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Ribble Valley woman's TV role in cystic fibrosis documentary
A CHARITY champion who has cystic fibrosis saw first-hand how scientists are fighting to find a cure for her illness for a new documentary.
Sophie Longton, 23, from Woodhead Road, Read, was chosen to take part in the BBC2 Horizon episode 'Miracle Cure? A Decade' of the Human Genome'.
Sophie was diagnosed with cystic fibrosis (CF), the UK's most common life-threatening inherited disease, at the age of eight.
It is caused by a defective gene that clogs the internal organs, specifically the lungs and digestive system, with a thick sticky mucus, leading to chronic infections and difficulty digesting food.
The documentary aims to explore the progress made by scientists working with human DNA to treat diseases.
Sophie, who won the Cystic Fibrosis Trust's Junior Fundraiser award in 2000, was one of three people followed by cameras behind the scenes at some of Britain's leading research laboratories.
The former Clitheroe Grammar student said: “I was really excited. I saw hands on what they were doing, met research assistants and looked at DNA under the microscope.”
Now an academic mentor at Mount Carmel High School, Accrington, Sophie does everything she can to keep her lungs in top condition.
This includes two hours of exercises, including physiotherapy and running to clear the mucus, and taking more than 30 tablets and five nebulisers, antibiotics pumped into her lungs, every day.
She said: “It's an awful lot. The reason I do that is if one day an effective treatment becomes available I will only be able to benefit if my lungs are in good condition.
“I fight so hard every day to keep myself as fit as possible.”
During filming Sophie met the scientist Simon Waddingon, from Nelson, who is researching foetal gene therapy.
It involves curing babies in the womb of serious diseases, but it is controversial because of ethical and safety concerns.
However, she believes it is worth pursuing, as currently there is no cure for CF. Three people die from the disease each week and the average life expectancy of a sufferer is 37.
She said: “Some people might argue they are trying to play God and change people's genetic make-up.
“But they are not changing the whole genetic make-up, just one aspect. They would replace a faulty copy of the CF gene with a healthy copy.
“One in 25 people in the population is a carrier, and if two carriers have a baby, there is a one in four chance of the baby having CF.
“If it had been an option for my parents to change that CF gene I'm sure they would have.”
The documentary airs at 9pm on Monday.
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