A nurse is updating the plan of care for a newborn who is undergoing phototherapy. Which of the following actions should the nurse include in the plan?

Choice A reason:

Stop breastfeeding. This is the correct answer because newborns and infants with galactosemia cannot metabolize galactose, a sugar found in milk and lactose-containing formulas, including breast milk. Galactose can build up in their blood and cause serious complications such as liver damage, cataracts, brain damage, and even death. Therefore, the therapeutic management for this newborn is to stop breastfeeding and switch to a soy or elemental formula that contains no galactose.

Choice B reason:

Add amino acids to the breast milk. This is incorrect because adding amino acids to breast milk will not prevent the accumulation of galactose in the newborn's blood. Amino acids are the building blocks of proteins, not sugars. Adding amino acids to breast milk will not change its galactose content or help the newborn metabolize it.

Choice C reason:

Substitute a lactose-containing formula for breast milk. This is incorrect because lactose is a disaccharide composed of glucose and galactose. Lactose-containing formulas will also expose the newborn to galactose, which they cannot break down. Lactose-containing formulas should be avoided in newborns and infants with galactosemia.

Choice D reason:

Give the appropriate enzyme along with breast milk. This is incorrect because there is no enzyme therapy available for galactosemia. Galactosemia is caused by a genetic defect in one of the enzymes involved in the breakdown of galactose, such as galactose-1-phosphate uridyltransferase (GALT), galactokinase (GALK), or uridine diphosphate galactose-4-epimerase (GALE). Giving an enzyme along with breast milk will not correct this defect or prevent the harmful effects of galactose accumulation.

Choice A reason:

All milk- and lactose-containing formulas, including breast milk, must be stopped during infancy. Soy protein is the formula of choice for newborns and infants with galactosemia. Breast milk should not be used in newborns and infants with galactosemia because it contains galactose, which they cannot metabolize properly.

Choice B reason:

Adding amino acids to the breast milk will not help the newborn with galactosemia, because the problem is not a lack of amino acids, but a deficiency of the enzyme that breaks down galactose. Amino acids are the building blocks of proteins, not sugars.

Choice C reason:

Substituting a lactose-containing formula for breast milk will worsen the condition of the newborn with galactosemia because lactose is composed of glucose and galactose. The newborns will still be exposed to galactose, which will accumulate in the blood and tissues and cause damage.

Choice D reason:

Giving the appropriate enzyme along with breast milk is not a feasible option for the newborn with galactosemia, because there is no oral enzyme replacement therapy available for this condition. The only treatment is dietary restriction of galactose.