He is just eight years old and should be enjoying football and computer games.
But Ben Scott suffers from a rare form of dementia – which is slowly robbing him of his mental faculties and taking his life away.
He can still recognise his mum and dad and give them a smile, but in recent months even that has begun to fade and his condition has worsened so much he can no longer walk, talk or feed himself.
Now his family in Yeovil, Somerset has returned from a research trip to America to try to gain a better understanding of the rare disease which has devastated all their lives.
For the past year since the diagnosis that Ben will “die before he is 20”, the lives of his parents, Andrew and Lyndsay, have been consumed by Nieman-Pick – a rare form of dementia that affects just 500 children worldwide and only 17 in the UK.
It occurs as a result of the build-up of cholesterol in all the body’s organs, including the brain, effectively stopping them from performing their jobs, causing a progressive loss of mental faculties as well as problems with movement. There is no cure and the pace of deterioration is unpredictable.
Click here for more
But Mr and Mrs Scott said they returned from Washington at the weekend after the week-long trip to the National Institute of Health with “useful information” for the future.
As Ben, who has a mental age of one, watched his favourite cartoon in the living room of their home in Lowther Road, Mrs Scott, 31, said: “I don’t know which is harder, that Ben is going to die or that it will get to the stage where he won’t know who I am. I dread the day he won’t smile at us.”
The family thought that day had arrived in December when suddenly the smile and the laughter disappeared. But it returned about a month later.
Mr Scott, 34, said: “When you think you have lost that smile it is heartbreaking and when it does go that will probably be the hardest thing to cope with.
“It turns out he was malnourished and, after a gastroscopy, it was like a ray of light when he began smiling and laughing again.”
But Ben’s deterioration has been quicker than is the norm in most cases.
Mr Scott said: “We were beginning to think: when is it going to plateau? When is it going to stop? What else is he going to lose? We could not even lay him on his back as he had no weight on him.”
The family were offered the opportunity by a Niemann-Pick charity to take part in the American study, which is funded by the US federal government. It has been on-going for three years.
They carried out a variety of scans and tests on Ben and looked at every aspect of him.
Mr Scott said: “It has given us handy information for the future. The tests showed that when Ben was swallowing food it was going straight into his lungs.
“They are really on the ball with everything. In England we have been trying to get splints for him for eight months. In America the physio sorted it out for us in a day.
“Things do take a long time over here. We had about a year-long battle to get him a wheelchair and in the end a charity came forward to pay for one.
“There is still a lot they don’t know about the condition and they need to understand it before they can think about looking for a cure.
“We are under no illusions. We know what is going to happen but it has given us a better understanding at what point he is and what to expect in the future.
“Our priority is to spend time with Ben and make as many memories as we can while he is as well as he is. We don’t expect huge things for the future.”
Copyright 2009 This Is Somerset