A rare form of epilepsy means three-year-old Harley Paterson has as many as 20 seizures a week – and his family literally can’t let him out of their sight. GIL LIGHTFOOT meets them
RUNNING around the garden on a sunny afternoon, three-year-old Harley Rigler loves getting up to mischief.
“He will run around, climb and jump. He has no fear. He is a typical little boy,” says his mum, Dawn Paterson.
But that’s where the similarity ends because unlike most toddlers, Harley has a rare form of epilepsy called Dravet Syndrome, which is recognised as one of the most catastrophic neurological disorders.
His condition, which puts him at constant risk of seizures – sometimes up to 80 a month – means that he has to be watched virtually 24 hours a day. His mum and dad, Adam, sleep with a TV monitor at their bedside which is linked to a camera in Harley’s room. When he plays outside, he has to wear a special helmet to prevent injury if he falls during a seizure.
“He’s at risk of seizures which can be triggered by temperature, a virus, over-tiredness, over-excitement, or simply occur for no apparent reason. The seizures are frequent and without pattern,” says Dawn at her Oscroft home.
“They persist despite treatment and Harley is now also on a specially modified diet which restricts his intake of carbohydrates, in a bid to limit their frequency.”
A constant fear is Harley will suffer status seizures – prolonged seizures which can cause brain damage.
“He is also more at risk from SUDEP (Sudden Unexplained Death in Epilepsy Patients) accidents through falls from seizures, regular sleep disturbances, lowered immunity and mild ataxia issues – balance, stumbling and foot deformities,” says Dawn.
“This is the reality we live with. We can only take each day as it comes.”
Harley had his first seizure at five months old when the family was holidaying in France. “It was so unexpected,” said Dawn. “He was red hot and foaming at the mouth. We went into panic mode and drove him straight to a nearby clinic.
“They cooled him down and when they checked him over everything seemed normal so they put it down to a febrile convulsion, which is common in young babies.”
However, on their return home, Harley’s seizures became increasingly frequent and at 18 months old he was diagnosed with Dravet Syndrome.
Since then, Dawn and Adam, who also have a five-year-old daughter, Hannah, have faced the relentless task of caring for Harley, trying out the various drugs available, monitoring his progress. In his three short years, Harley has suffered more than 450 seizures.
“He suffers from a mutation of his chromosome chain and the seizures are actually a symptom of his condition,” explains Dawn.
Trying a different drug recently brought Harley a period of several weeks with few seizures.
“If he has a good patch you let your guard down a little. You think you are going to have some sort of normality back. Now we are back to about 20 seizures a week. It’s absolutely exhausting,” said Dawn.
A local nursery provides one-to-one care for Harley for two hours, twice a week. He enjoys being with the other children but has difficulty communicating.
“Harley is very sociable and very happy,” says Dawn, “but the seizures mean he has delayed speech so instead of speaking in sentences he uses around 20 or 30 single words.”
Dawn and Adam are fundraising for the IDEA League, a non-profit charitable organisation which unites parents and professionals to promote research and education for the early diagnosis, treatment and cure of the condition.
They are organising a sponsored bike challenge on August 3, setting off and returning to the Old Hall Country Club in Huntington. There is a choice of three routes, varying in distance from 12.5 to 50 miles. All proceeds will go to the IDEA League.
For further details, visit www.chesterchallenge.co.uk or contact Dawn on 07967 405330.