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Diet for Galactosemia

By Sharon Perkins ; Updated July 18, 2017

Galactosemia, an inherited disease, affects around one in 62,000 newborns, the Merck Manuals Online Medical Library reports.These children lack an enzyme that converts galactose, a milk sugar, to glucose. People with the classic and most common form lack galactose-1-phosphate uridyl transferas, or GALT. Galactosemia is especially dangerous for newborns, whose entire diet consists of milk. Newborn screening diagnoses galactosemia so treatment can start promptly, minimizing damage. Eliminating galactose from the diet treats the disease.

Definition

Classic galactosemia occurs when an infant inherits the defective gene from both parents. A variant of classic galactosemia, called Duarte galactosemia, occurs when an infant inherits a Duarte variant gene from one parent and a classic gene from the other. Children with Duarte galactosemia have 25 to 50 percent of normal enzyme activity on the GALT gene, the Parents of Galactosemia Children website explains. People with Duarte galactosemia have fewer dietary restrictions and complications than people with the classic form.

Infant Diet

Infants with classic galactosemia cannot have products containing lactose because galactose, which makes up half of lactose, builds up in the bloodstream, damaging the brain, eyes, kidney and liver. Infants with galactosemia cannot have animal or human milk and must consume soy milk instead.

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Other Restrictions

Although milk is the most common source of galactose for infants, sources of galactose outside of dairy products are also forbidden as the child grows. Many processed foods contain lactose, so learn to read labels for milk products such as casein, dry milk solids, lactose, curds and whey. Organ meats, dough conditioners in processed foods and hydrolyzed proteins are a few of the additional substances that must be avoided. Legumes and fermented food additives, such as soy sauce, should also be avoided. People with galactosemia can eat lactate, lactic acid, lactylates and calcium compounds because they don’t contain lactose. Some medications also contain lactose as a filler; make sure your pharmacist knows your restrictions.

Modifications

Children with Duarte galactosemia may be able to follow a less restricted diet, limiting galactose for the first year, then gradually introducing items and checking blood levels, Parent of Galactosemic Children states. Different doctors have different opinions about diet and Duarte galactosemia, so follow your own doctor’s recommendations.

Symptoms

Eating food containing galactose doesn’t cause an immediate reaction as an allergy would, but problems like vomiting, poor growth, lethargy, cognitive defects, jaundice, convulsions and speech, gait and balance problems can develop. Osteomalacia, or weakened and soft bones, can occur from high levels of calcium in the blood. Cataracts can affect the eyes. Girls with galactosemia can develop premature ovarian failure.

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