September 5, 2019 - Health officials, state leaders and patient advocates gathered at the state public health laboratory in Austin today to celebrate the addition of a new test to the health screening done for every baby born in Texas. In August, the Texas Department of State Health Services began testing all newborns for X-linked adrenoleukodystrophy making Texas the 16th state to add X-ALD to its screening panel.
X-ALD is a rare, serious genetic disorder characterized by damage to the nervous system and adrenal glands that do not work properly. A mutation of a gene on the X chromosome leads to a build-up of fatty acids in the body, causing damage to nerve tissues.
“X-ALD affects about one in 17,000 people worldwide, and we expect adding this screening will help identify 10-14 cases a year in Texas and connect those children to treatment before they show any symptoms,” said Dr. John Hellerstedt, DSHS Commissioner. “Early diagnosis is the key to preventing the most serious, harmful effects.”
Newborns don’t show any signs of X-ALD, but symptom onset may occur as early as two years of age. Children with the most severe form of the disorder may first exhibit learning and behavioral problems, and children with it may experience rapid neurologic decline. The timeframe between symptom onset to being unable to move and communicate or death is approximately three years but can vary widely.
“Newborn screening can be a life changer for babies with rare disorders, and we’re so pleased to broaden our screening panel to include X-ALD,” said Dr. Courtney N. Phillips, HHS Executive Commissioner. “Early interventions make a dramatic difference in quality of life, and every day our work at the state’s health lab helps improve the lives of Texas babies. Nothing is more important than the health of our children.”
The Texas Newborn Screening Laboratory tests nearly 800,000 specimens each year for close to 400,000 newborns. A simple heel stick blood sample collected from newborns one to two days after birth and again two weeks later enables testing of every Texas baby for 54 disorders or medical conditions. Finding and treating these disorders early can prevent serious complications, such as growth problems, developmental delays, deafness, blindness, intellectual disabilities, seizures and sudden or early death.
Texas law requires newborns to be screened for disorders on the federally-endorsed Recommended Uniform Screening Panel as funding allows. The Texas Legislature has provided funds that will allow DSHS to add testing for spinal muscular atrophy, and recent legislation created a dedicated fund to support the addition of new conditions to Texas’ screening panel in the future.
"During the 86th Legislative Session, I was proud to have authored and passed Senate Bill 748 that created the Newborn Screening Preservation Account," said Sen. Lois Kolkhorst. “With this and other maternal and neonatal legislation passed this session, Texas is poised to become a national leader in newborn and maternal health.”
Rep. Sarah Davis, House sponsor of SB 748, noted “These funds will enable DSHS to implement new federally recommended screenings as they become available. I am excited to see DSHS’s implementation of these efforts and look forward to the positive results this will bring to Texas children and families.”
Chair of the House Public Health Committee and bill sponsor Rep. Senfronia Thompson said, “With a newly dedicated account and financial stability, infants will continue to receive proper screening and treatments. I am excited to see all the good that comes from the newborn screening program and look forward to supporting its efforts in the future.”