What kind of problems can happen?
  If a woman with PKU becomes pregnant, and she is not on a
well-controlled diet, there is a very high chance that her baby will have
serious problems, like mental retardation and birth defects. This is
because people with PKU who do not follow a low-phenylalanine diet have
extra phenylalanine stored up in their bodies. This phenylalanine is
passed to the baby while a mother is pregnant, and can be very harmful
for the developing baby.

Birth defects due to uncontrolled diet in the mom are separate from the
inherited PKU condition. Most moms with PKU will not have kids with PKU.
Brain and other damage that happens before birth to babies born to PKU
moms with high phenylalanine levels cannot be fixed or helped by a special
diet. These problems are permanent.

Because some women experience nausea and vomiting with their pregnancies, this can further complicate staying on the low protein plus formula diet for women with PKU.
How can these problems be prevented?
  Although the chance for having a baby with these or other types of birth
defects is never zero, the good news is that girls and women with PKU who
follow their special diet to lower their phenylalanine levels before and during pregnancy are more likely to have a healthy baby. Before becoming pregnant, it is helpful for young women with PKU to have their blood levels checked and it is very important that they get help from a doctor or nutritionist to plan their diet before and throughout pregnancy.
Why should I think about this NOW?
  Even if you are not planning to become pregnant, if there is any chance at
all that pregnancy might happen accidentally, good diet control now may
help prevent serious problems in a surprise pregnancy. If you are sexually
active and do not want to become pregnant, or feel that you could not control your diet right now, it is important that you consistently use a reliable method of birth control. Your doctor or nurse can confidentially give you more information about birth control if you need it. Finally, even if pregnancy is a possibility far in the future, it will be easier to "stay on" the diet then if you practice good control now. Plus good diet control is better for YOUR health right now and in the future!