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Spring 2002
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Maternal
PKU and Fetal Welfare
Martha J, a 23-year-old female, was born with PKU (phenylketonuria), an
enzyme deficiency that prevents the metabolization of phenylalanine.
Children born with PKU are ordinarily placed on a special low-phenylalanine
diet for at least the first five years of their life. Although the diet
is necessary to prevent severe retardation, it is very burdensome, not
only because normal foods are very limited but also because the main
source of protein is a bad-tasting “medical food”. Because Martha J
was placed on this special diet in her childhood, she does not suffer
from retardation.
Martha J is four-months pregnant. Although her inability to metabolize
phenylalanine is no longer a problem for her own well being, there is a
problem for the fetus she is carrying. Unless Martha J maintains the
same low-phenylalanine diet throughout the course of her pregnancy, her
fetus is at grave risk for severe retardation, microcephaly, congenital
heart disease, and other disorders. Martha J’s religious beliefs have
motivated her to decide against abortion. Nevertheless, she is
ambivalent about her pregnancy, because she is unmarried and depressed
by the breakdown of her relationship with the child’s father. She is
also finding it very difficult to adhere to the same dietary
restrictions that she found so oppressive in her childhood. Dr. R, the
obstetrician who is caring for Martha J, has repeatedly emphasized the
importance of adhering to the prescribed diet, but Martha J acknowledges
that she has been inconsistent in doing so.
Should Dr. R encourage Martha J to
reconsider the possibility of abortion?
- If Martha J is resolved to carry her fetus to term,
how should Dr. R. deal with the fact that she is not maintaining the
prescribed diet?
- If all else fails, should Dr. R seek a court order
that would place Martha J in a supervised setting where dietary
restrictions could be enforced?
Would your approach to this problem differ is Martha
J. was HIV positive?
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Course
Overview