How is IVA inherited?
IVA is inherited in an autosomal recessive manner. It affects both boys and girls equally. Everyone has a pair of genes that make the isovaleryl-CoA dehydrogenase enzyme. In children with IVA, neither of these genes works correctly. These children inherit one non-working gene for the condition from each parent. Parents of children with IVA rarely have the condition themselves. Instead, each parent has a single non-working gene for IVA. They are called carriers. Carriers do not have IVA because the other gene of this pair is working correctly. When both parents are carriers, there is a 25% chance in each pregnancy for the child to have IVA. There is a 50% chance for the child to be a carrier, just like the parents. And, there is a 25% chance for the child to have two working genes. Genetic counseling is available to families who have children with IVA. Genetic counselors can answer your questions about how the condition is inherited, choices during future pregnancies, and how to test ot
