Galactosemia is often confused with lactose intolerance since both conditions have to do with digestion of sugars from dairy products, but there is a difference. While lactose intolerance is a miserable inconvenience cause by the inability of the body to produce lactase, galactosemia is a life threatening genetic condition that affects infants from birth.

In children with galactosemia the body produces lactase which then breaks down the lactose into galactose and glucose. It is the galactose then that becomes the problem as the liver does not produce the enzymes necessary to digest galactose. When this happens the galactose builds up in the blood to toxic levels leading to an enlarged liver, cirrhosis, renal failure, cataracts, and brain damage. If not treated the mortality rate for this condition is 75% in infants.

Infants are now routinely screen in the US for this deadly genetic condition. Every effort is made to make sure that galactosemia is diagnosed early.  Symptoms of galactosemia include lethargy, vomiting, diarrhea, failure to thrive and jaundice. Since these symptoms can be present in many disorders galactosemia may not be easily or quickly diagnosed. Luckily there is a routine screening for most infants now, a skin prick in the heel is done, and the blood is checked for the presence of three enzymes that are needed to digest galactose properly. If an infant has galactosemia one of these three enzymes will be absent and levels of galactose in the blood will be too high.

The treatment for galactosemia is similar to the treatment for lactose intolerance; the elimination of lactose and also of galactose as well. Many of the children with this condition can still experience long term complications even if it is caught early and treated promptly such as neurological impairments, speech difficulties such as ataxia, and learning disabilities.

The long term complications of galactosemia make this a far more serious condition than lactose intolerance and unlike lactose intolerance these children will never be able to consume lactose in their lifetime.