Ten-year-old Sheldon Steuart has Batten disease — a group of rare, neurodegenerative disorders that are often fatal. The symptoms of the disease came upon Sheldon suddenly. In an interview with CBC Radio, Sheldon’s mother, Eve Courchene said, “He went from one weekend going sledding, going crazy, running around to the next weekend every time he’d walk he’d fall. He couldn’t climb up the stairs properly. He was unbalanced.”
The sudden change in Sheldon’s physical ability has left him feeling somewhat alone. Fortunately, it is the season for Christmas cards and a social media campaign began to have people send cards to Sheldon to lift her spirits. The response has been overwhelming.
Batten disease advocate, Jody Zarn of Laps 4 Life, said, It’s been a really good response. I’ve been getting messages from people all over the country and in the U.S. and even teachers wanting their students to be involved.”
Cards can be sent to:
PO Box 50029
Rivergrove Post Office
Batten disease is a rare neurological disease that usually strikes in early childhood. Symptoms may begin subtly, with mild behavior changes, clumsiness, or visual problems. Over time, worsening seizures and blindness occur, with progressive loss of motor skills, and ultimately dementia and death.
In Batten disease, there is an abnormal buildup of lipofuscins in the body that can lead to neural damage.
The most common form of Batten disease is juvenile Batten disease (also referred to as infantile CLN1 disease), in which symptoms begin in early childhood. This is traditionally what clinicians meant by the term ‘Batten disease’. However, more recently, the term Batten disease is used to describe other related conditions that involve lipofuscin buildup.
So far, 8 different genes have been implicated in the various forms of Batten disease, with many others yet to be identified.