Tay-Sachs disease is a fatal inherited disease of the central nervous system as a recessive gene. The central nervous system includes the brain, the coverings of the brain, and the spinal cord. The most common form of the disease affects babies. Babies with Tay-Sachs disease lack hexosaminidase A, an enzyme that is necessary for breaking down certain fatty substances in brain and nerve cells. These fatty acids then accumulate in the brain causing neurological deterioration. Affected babies appear healthy at birth and seem to develop normally for the first few months of life. The destructive process begins in the fetus early in pregnancy.

Symptoms of classical Tay-Sachs disease first appear at 4 to 6 months of age when an apparently healthy baby gradually stops smiling, crawling or turning over, loses its ability to grasp or reach out

Tay-Sachs disease is only transmitted through heredity. A Tay-Sachs carrier has one normal gene for hex A and one Tay-Sachs gene. The carrier does not have the illness and leads a normal, healthy and full life. However, when two carriers become parents, there is a one-in-four chance that any child they have will inherit a Tay-Sachs gene from each parent and have the disease. There is a one-in-four chance that the child will inherit the normal gene from each parent and be completely free of the disease and the Tay-Sachs gene. There is a two-in-four chance that the child will inherit one of each kind of gene and be a carrier like the parents and free of disease. A pair of genes on chromosome 15 controls Tay-Sachs disease. These are the genes that code for the enzyme Hex-A. If either or both Hex-A genes are active, the body produces enough of the enzyme to prevent the abnormal build-up of the GM2 ganglioside lipid.

Tay-Sachs disease occurs most frequently in descendants of Central and Eastern European (Ashkenazi) Jews. 85% of Tay-Sachs disease victims are Jewish. Some non-Jewish individuals of French-Canadian ancestry and members of the Cajun population in Louisiana, are at similarly increased risk. These groups have about 100 times the rate of occurrence of other ethnic groups. Approximately one in every 27 Jews in the United States is a carrier of the TSD gene. The carrier rate in the general population is about one in 250. Fewer than 100 children are born with the disease each year in the U.S.

Although a cure for Tay-Sachs disease does not exist at the present time, support for families of affected children is available through organizations such as the National Tay-Sachs and Allied Diseases Association. Time in an extended-care facility for basic care if parents are unable to provide it for the child at home. Psychotherapy or counseling for parents and siblings are available to learn how to cope with the distress produced by this condition. The child’s doctor may prescribe anti-convulsants to control the child's seizures, stool softeners and laxatives to relieve constipation, and other medicines to control symptoms. In the early stages of the disease, parents should encourage the child to be as active as possible. Increasing mental, nervous and mu

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