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