TAY-SACHS
DISEASE & SANDHOFF'S
DISEASE
Tay-Sachs
disease is a genetic disease in which the enzyme hexosamindase-A is
deficient; Sandhoff’s disease occurs when hexosamindase A and
B are low or absent and results in major neurological damage to the
child. Although originally thought to occur only in families of Jewish
heritage the defective gene has now been found in diverse populations
in the Middle East, Ireland, Italy, Phillipines, Eastern Europe.
Until
recent times there has been no treatment available for either of these
conditions.
The
infantile form of Tay-Sachs is noted by a normal developmental period
the first six months after which the baby loses its neck control, ability
to turn over front to back or reverse; unable to sit or crawl or stand.
Later between 15-24 months of age the child develops a cherry red spot
in the eyes, loses its ability to see and hear. A juvenile form of the
disease also occurs in some cases in which the neurological degeneration
oocurs after five years of age.
Sandhoff’s
disease is very similar to Tay-Sachs except that both the hexo A and
B enzymes are deficient.
STEMCELLREGENMED
has developed a protocol which includes the following: