March 20, 2019
A mother from Warwickshire shares her son’s battle with a rare disease and how they are making the most of the time they have left with their five-year-old son.
As one of only two children in the UK, George Young suffers from Batten Disease. One of its symptoms is dementia and has already caused various impairments.
"I wasn’t aware of childhood dementia, I didn’t know it could happen to little ones at all, and you especially don’t expect it to happen to one of your own,” Claire, George’s mother told the Independent.
When George was a baby, there was no sign of impending disease as he developed normally and “hit all his major milestones.” Since being diagnosed with the condition in February 2017, George lost the ability to speak and his mobility.
The fatal genetic neurological disorder affects the nervous system and causes cognitive impairment, loss of sight, seizures and the onset of dementia.
Batten Disease has 14 variants, with George’s CLN8 strain being one of the rare ones. Only two boys in the UK has that strain of the condition.
First diagnosed when he was three years old, George is now losing his sight in addition to his speech and mobility while also being fed through a tube.
35-year-old Claire Young wants to have as many experiences with her son before the disease progresses any further as she can. She set up a “bucket and spade list” of activities they would have done together, and the two have made good work of it so far.
Young George met soldiers at Buckingham Palace, driven a sports car, went on a helicopter ride, and had fun riding the roller-coaster at Peppa Pig world.
Batten Disease affects up to four out of every 100,000 births in the United States with the CLN8 variant referring to a gene on the eighth chromosome, passed on from two unaffected parents.
Similarly, Paul and Carly Hadman’s daughter, Effie also suffers from Batten Disease after being diagnosed with the disease when she was five years old.
Much like George, Effie lost her ability to speak, walk, feed herself and remember basic things.
“Piece by piece, we lose a little bit more of Effie every single day. Seeing her with this disease is like one long, painful goodbye,” Carly said. “She’s got a healthy brother who has now overtaken her in the way he’s developed.”
Effie’s parents, also living in the UK are aware that she won't make it to her teens and make the most of the time they have left with her as Carly added:
“We know we are going to lose her, but we’ve got the rest of our lives to grieve. For now, we’re just trying to make her smile. Effie is five now.”
Sadly, Effie passed away when she was six years old in February 2017, roughly a year after the interview with her mum Carly.
Last year the younger brother of sixteen-year-old Christiane Benson from Texas, who also suffers from Batten Disease, took on a monumental task in trying to raise funds to help his sister.
December 29, 2018