Friday, August 01, 2008

Huntingtons' Disease and seizures

A family has paid tribute to their "hero" who fought to the last after becoming the youngest person in the North East to be diagnosed with Huntington's Disease.

Steven Smith was just 13 when he was found to have the rare illness, which attacks the brain.The 25-year-old, from Abercorn Road, Farringdon, Sunderland, died last month, after a courageous 12-year struggle against the condition.Today his mum Jackie said Steven had suffered for such a long time, but fought bravely and never let it get him down.

She said: "He never complained. He was a hero. That's what we played at his funeral, Hero by Maria Carey, because he was my hero. He fought, he really fought."Steven was diagnosed with Huntington's Disease, which affects only a handful of people in every hundred thousand, after his parents noticed stark changes in his behaviour."

The condition, which usually affects adults, is caused by a faulty gene which damages nerve cells, causing gradual physical, mental and emotional changes.The teenager had become short-tempered and was getting into trouble at school, but parents Jackie and John Dixon could not understand why.It was by chance that Jackie, 46, finally found out what was wrong with her son.

While out shopping she saw Steven's biological father – who she had split up with before Steven was born – being carried out of a car in a wheelchair.She then learned the terrible truth. Both Steven's father and his grandfather had Huntington's Disease, a hereditary condition that can be handed down from parent to child.Steven's father was 27 when he developed the condition, and his father was even older. But Steven had a mutant strain of the illness and started displaying advanced symptoms when he was just 13.

Doctors originally told Jackie and John that it was not possible for Steven to have the disease, which has a 50/50 chance of being passed on, because he was so young. But tests confirmed he had Juvenile Huntington's Disease.

"I was devastated, but I knew we just had to try and keep him going as long as possible," she said."I had to explain to him how he came to have this horrible disease and try and do it in simple terms. I told him when a mam and a dad have a baby it gets so many toffees from each, and his dad had passed a broken toffee down to him. He never spoke about it again afterwards. He never spoke about how he felt. I suppose he knew he was going to die, but it was never mentioned."After his diagnosis, Steven left Farringdon School and transferred to Barbara Priestman School. He enjoyed going on trips and visits, and was given an award for his never-ending happiness."He was always smiling, right up until he died. He was always happy," said Jackie.Steven, who had one sister, Sarah, 20, a law and criminology student at the University of Teesside, was an avid film buff.

His parents said he loved holidays to Scotland, the Lake District and the Northumberland Coast, and said his favourite place to visit was Holy Island.Steven's condition continued to deteriorate and he spent the last four-and-a-half years in Victoria Lodge nursing home, where his seizures and hospital admissions became more and more frequent.Most people with Huntington's Disease eventually die from secondary illnesses like pneumonia. But Steven fought off these illnesses and seizures.

"Two weeks ago he had 22 seizures in six hours," said Jackie. "But Steven just kept bouncing back."Eventually Steven could no longer feed and simply did not have enough energy left to live.


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