During digestion, lactose (a complex milk sugar) is broken down into two simple sugars, glucose and galactose. The galactose and glucose then enter the bloodstream where the galactose is converted in more glucose.  Glucose is used as a primary source of energy for the body.

Not to be confused with lactose intolerance, Galactosemia is a much more dangerous disorder that involves the inability to break down galactose.  While someone with Galactosemia may have no problem in digesting lactose, their body lacks the ability to metabolize galactose.  As a result, galactose accumulates in the body, where it may damage the liver, central nervous system, eyes, kidneys, and other body systems.

Individuals with galactosemia cannot tolerate any form of milk, including human breast milk.  After drinking milk for a few days, a newborn with galactosemia will refuse to eat.  Symptoms that may occur include jaundice, vomittting, poor weight gain, lethargy, irritability, and convulsions.  Continued feeding of milk to infants with galactosemia will result in cirrhosis of the liver, cataract formation in the eye, and mental retardation.

Treatment for galactosemia involves a strict lifelong milk-free diet, abstaining from all milk and products containing milk, essentially a dairy-free diet.

For more information on this condition, visit:

Parents of Galactosemic Children - www.galactosemia.org
Medline: Galactosemia - http://www.nlm.nih.gov/medlineplus/ency/article/000366.htm

Thank you to Jennifer for bringing this disorder to our attention.