Batten Disease is a rare, fatal genetic disease affecting 2 – 4 out of every 100,000 infants. It is the most common form of a group of neurological disorders known as Neuronal Ceroid Lipofuscinosis. Symptoms may start as seizures and vision problems or be as subtle as personality changes. The age of onset varies and can be as young as six months; however, most children start between ages 4 yr and 10 yr. Symptoms progress steadily to include inability to walk or even hold their head up and mental impairment. Eventually, children become bedridden and blind. They also lose the ability to communicate. The condition is always fatal – usually before their teen-age years.
Currently, there is no cure or any way to prevent the disease. Parents can have genetic testing, but unfortunately this is in hindsight after a child has already been diagnosed with the condition. Treatment is merely supportive. Anti-convulsant medications are used to help lessen the frequency of seizures. Physical and Occupational therapies are both used to help children remain as functional as possible.
There is great promise in stem cell research for these children. Unfortunately, there are only a few facilities in America that are doing any research or treatment studies. These facilities are limited on the number of patients that they treat. This is very disheartening for parents.
China holds a world-class neurological treatment facility. They are leaders in stem cell research and treatment. This facility is seeing amazing improvements in people with neurological diseases. Children with Batten Disease that have been treated with stem cell injections are once again holding their heads up, smiling, laughing, and attempting to communicate with their families. This gives parents great hope that their children may live long enough to see a cure discovered. This can be a parent’s one chance for their child’s survival.
We currently have a family in our community that has been struck hard by this devastating disease. The Goranflo’s have two beautiful children that have both recently been diagnosed with Batten Disease and are now in the fight of their lives. Here is the website where their story can be read: www.haileyandcartersdream.com.
Below is a listing of a few websites where more information on Batten Disease can be found:
www.Bdrsa.org / www.ninds.nih.gov
Article By: Stacey Koch,
RN Senior Public Health Nurse