Nurses' role in preventing birth defects in offspring of women with phenylketonuria.
Most women who began nutrition support as neonates for a diagnosis of phenylketonuria, an inherited defect in phenylalanine metabolism, are of normal intelligence, no longer require a restricted diet, and wish to have children of their own. Phenylketonuria that is untreated when a woman conceives and during gestation results in poor reproductive outcomes. Treatment with and careful monitoring of a phenylalanine-restricted diet can improve reproductive outcome. Nurses have the primary responsibility in locating women of childbearing age with phenylketonuria; developing strategies to improve palatability of the diet, thereby enhancing compliance; providing ongoing monitoring and support of the mother-child dyad; and counseling couples at risk.
Acosta
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PB
Ross Laboratories, Columbus, Ohio.
Wright
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D000013:Congenital Abnormalities / Q000209:etiology / Q000517:prevention & control*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008403:Mass Screening; D008991:Monitoring, Physiologic; D015997:Neonatal Screening; D010661:Phenylketonurias / Q000150:complications / Q000188:drug therapy / Q000451:nursing*; D016742:Preconception Care; D011247:Pregnancy; D011248:Pregnancy Complications / Q000188:drug therapy / Q000451:nursing*; D011256:Pregnancy Outcome; D011295:Prenatal Care