According to a story from 13abc.com, a family is working to spread awareness about Batten disease after second grader, Charles Klein was diagnosed with the condition. The disease has no cure and is ultimately fatal. His diagnosis comes to a family of four boys that has already been struck by tragedy. The boy’s older brother Max died six years ago at only three years old.
Batten disease is a condition that affects the nervous system and appears in childhood, normally between the ages of five to ten years. In the case of Charles, symptoms first appeared when he began having trouble with his eyesight. Seizures are also common, and other symptoms include alterations to behavior or personality, problems with learning, regression, stumbling and clumsiness, and repetitive speech. Over time, these symptoms progressively worsen, ultimately causing death. Life expectancy depends on the severity and variation of the disease. There are very limited treatment options available for Batten disease. Brineura, a treatment for a specific type of Batten Disease known as CLN2, is able to slow the loss of walking ability. However, Charles is not a candidate for the treatment, as he has CLN3. Most care is palliative, focusing on maintaining quality of life and reducing the effects of symptoms. To learn more about Batten disease, click here.
Since Batten disease is caused by genetic mutation, there is a slim chance that the other siblings could be at risk for the disease as well. Mike, Charles’ father, says that there is a 25% chance they could be affected.
