What is Batten Disease?

Save the date for June 6 for the Batten Warrior 5K Run-Walk

Everyone I meet always asks the question, what is Batten disease? I really wish I didn’t know the answer! We have so much more to learn about this horrible disease. But here I go…There are 14 known and recorded types of Batten disease (CLN 1-14) and each variant share similarities and differences but the outcome is the same. Death.
Most forms of Batten disease/NCLs usually begin during childhood. Children with the disease often appear healthy and develop normally before they begin to show symptoms. Children with infantile or late-infantile forms usually show symptoms earlier than age 1. Common symptoms for most of the forms include vision loss, seizures, delay and eventual loss of skills previously acquired, dementia, and abnormal movements. As the disease progresses, children may develop one or more symptoms including personality and behavior changes, clumsiness, learning difficulties, poor concentration, confusion, anxiety, difficulty sleeping, involuntary movements, and slow movement. Batten disease is an inherited genetic disorder that appears to affect the function of tiny bodies within cells called lysosomes. Lysosomes are the “recycle bin” of the cell and regularly break down waste, proteins, and naturally occurring fatty compounds called lipids into smaller components that can be discarded out of the cell or recycled. Lipids include fatty acids, oils, waxes, and sterols. In Batten disease/NCLs, the mutated genes do not produce the proper amounts of proteins important for lysosomal function.
Basically, most children suffer from not being able to see, think and walk normally. They fall quite a bit often tripping on their own feet, eventually ending up in a gait trainer, a wheelchair, hospital bed then…We need to stop allowing this disease to rob kids of a full life! So we have created a charity “Project Sebastian” to build awareness and raise funds for a cure. Our next event is the Batten Warrior 5K Run-Walk Saturday, June 6th at West Creek Park in Valencia Ca, benefiting Project Sebastian’s mission to cure Batten disease. This will be a fun morning of vendors, DJ, Silent auction, raffle, and coffee!
You can sign up at www.projectsebastian.org or visit the events page on Facebook to register as well! If you have any questions or want to be a sponsor please reach out to Christopher Velona at info@projectsebastian.org

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