Tay Sachs disease is a genetic disorder which primarily occurs in children and can be fatal and destructive. The disease spreads slowly through the child, destroying the central nervous system in the process and causing countless disabilities. Also known as TSD, the disease is named after the famous British ophthalmologist Warren Tay and the New York based neurologist Bernard Sachs.

The disorder came to prominence when Warren Tay discovered a red cherry-like spot in the retina of a patient's eye. Sachs contributed enormously towards explaining the cellular changes caused by the disease in the human body. Sachs also discovered the chillingly true fact that the disease was familial and was widely prevalent among the Ashkenazi Jews of Eastern Europe.

Let us look at what exactly the disease does in a brief and consolidated manner. Babies that are born healthy develop their major vital body functions like vision, hearing and movement because the enzymes present in their body clear out al the unwanted material like fatty proteins. These materials are primarily destroyed because of their ability to interfere with the growth of the child. When the baby is born without one of these crucial and important enzymes, the fatty proteins tend to build up in the brain and hence the vital functions of the child are affected to a large extent. Babies suffering from Tay Sachs disease do not have the enzyme Hexosaminidase A, also known as Hex A. The lack of this enzyme results in a breakdown in normal body functions and hence impairs movement, eyesight, hearing and overall mental well being.

Since the disease is inherited, the destructive process of the disease begins during the phase of early pregnancy. But then, it largely remains undetected till the child is a few months old. The disease reaches a critical stage at the age of three or four, as the nervous system totally gives way and life can no longer be supported. All the reported cases of Tay Sachs disease have recorded fatality by the age of four to five.

The disease progresses through various stages and symptoms rise only after the baby's first six months have passed. The baby's peripheral vision gives way first, and an abnormal startle response can be noticed in the infant around this time. As the disease progresses, seizures follow along with diminished mental response and function. This leads to mental degradation in the infant, and essential functions and skills are lost one by one. The child loses its ability to crawl, sit, turn over or even reach out. These are the primary symptoms of diminishing mental well being. Secondary symptoms include difficulty in swallowing, difficulty in breathing and a lack of coordination in the child's actions. These symptoms are followed by a more severe range of effects during the closing phases of the disease, with the child going permanently blind and non responsive to the events around it, and falling prey to temporary paralysis. There is no effective cure for the disease so far, but measures can be taken by the parents to prevent the occurrence of the disease in their children.