Dear Dawn, Sandy and others who may have been wondering ...
 
Galactosemia is a rare enzyme deficiency which results in sufferers being
unable to metabolize galactose, which is one of the components of lactose.
The galactose is partially metabolized to a toxic by-product, which can
then build up in the body causing developmental problems for children with
this condition.
 
Treatment is by totally excluding sources of galactose from the diet, which
in practice means excluding lactose, since free galactose doesn't occur in
very many foods. Hence my reason for joining this list. My 3-year-old son
Hugh has galactosemia. I spend a lot of time telling people that Hugh's
problem is _not_ lactose intolerance, which many people seem to have some
knowledge of, and a lot of time reading the ingredients lists of the food
that we buy (which I guess that many of you on this list must also spend a
lot of time doing).
 
In case you wanted more detail on what actually goes wrong ...
 
The lactose is broken down into its components glucose and galactose, and
the galactose is then supposed to be converted (through a fairly convoluted
chemical route) to glucose, mainly in the liver. In galactosemia, one of
the intermediate stages in this process is blocked (because the enzyme
which catalyzes the reaction is inoperative). There would be no visible
reaction to ingestion of milk products, but over a period of time the
damage would be done. Hugh has blood tests every three months to make sure
that the condition is well controlled.
 
Thank you Dawn and Sandy for being interested ...
 
Cheers,
 
Elaine
 
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*  Elaine Lally                                                          *
*        http://www.nepean.uws.edu.au/humanities/postgradstud/pg_elally/ *
*  Humanities and Social Sciences                                        *
*  University of Western Sydney, Nepean                                  *
*  PO Box 10, Kingswood            *!* Please note - new email address *!*
*  NSW, Australia  2747              [log in to unmask]  *
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