How does tay sachs kill?
Tay-Sachs disease is a fatal genetic disorder that destroys nerve cells in the brain and spinal cord. It is caused by a deficiency of the enzyme hexosaminidase A, which is responsible for breaking down a fatty substance called GM2 ganglioside. When GM2 ganglioside accumulates in the brain, it damages nerve cells and leads to a progressive decline in mental and physical abilities.
Tay-Sachs disease typically begins in infancy. Babies with the disease may appear normal at birth, but they soon begin to develop symptoms such as:
* Loss of muscle tone
* Difficulty swallowing
* Seizures
* Blindness
* Deafness
* Mental retardation
As the disease progresses, children with Tay-Sachs disease become increasingly disabled. They may lose the ability to walk, talk, and even breathe. Most children with the disease die before the age of 5.
There is no cure for Tay-Sachs disease. Treatment is focused on relieving symptoms and providing supportive care. Some children with the disease may benefit from enzyme replacement therapy, which involves replacing the missing enzyme.
Tay-Sachs disease is a devastating disorder, but it can be prevented. There is a genetic test available to identify carriers of the Tay-Sachs gene. Couples who are both carriers have a 1 in 4 chance of having a child with the disease.