The pediatric nurse is providing care to an infant diagnosed with phenylketonuria. What education is vital for this nurse to provide the parents?
A.
Information available from the Centers for Disease Control and Prevention
B.
High-protein, low-carbohydrate diet for the life of the baby
C.
Special phenylalanine-free infant formula and diet restriction
D.
Very-low-protein diet supplemented with thiamine during childhood
ANS: C
Phenylketonuria (PKU) is an autosomal recessive inborn error of metabolism. Individuals with PKU cannot convert phenylalanine to tyrosine, and if left untreated, the condition causes complications such as intellectual deficits. The person must follow a phenylalanine-free diet, which means eliminating protein, for the rest of his or her life. There are special formulas for infants with PKU. Information for the parents about informational resources is important, too, but the priority is on educating them regarding the diet. The child should not be on a high-protein diet. A low-protein diet supplemented with thiamine is the treatment for maple syrup urine disease, not for PKU.
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