical professionals. By shouldering many of the caregiving responsibilities, this option allows caregivers to also have an improved outlook.
Additionally, palliative care can also ensure that someone with Dravet syndrome can stay in their home and be with loved ones rather than being relocated to a permanent healthcare facility.
- Physical therapy: This may be needed to help prevent atrophy and contractures due to a lack of activity.
- Occupational therapy: This may also be helpful with some aspects of self-care, like using the toilet, brushing teeth, and eating.
- Speech therapy: Speech therapy or special education can help some children learn to communicate better.
Dravet syndrome is a brain disorder that affects about 20% of children, who start showing symptoms like prolonged seizures before the age of 1 year. This is a difficult diagnosis to face and manage. However, 80% of people with the disease reach adulthood. Also, some medications can work to control seizures, improving your child’s quality of life.
Developmental and behavioral delays are possible, and the majority of people with Dravet syndrome will need some form of caregiving support throughout their lives.
There are treatment options to help manage the condition as well as resources to help a person with Dravet syndrome and their loved ones cope with the diagnosis.