Washington State
House of Representatives
Office of Program Research
BILL
ANALYSIS
Health Care & Wellness Committee
SB 6234
Brief Description: Screening newborn infants for branched-chain ketoacid dehydrogenase kinase deficiency.
Sponsors: Senators Wilson, L., Hasegawa and Lovick.
Brief Summary of Bill
  • Requires the State Board of Health to consider whether to add the branched-chain ketoacid dehydrogenase kinase deficiency screening to the mandatory newborn screening panel and submit a report to the Legislature by June 30, 2025.
Hearing Date: 2/21/24
Staff: Emily Poole (786-7106).
Background:

Newborn Screenings.

The Department of Health (DOH) tests all infants born in Washington for a number of rare but treatable disorders that may lead to intellectual disabilities or physical defects as defined by the State Board of Health (Board).  Tests may not be given to any newborn infant whose parents or guardians object on the grounds of religious tenets and practices.

 

In order to determine which conditions to include in the newborn screening panel, the Board convenes an advisory committee to evaluate candidate conditions using guiding principles and an established set of criteria, including the availability of screening technology and the benefits of detecting a condition early.

 

Branched-Chain Ketoacid Dehydrogenase Kinase Deficiency.

Branched-chain ketoacid dehydrogenase kinase (BCKDK) deficiency is a genetic condition that is understood to cause branched-chain amino acid depletion.  The condition is linked to a neurodevelopmental disorder characterized by autism spectrum disorder, intellectual disability, motor impairment, and microcephaly, which is a condition where a baby's head is much smaller than expected.
Summary of Bill:

The Board must consider whether to add the BCKDK deficiency screening to the mandatory newborn screening panel.  The Board is required to submit a report to the Governor and the Legislature by June 30, 2025, including a summary of the Board's evaluation and any findings and recommendations on the addition of BCKDK deficiency to the newborn screening panel.
Appropriation: None.
Fiscal Note: Available.
Effective Date: The bill takes effect 90 days after adjournment of the session in which the bill is passed.