Maternal PKU Knowledge Test

Knowledge is power, right? That appears to be the approach that PKU-related research has taken over the past two decades.

The Maternal PKU Knowledge Test was developed in 1990 by Shiloh, St. James and Waisbren and was recommended as a way for clinics to screen and identify adult women with PKU who might benefit from more education. The major premise for this approach was that education would enable patients to self-manage their condition and prevent the occurrence of maternal PKU syndrome. A least one, more recent study—conducted in 2011 by Kimberly R. Regis RN, MS, CPNP, and titled Childbearing age women with PKU: Assessment of care needs and preferences—acknowledged that while awareness is an important component, perhaps education alone is not enough to improve pregnancy success rates among adult PKU women.

Take the 10-question quiz below to see how well-informed you are about maternal PKU, or to read more about this shift in maternal PKU research, visit the three-part blog series: Caring for Maternal PKU.

1.  PKU is __________.

A. a blood disease

B. an enzyme deficiency

C. a kidney disorder

D. a protein deficiency

E. an iron deficiency

 2.  Mental retardation in babies born to mothers with PKU is likely caused by ________?

A. an enzyme deficiency in the baby

B. PKU in the baby

C. high blood phenylalanine

D. the father carrying the gene for PKU

E. too little protein in the mother’s diet during pregnancy

 3.  The best known treatment for maternal PKU to prevent damage to the baby is ____?

A. following a well-balanced diet

B. following a vegetarian diet

C. following a high protein diet during pregnancy

D. following a low phenylalanine diet after a positive pregnancy test

E. following a low phenylalanine diet before conception and throughout pregnancy

 4.  In addition to mental retardation, other problems that have been seen in babies born to mothers with PKU include __________.

A. low birth weight

B. heart problems

C. small head size

D. all of the above

E. none of the above

 5.  Twenty mg/dl is considered a high blood phenylalanine level. On a low phenylalanine diet during pregnancy, blood phenylalanine levels should be controlled to what level?

A. Less than 1 mg/dl

B. 2-6 mg/dl

C. 6-8 mg/dl

D. 10-15 mg/dl

E. 16-20 mg/dl

6.  Which of the following snacks has the least amount of phenylalanine?

A. Chocolate chip cookies

B. An apple

C. A hamburger

D. Potato chips

E. A bagel with jelly

7.  Children born to mothers with PKU ________________.

A. never have PKU

B. have a 1 in 1000 chance of having PKU

C. have a 1 in 100 chance of having PKU if the father carries the gene for PKU

D. have a 50-50 chance of having PKU if the father carries the gene for PKU

E. will always have PKU

8.  The problems that are seen in babies from untreated pregnancies in mothers with PKU _____________________.

A. are entirely reversible

B. can be corrected by surgery

C. can be corrected by treating the baby with a low phenylalanine diet

D. generally result in the child having mental retardation, learning difficulties, birth defects, and the need for special services

E. go away as the child grows older

9.  After a child is born to a mother with PKU, it is important ________________.

A. to place the child on a high protein diet

B. to perform newborn screening for PKU with special care and consideration so that if the child has PKU, he or she can begin dietary treatment

C. to hold off on newborn screening for PKU for a month since the baby has had enough stress

D. for the mother to be on a high protein diet

E. to immediately place the child on a low protein diet

10.  The best advice to give a young woman with PKU who thinks she might be pregnant is to ________________.

A. wait and see if it is true

B. wait, but stop eating meat in the meantime

C. wait, but start using birth control

D. immediately contact the PKU clinic for guidance

E. contact her friends for guidance

Answers: 1b, 2c, 3e, 4d, 5b, 6b, 7d, 8d, 9b, 10d

Source: Shiloh, S., St. James, P., & Waisbren, S. (1990). The development of a patient knowledge test on maternal phenylketonuria. Patient Education and Counseling, 16(2), 139-146.

One response to “Maternal PKU Knowledge Test

  1. Pingback: Caring for Maternal PKU: Part II | PKU Parlor

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