Rep. Jim Weidner is working on a bill to start a pilot program to screen newborns for Krabbe disease. Unless the disease is caught very early, it is fatal. The parents of 5-year-old Marshall Wilson, who is dying of the disease, urged Oregon legislators to include Krabbe testing for all newborns. The Wilsons are working on legislation with the Hunter’s Hope Foundation, founded by NFL player Jim Kelly after his infant son was diagnosed with the disease.
Krabbe disease, also known as globoid cell leukodystrophy, is a rare condition that occurs in about one in 100,000 births. An inherited genetic mutation causes an enzyme deficiency which destroys the protective coating (myelin sheath) of nerve cells in the brain and nervous system. A baby who is born with the disease usually dies before age two unless the disease is caught before the baby experiences any symptoms.
If the disease is discovered in a newborn and treated, it can be stopped. Marshall’s baby brother, Michael, was tested at birth. He, too, had the disease, but he was given an umbilical cord blood transplant, which stopped the disease’s progression. Marshall was diagnosed too late to save his life.
Weidner’s bill would make screening for Krabbe available for newborns. Parents would be informed and could opt for the screening. They would be responsible to pay for it. Weidner’s Chief of Staff, Jameson Gideon, said the cost would be anywhere between $2 and $50, depending on the method of testing used.