State Representative Scott Oelslager, Chair
Ohio House Finance Committee
Ohio House of Representatives
77 South High Street
Columbus, Ohio 45215
April 19, 2021
CC Members, Ohio House Finance Committee,
My name is Morgan DeBoth and I serve as the Director for Family Support at Batten Disease Support and Research association, BDSRA. We are a national organization with an international reach based in Columbus, OH. I am writing you in support of HB110, which requires Ohio’s expert newborn screening advisory council to promptly review any new condition added to the federal Recommended Uniform Screening Panel (RUSP), and if recommended by this council, add that condition to the Ohio’s newborn screening panel.
Batten Disease (Neuronal Ceroid Lipofuscinosis) is a rare lysosomal storage disease that effects roughly 1 in 100,000 to 1 in 200,000 children in the U.S. annually. Of our 13 variants of Batten, one has a treatment, CLN2. We’re currently advocating for it to be added to the RUSP. While we continue to pursue its place on the RUSP, we want to ensure that when that time comes Ohio will be ready to add it to the newborn screening process. The language in HB110 will eliminate any unnecessary delay in screening for diseases such as Batten, ensuring all Ohio babies born with debilitating and life-threatening diseases are diagnosed and treated at the earliest age possible to avoid devastating delays.
The burden of disease can be greatly decreased with adequate and timely diagnosis. In the last 6 years, we have shifted the average age of diagnosis from 6-8 years of age to an average of 3-6 years of age. That may seem like a small shift, but to a parent looking for answers, those 3 years make a world of difference.
At BDSRA, we understand moving the needle for others in our rare disease community moves the needle for us all. We stand with others who support this legislation. We ask you to support the passing of HB110 and for the continued to support of the rare disease community.
Director of Family Support
Batten Disease Support and Research Association
PH: (614) 973-6013