* spielmeyer Fogt Hergan
*
* Batten Disease is a degenerative neurological condition that affect people of all ages, particularly between mid childhood and their early twenties.
* The most common form of Battens is Juvenile Battens affecting children between 5-25 years.
* It was discovered in 1903 Frederick Batten, a British Paediatrician. [22]
* Detected when symptoms such as loss of vision and balance, seizures, minor behavioural changes occur. These are often diagnosed as epilepsy or other conditions.
* As the disease progresses, the individual will often go completely blind, loss all motor skills e.g. walking, swallowing severe mental disabilities. Many individual will end up on feeding tubes or life support until the disease become …show more content…
[4,7,17]
* Discovered that built-up material is subnit C, usually found in mitochondria. Using large animal models e.g. dogs and sheep to see why it accumulates and what kinds of treatment may prevent it. [20]
* Funding has allowed a number of clinical trials to take place in the fight to find a treatment or cure for Battens. This includes $6 million from Beyond Battens Foundation to enhance number of trials. Stem cell treatments in which stem cells lines are taken from patients and their families are currently being used in the effort to find a cure. [7,9,11,13]
Families with an affected individual are often faced with all the decision regarding treatment. As the disease worsens some chose to withdraw or withhold treatment or request that their child is euthanised. Before death, individuals are completely bed bound and unable to communicate. This poses complication as the family is forced to make a decision regarding life sustaining treatment. Most will either keep their child on life support until death, or take them off and allow the disease to progress without any medical interference. Some families apply to have their child euthanised at the height of the illness, however it is illegal in nearly all countries around the world and will often result fronting the court. For most individual care is predominantly palliative in the exception of clinical trials and individual medications for symptoms. [10
*
* Batten Disease is a degenerative neurological condition that affect people of all ages, particularly between mid childhood and their early twenties.
* The most common form of Battens is Juvenile Battens affecting children between 5-25 years.
* It was discovered in 1903 Frederick Batten, a British Paediatrician. [22]
* Detected when symptoms such as loss of vision and balance, seizures, minor behavioural changes occur. These are often diagnosed as epilepsy or other conditions.
* As the disease progresses, the individual will often go completely blind, loss all motor skills e.g. walking, swallowing severe mental disabilities. Many individual will end up on feeding tubes or life support until the disease become …show more content…
[4,7,17]
* Discovered that built-up material is subnit C, usually found in mitochondria. Using large animal models e.g. dogs and sheep to see why it accumulates and what kinds of treatment may prevent it. [20]
* Funding has allowed a number of clinical trials to take place in the fight to find a treatment or cure for Battens. This includes $6 million from Beyond Battens Foundation to enhance number of trials. Stem cell treatments in which stem cells lines are taken from patients and their families are currently being used in the effort to find a cure. [7,9,11,13]
Families with an affected individual are often faced with all the decision regarding treatment. As the disease worsens some chose to withdraw or withhold treatment or request that their child is euthanised. Before death, individuals are completely bed bound and unable to communicate. This poses complication as the family is forced to make a decision regarding life sustaining treatment. Most will either keep their child on life support until death, or take them off and allow the disease to progress without any medical interference. Some families apply to have their child euthanised at the height of the illness, however it is illegal in nearly all countries around the world and will often result fronting the court. For most individual care is predominantly palliative in the exception of clinical trials and individual medications for symptoms. [10