HARLEY’S STORY

Harley was just five months old when he had his first seizure.

Harley’s first seizure was initially thought to be febrile, but he had another two weeks later and another 2 weeks after that. Each time the seizures went on for longer and longer. Finally, many months later after lots of tests and drugs that aggravated the condition, he was diagnosed with Dravet Syndrome or SMEI- Severe Myoclonic Epilepsy of Infancy – a rare form of epilepsy affecting children.

Harley’s condition means he has to be watched constantly twenty-four hours a day.  He’s at constant risk of seizures which can be triggered by temperature, a virus, over tiredness, overexcitement,  or can occur out of the blue for no apparent reason.   The seizures are frequent and without pattern -no week or month is ever the same. They persist despite treatment.   Every day, week and month brings new challenges for him and his family.  Harley is developmentally delayed and has limited speech.  He suffers with prolapsed ankles and requires supportive shoes and insoles.  He has to wear a protective hat when playing outside due to his danger of falling through seizure or from his unsteady gait.  

Now Harley’s parents Adam and Dawn have established the Chester Challenge cycling event in a bid to help raise awareness and benefit children in the UK suffering with Dravet Syndrome. 

WHAT IS DRAVET SYNDROME? 

Dravet Syndrome is an extremely rare condition affecting children who experience many different types of seizures. Children with Dravet can have hundreds of seizures a day and don’t respond well to treatment. 

Dravet Syndrome is one of the most catastrophic paediatric epilepsy syndromes. 

It is very rare, representing about 7% of all severe epilepsies starting before the age of 3 yrs.  It is highly resistant to treatment and its prognosis is poor.  Children are often left cognitively and developmentally impaired – often severely.

 Only 1-5 % of cases have been diagnosed but there are at least 306,000 individuals in the world that have Dravet syndrome. 

Symptoms  generally begin in the first year of life around five months of age with boys twice as affected as girls. 

When it starts sufferers usually have one-sided or grand mal (tonic-clonic) seizures, which are difficult to stop and last longer than 10 minutes. 

Child sufferers are at risk of serious seizures (status), due to illness, tiredness or over-excitement, which usually requires hospitalisation. 

There is currently no cure.  Response to medication is variable and often seizures persist despite treatment . 

For more information go to http://www.idea-league.org.uk/