Two young children in Washington have been diagnosed with a dementia-related condition. The children are only two and six years old, and parents Kristian and Bryden Tucker say the diagnosis has devastated them.
Six-year-old Michael and his two-year-old brother Oliver live in Spokane, Washington, and have a rare form of nervous system disorder known as Batten disease, which causes the brain to shut down gradually over five to ten years. When Michael was first diagnosed, the rest of the family decided to undergo testing, which revealed that Oliver had the same condition.
Kristian and Bryden Tucker say they first became concerned about Michael when he began suffering from seizures, which they say were so subtle, they didn’t immediately recognize them as seizures. The fitting episodes increased and became more pronounced when the child turned five, and his parents sought medical advice. Tests revealed that the couple were both carriers of the devastating condition, which they passed on to two children. Their daughter Talia is not a sufferer but is a carrier.
The National Institute of Neurological Disorders describes Batten disease as “a group of inherited nervous system disorders that most often begin in childhood.” Its formal name is neuronal ceroid lipofuscinosis and symptoms include movement disorder, loss of sight, seizures, and dementia. It is treated with cerliponase alfa, which slows the condition’s progress, but no cure exists.
Another potential treatment is a new drug, Milasen, developed at the Boston Children’s Hospital to treat a young girl named Mila Makovec. Mila was diagnosed with Batten disease in 2017 and Dr. Timothy Yu became aware of her case. Around the same time, the FDA approved a drug called Spinraza, which treated genetic neurodegenerative conditions.
Dr. Yu attempted to develop that drug specifically to meet Mila’s needs. Following trials and an initial downturn in the child’s health, Mila is now down from 30 seizures a day to “just a few,” and her condition has vastly improved.