Galactosemia

Galactosemia
What is it?
*Galactose- high levels, usually a lack of an enzyme to metabolize galactose,
present from birth
*An inborn error of metabolism -
-necessitates the removal of all food sources of galactose
*Mothers- are not encouraged to breast feed
Facts:
*Babies- 1 out of 50,000 to 70,000 born without this enzyme
*Appearance- normal at birth,
-few days or weeks- jaundice, nausea, vomiting, decreased appetite,
normal growth stops
*Liver- enlarged, excess amount of protein/amino acids in urine,
body retains fluids- may need lower sodium talk with your Physician
*If no treatment- child becomes mentally retarded, short stature, cataracts, death
*Child’s IQ- lower than siblings, frequently have speech problems
*Girls- normally have problems conceiving
*Galactose- is converted to glucose in the liver, legumes contain galactose
*During lactation- body converts glucose to galactose to form breast milk
*Diet- to lower Red Blood Cell galactose and galactose-1-phosphate
to near normal levels
-Prevent buildup- in brain, eyes, liver, kidney, tissues
What do I need to do?
*See your Health Care Provider- immediately
*Eliminate- milk/milk products, lactose, nonfat dry milk solids, casein, whey,
whey solids, also found in some fruit and vegetables, seaweed
-Acceptable- lactate, lactic acid, lactalbumin
-Avoid if pregnant- galactose can cross to fetus, can cause cataracts
*See a Registered Dietitian- on line with FFF or see a local
RD for help with meal planning
*READ ALL FOOD LABELS