Think about it that way:
Your genetic code has 2 letters, A and B. Having B in your code means you can have the defect.
You are AB (which means you have a 50% chance of having the defect), and so is your sister.
If you have a child, he/she will have a 25% of definitely having the defect (BB), 50% chance of having it half of the time (AB), and 25% of definitely being healthy.
Now think there’re the pairs AB and CD, that have the same mechanic (AB is ABCC and CD is AACD). If a ABCC has a child with a AACD, the chances of having the defect are lower (AB with AA results in a 50% chance of maybe; same for CC with CD) than if you crossed ABCC with ABCC.
This is very *very* oversimplified, but it gives the idea, I think.
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